Cardiologia Croatica. 2023;18(5-6)

Luka Perčin, Goran Međimurec, Dubravka Šipuš, Petar Martinčić, Mislav Planinc, Irena Ivanac Vranešić, Maja Hrabak Paar, Darko Anić, Kristina Marić Bešić

**Introduction**: Sinus of Valsalva aneurysm (SVA) is a rare cardiac anomaly with an incidence of approximately 0.09% in the general population (1). Although it can develop secondary to events and conditions that weaken the aortic wall, such as trauma, connective tissue disease, bacterial endocarditis, syphilis, cystic medial necrosis, and atherosclerosis, it is most commonly a congenital disorder. Furthermore, about one-third of patients may have additional cardiac abnormalities such as a ventricular septal defect (VSD) or aortic regurgitation (AR) (2). The most frequent complication is rupture of the aneurysm into the right ventricle, which has serious hemodynamic implications. **Case report**: 30-year-old male patient with a medical history of restrictive VSD and mild AR was admitted to the hospital due to progressive shortness of breath. On physical examination, the patient was hemodynamically stable and mildly dyspneic at rest. Heart sounds were covered with a loud continuous murmur and bilateral fine basal crackles were noted on pulmonary auscultation. Jugular venous pressure was elevated, but there was no evidence of peripheral edema. 12-lead electrocardiogram showed mild sinus tachycardia while laboratory tests pointed on markedly elevated NT-proBNP levels. Chest radiography displayed enlargement of the cardiac silhouette with mild pleural effusion and cranial redistribution of the pulmonary vasculature. Transthoracic echocardiography (TTE) exam revealed a rupture of the right sinus of SVA into the right ventricle with significant left-to-right shunt (**Figure 1A**). The patient subsequently underwent cardiac surgery (**Figure 1B**). Excision of the aneurysm and patch repair of the right SVA were successfully performed followed by an uneventful postoperative period (**Figure 1C**). Control TTE examination verified completely repaired SVA, which correlated with the patient’s good clinical recovery (**Figure 1D**). The patient was discharged from the hospital in a stable condition. FIGURE 1. A Preoperative transthoracic echocardiography (TTE) long-axis parasternal view. The red arrow points on the ruptured right sinus of Valsalva aneurysm (SVA). B Intraoperative view before the repair. The black arrow points to ruptured SVA. C Intraoperative view after the excision of the aneurysm and repair of the right SVA with BioIntegral porcine. D Postoperative TTE long-axis parasternal view. The red arrow points on the repaired site. **Conclusion**: We present the rupture of SVA as a differential diagnosis of acute heart failure in young, otherwise healthy patients. In addition, we would like to encourage regular cardiac follow-up of patients with restrictive VSD, especially in the presence of concomitant aortic disease, to prevent this severe complication.

Lucija Klobučar, Željka Breškić Ćurić, Ivana Lukić, Kristina Selthofer-Relatić

**Introduction**: Left ventricular hypertrophy (LVH) diagnosed by echocardiography has broad differential diagnosis. LVH is most frequently caused by abnormal loading conditions (i.e. uncontrolled arterial hypertension and valvular heart disease). When LVH cannot entirely be explained by abnormal loading conditions the diagnosis of hypertrophic cardiomyopathy (HCM) is made. The most common cause of HCM is mutation in cardiac sarcomere protein genes. Some of the less common causes of HCM are metabolic, infiltrative and neuromuscular diseases (1). Genetic testing is important in recognizing family members at risk of developing HCM. Detected variants of cardiac sarcomere protein genes can be classified (and reclassified) as pathogenic/ likely pathogenic, benign/ likely benign and variant of unknown significance (VUS) (2). **Case report**: We present the case of 55-year-old, hypertensive patient who was admitted to the hospital because of recurrent presyncope and signs of LVH with typical load signs in the ECG that could not be related to hypertensive heart disease. Non-obstructive HCM with preserved ejection fraction was diagnosed by echocardiography, elevated hsTn and NTproBNP were detected and atherosclerotic coronary artery disease was excluded (**Figure 1**). By ECG monitoring ventricular nonsustained tachycardia was detected and an implantable cardioverter defibrillator was implanted in primary prevention of sudden cardiac death. Genetic testing identified a heterozygous missense variant rs201381085 in TTN gene and heterozygous missense variant in MYH6 gene. Currently, both of these variants are classified as VUS but with known HCM cases in literature related to these mutations. Genetic screening also revealed the same heterozygous variant in TTN gene in his 26-year-old son who is currently phenotype-negative (**Figure 2**). FIGURE 1. Cardiac magnetic resonance image of presented patient. FIGURE 2. Cardiac magnetic resonance image of patient’s phenotype-negative son. **Conclusion**: In daily clinical practice, genetic testing of HCM patients is still unfortunately underutilized due to work overload, administrative and organizational difficulties. Knowing the importance and potential benefits of genetic testing for the patient, as for their families, the goal is to simplify the entire process.

Anela Subo, Omer Manov, Azra Sabic, Anita Glavinić, Antonela Krasic Arapovic

**Introduction:** Aorto-pulmonary fistula is a very rare type of anomalous vascular communication and mainly associated with a risk of heart failure (volume overload, pulmonary hypertension), rupture or dissection, myocardial ischemia, arterial aneurysm or endocarditis. (1-4) The presence of symptoms and evidence of a significant hemodynamic left-right shunt necessitated intervention. **Case report:** 73-year-old woman presented to hospital on routine cardiology control exam with the symptom of the progressive dyspnea on exertion over the last 5 years. The patient followed up by the cardiologist a couple of years because of history of dyspnea and fatigue. Except for hypertension, there was no significant other past medical history and all diagnostic tests which were performed to detect etiology of dyspnea was non-diagnostic. 12-led electrocardiogram showed normal sinus rhythm, rate of 72 beats per minute, normal heart axis, and diffuse non-specific T-wave abnormalities. Transthoracic echocardiography demonstrated mild pulmonary regurgitation and pathological, shunt flow, which drained into the left lateral aspect of the main pulmonary trunk. The main differential diagnosis was fistula, patent arterial duct, although other congenital arteriovenous shunts need to be excluded. A high velocity flow shunt was evident in diastole. CT coronary angiography demonstrated a communication between the aortic root and pulmonary artery. It was a well-defined fistula between the right coronary sinus and main pulmonary artery, 1 – 2 cm above the pulmonary valve. The fistula appeared separate to the right coronary ostium and exam ruled out communication with the coronary circulation. **Conclusion:** Aorto-pulmonary fistula is very rare and easily misdiagnosed. Pulmonary artery fistulas should be part of the differential diagnosis in a patient symptomatic for dyspnea. Patient with aorto-pulmonary fistula has been referred for surgical or percutaneous correction.

Mario Udovičić, Hrvoje Falak, Anđela Jurišić, Vanja Ivanović Mihajlović, Danijela Grizelj, Sandra Jakšić Jurinjak, Igor Rudež, Šime Manola

**Background**: Danon disease (DD) is a rare X-linked dominant cardioskeletal myopathy, caused by mutations in the Lysosome-Associated Membrane Protein-2 gene (LAMP-2). The X-linked inheritance causes differences in phenotypic severity between males and female (1). Classical clinical features in males include skeletal myopathy, mental retardation, and hypertrophic cardiomyopathy (HCM), while female carriers show a later onset of milder symptoms and an equal prevalence of dilated cardiomyopathy and HCM (2). **Case report**: We report a case of a female patient who first presented in 2015 at the age of 26 years with a transient ischemic attack. Initial echocardiographic assessments revealed typical images of dilated cardiomyopathy with a mildly reduced left ventricular ejection fraction at 45%, a moderately dilated left ventricle (EDD 60 mm) with normal wall thickness (11 mm). Clinically she was NYHA 2 class, her ECG showed sinus rhythm and no paroxysms of atrial fibrillation could be verified, while coronary artery disease was ruled out. Guidelines directed medical therapy was started along with warfarin, and in 2017 a CRT-D was implanted. Over the course of years, her EFLV gradually deteriorated to 25% in 2019, while the dimensions of the left ventricle remained almost the same, with development of severe symptoms (NYHA 3). Genetic analysis, using targeted next generation sequencing, showed that the patient carried a LAMP2 missense variant, c.928G > A, confirming the DD. The patient was included to the heart transplantation waiting list, and in 2020 a successful heart transplantation was performed. **Conclusion**: Although the DD is typically associated with HCM phenotype, the dilated cardiomyopathy phenotype is also prevalent in women, confirming phenotypic heterogeneity of DD, while genetic testing is essential for diagnosis.

Mirjana Isailović-Keković, Predrag Keković

**Introduction**: The aim of this study is to demonstrate unrecognized tricuspid regurgitation leading to heart failure. (1-4) We would like to point out the importance of the ultrasound examination of the heart in detecting severe tricuspid regurgitation. **Case report**: 81-years-old patient was admitted to the hospital because of the first clinical manifestation of right ventricular failure. He had acute myocardial infarction in 2009. He hasn’t seen a doctor in 14 years. On admission, he had dyspnea and bradyarrhythmia and massive pretibial edema. NT-proBNP was over 12000 pg/ml. The therapy includes a diuretic, bronchodilator, ACE inhibitor and other necessary drugs. He already has atrial fibrillation, and he is already at oral anticoagulant therapy. 12-lead ECG: dextrogram, atrial fibrillation with ventricular response around 60/beats per minute, right bundle branch block. Echocardiography: aorta normal, left atrium enlarged (**Figure 1**), diastolic dysfunction, mitral regurgitation 3-4+ (EROA 0.3 cm2 and RVol 56ml) (**Figure 2**); left ventricular ejection fraction 45%, inferior wall akinesis; right atrium and right ventricle are extremely enlarged with spontaneous echo contrast (**Figure 3**); tricuspid leaflets impaired coaptation; severe TR 4+ in two jets with SPDK=80mmHg (**Figure 4**); inferior vena cava greatly expanded (about 40mm); vena contracta 15mm.Roentgenogram of lungs and heart: bilateral pleural effusion. Abdominal ultrasound: signs of liver congestion, VCI diameter 42 mm; ascites fluid perihepatic and perisplenic. Therapy at hospital discharge: furosemide, spironolactone, direct oral anticoagulant therapy, ACE inhibitor with mandatory prophylaxis of bacterial endocarditis. FIGURE 1. Enlarged left atrium. FIGURE 2. Severe mitral regurgitation. FIGURE 3. Enlarged right atrium and right ventricle with spontaneous echo contrast. FIGURE 4. Severe tricuspid regurgitation. **Conclusion**: This case report indicates the importance of regular visits to the cardiologist, as well as the importance of timely diagnosis to prevent unwanted cardiac events.

Anja Mandrapa, Paula Radić, Vedran Carević, Tea Domjanović Škopinić, Ivona Mustapić, Darija Baković Kramarić

**Introduction**: Mitral annulus disjunction (MAD) is an abnormal insertion of the mitral annulus flexion line into the atrial wall, often described in association with Barlow’s syndrome, a myxomatous mitral valve degeneration, causing mitral valve prolapse (MVP). It is the most common cause of primary mitral regurgitation (MR) (1, 2). Both have been linked to increased arrhythmic risk and sudden cardiac death (3). **Case report**: 28-year-old patient with no previous medical history presented with palpitations, occasional shortness of breath and near loss of consciousness, especially during sport activities. Auscultatory, heart sounds were rhythmic with no murmurs except mid-systolic click. All blood tests were completely normal. Resting 12-lead electrocardiogram revealed sinus rhythm without T wave inversion or ventricular ectopic beats. Transthoracic echocardiography and transesophageal echocardiography verified borderline size and normal function of left ventricle, clear signs of MAD (**Figure 1**) and myxomatous changes of both anterior and posterior mitral valve leaflets with mild mitral regurgitation (**Figure 2**). Cardiac magnetic resonance imaging (CMR) confirmed MAD (**Figure 3**). The 24-hours Holter ECG monitoring did not confirm the presence of malignant ventricular arrhythmia. However, we decided to implant loop recorder for prolong rhythm monitoring of patient with MAD and Barlow’s disease. FIGURE 1. Transthoracic echocardiography. Four chamber view: green arrows are pointing at mitral annulus disjunction. FIGURE 2. Transesophageal echocardiography; left image 2D echocardiography and right image 3D echocardiography. Myxomatous mitral valve changes with mitral valve prolapse. FIGURE 3. Cardiac magnetic resonance imaging: white arrow is pointing at mitral annulus disjunction. **Conclusion**: Echocardiography is unavoidable method in diagnosis of mitral annulus disjunction. MAD is common finding in myxomatous valve disease and can cause symptoms of heart failure and potential life threatening ventricular arrhythmias highlighting the importance of its recognizing and treating.

Amer Iglica, Edin Begić, Indira Melezović, Alden Begić, Alen Džubur, Azra Durak-Nalbantić, Buena Aziri, Zijo Begić, Nadina Jakirlić, Fuad Zukić

**Goal:** To demonstrate an accidental verification of sinus venosus atrial septal defect (SVASD) associated with a partial anomalous venous inflow of the pulmonary veins (upper right pulmonary veins drain into the superior vena cava). **Case presentation:** The patient presented for a systematic examination, where the competent physician heard a murmur with an intensity of 2 out of 6, according to Levine, over the precordium and was referred to transthoracic echocardiography (TTE), verifying enlargement of the right ventricle (basal 4.3 cm, TAPSE 21 mm), right atrium (5.3 cm), with moderate tricuspid regurgitation and right ventricular systolic pressure (RVSP) 55 mmHg, as well as a positive bubble test (right ventricular outflow tract (RVOT) 25 mm, RVOT velocity time integral (VTI) 42.1 cm, left ventricular outflow tract (LVOT) 20 mm, LVOT VTI-Qp/Qs ratio 2.53). A TEE was performed, which revealed sinus venosus atrial septal defect (SVASD) with a diameter of 6 mm, and then computed tomography (CT) angiography of the heart, with verification of partial anomalous pulmonary venous return (PAPVR) (**Figure 1**). FIGURE 1. Sinus venosus atrial septal defect with partial anomalous pulmonary venous return. **Conclusion:** In the case of enlargement of the right cavities, it is imperative to find the cause of the same, with the gold standard in the form of TEE (1, 2), SAVSD is a rare adult congenital heart disease, and the diagnosis itself must be accompanied by the findings of computed tomography of the heart, in order to analyze the existence of vascular anomalies.

Koraljka Benko, Tamara Hlača Caput, Saša Matulić, Danijel Premuš, Ivana Grgić Romić, Ana Hrelja, Iva Žuža, Teodora Zaninović Jurjević

**Introduction:** Transthyretin amyloidosis cardiomyopathy (ATTR-CM) is a rare, progressive, life-threatening, hereditary disorder induced by a misfolded precursor protein, caused by mutations in the transthyretin gene. ATTR-CM is a challenging disease to recognize in early stages owing to its multisystem and nonspecific manifestations (1-3). **Case report:** 65-year-old patient was hospitalized for the second time to our Clinic for Cardiovascular diseases in April 2022. Previous hospitalization happened 5 years ago and there was no ambulatory cardiology check-up between hospitalizations. He had progressive dyspnea on mild exertion and lower extremity edema. Therapy at home was furosemide occasionally. In past medical history he had left carpal tunnel surgery and Covid 19 infection in April 2022. On arrival blood pressure was 140/80 mmHg, pulse 100 beats per minute, absolutely arrhythmic. Basal weakened breath sounds, and leg edema were present. Troponin and NT pro BNP were elevated (91 ng/l and 3222 ng/l). 12-lead electrocardiogram showed peripheral micro-voltage, atrial fibrillation with ventricular rate around 110 bpm. Biatrial enlargement, increased left and right wall thickness, thickened papillary muscles, mildly reduced left ventricular ejection fraction and mono-phasic transmittal flow was found on transthoracic echocardiography. Testing for Fabry disease was negative. Cardiac magnetic resonance (CMR) found morphological changes and the pattern of contrast accumulation that suggested cardiac amyloidosis. Immunofixation electrophoresis showed no monoclonal (M) spike, gammopathy was unlikely. A biopsy of the buccal mucosa was performed, no amyloid deposits were found. Bone scintigraphy found accumulation of labelled hydroxydiphosphonate (HDP) that was visible in the myocardium, which points to ATTR-CM. Genetic testing is in progress. **Conclusion:** ATTR-CM requires a high index of suspicion, and it should be suspected in patients with LV hypertrophy and heart failure. The diagnosis of cardiac amyloidosis requires a combination of multi-modality imaging including echocardiography, CMR and scintigraphy. An imaging modality that can accurately diagnose ATTR-CM without the need for invasive cardiac biopsy is nuclear scintigraphy using bone-avid radio-tracers (4). Timely diagnosis is important since the treatment is possible and improves prognosis in these patients.

Luka Mitar, David Palijan, Dubravka Šipuš, Jure Samardžić, Dora Fabijanović, Marijan Pašalić, Hrvoje Jurin, Boško Skorić, Davor Miličić, Daniel Lovrić

**Introduction**: Influenza affects millions worldwide every year. Although most cases are mild, severe complications can occur, including myocarditis (1). Extracorporeal membrane oxygenation (ECMO) is a treatment option for patients with severe respiratory and/or cardiac failure. We present a case report of a patient with influenza-induced myocarditis and subsequent heart failure treated successfully with ECMO. **Case report**: 21-years-old male with no known history of medical illness presented to the Emergency Department at University Hospital Centre with fever, cough, and shortness of breath starting three weeks earlier. Chest X-ray showed pneumonia, PCR was COVID-19 negative but influenza positive. 12-lead electrocardiogram showed diffuse ST-segment elevation, cardiac biomarkers were elevated, and echocardiography verified reduced left ventricular ejection fraction (LVEF) of 44% with pericardial effusion. Patient was diagnosed with acute myopericarditis and pneumonia, admitted to hospital and started on broad-spectrum antibiotics. Four days later patient’s respiratory distress worsened requiring intubation and mechanical ventilation. Hemodynamic status deteriorated requiring noradrenaline and dobutamine support. Bedside echocardiogram showed akinesia of inferolateral and anterolateral wall with severely reduced LVEF. Due to escalation of support and hemodynamic instability, decision was made to initiate veno-arterial (V-A) ECMO support. During the procedure patient had cardiac arrest and was successfully resuscitated two times. Two days later, patient was transported to University Hospital Centre Zagreb. Echocardiography showed LVEF of 20% while the chest X-ray showed signs of severe congestion interpreted as ECMO lung oedema. Due to that, an immediate implantation of Impella was performed. However, as soon as Impella established adequate cardiac output, a severe case of Harlequin syndrome developed which required conversion of ECMO configuration to V-A-V that stabilized the situation and enabled conversion into V-V ECMO two days later. Following further stabilization, VV ECMO was removed two days later, Impella the following day, and patient was extubated. Cardiac recuperation was dramatic and cardiac MRI showed an LVEF of 57%. Patient was discharged home after 24 days. **Conclusion**: This case highlights the appropriate use of different mechanical circulatory support modalities guided by different imaging modalities for bridging a case of severe influenza-induced myocarditis from a cardiac arrest situation to successful hospital discharge.

Ana Lanča Bastiančić, Ivana Smoljan, Sandro Brusich, Marina Klasan, Teodora Zaninović Jurjević

**Introduction**: Magnetic resonance is the most sophisticated imaging technique for morpho-functional and tissue characterisation of the heart (1). Echocardiography is the first-line imaging tool used for heart evaluation but is not sensitive enough to detect subtle myocardial fibrosis (2). Both techniques are essential for right ventricle arrhythmogenic cardiomyopathy diagnosis, which can be genetically inherited or “exercise induced”. Right ventricle wall stress during intense sports may lead to chronic remodelling and proarrhythmogenic fibrosis formation (3). **Case report**: We report a case of a 45-year-old cyclist who presented to our hospital with syncope. During a bicycle race he suddenly lost his consciousness. At that moment his smart watch recorded extremely high heart rate of around 260 bpm. He was never ill before, and he did not take any medications or supplements. After the admission to our hospital routine examinations were done. Laboratory findings showed elevated high sensitive troponin T and NT-proBNP, and 12-lead electrocardiogram was without deviation. Transthoracic echocardiography showed an aneurism of basal and mid segment of right heart lateral wall, with preserved systolic function. Coronary angiography was normal. Magnetic resonance imaging was done showing no dilatation and no fibrosis of the right ventricle. Patient underwent an electrophysiological study and ventricular tachycardia provocation. In basal conditions there was no documented tachycardia. After isoproterenol administration and programmed stimulation persistent ventricular tachycardia with left bundle branch block with inferior axis and frequency of 250 bpm was induced. A three-dimensional voltage map of the right ventricle indicated a small zone of “patchy” fibrosis of the outflow tract basal anterolateral wall. Activation map in tachycardia showed focal tachycardia pattern (microreentry) from documented region which was successfully terminated by radiofrequency ablation. **Conclusion**: Echocardiography indicated deformation of the right heart with high suspicion for fibrosis, which was confirmed by CARTO electrophysiological mapping system while magnetic resonance showed no pathological changes of right ventricle.

Amer Iglica, Edin Begić, Nirvana Šabanović-Bajramović, Buena Aziri, Zana Tafi, Zijo Begić, Azra Durak-Nalbantić, Fuad Zukić

**Goal:** To present a 24-year-old patient with a diagnosis of Trilogy of Fallot who underwent cardiac surgery. **Case presentation:** The patient has been followed for a long time under the diagnosis of pulmonary stenosis (since childhood) with verification of mild to moderate pulmonary stenosis. Four years ago, he noticed cyanosis of the lips during daily activities, and lung pathology was ruled out. He presents for a consultative opinion, where transthoracic echocardiography is performed, which verifies the right atrium with a diameter of 4.5 cm, the right ventricle with regular dimensions (tricuspid annular plane systolic excursion (TAPSE) 21 mm, fractional area change (FAC) 34%, free wall thickness 17 mm), inferior vena cava 2.2 cm, and respiratory collapse more than 50%. Severe pulmonary stenosis is verified (peak pulmonary valve velocity (Vmax) 6 m/s, peak gradient 85 mmHg) with mild pulmonary regurgitation. The systemic flow ratio (Qp/Qs) ratio was 0.98. The bubble test demonstrated the movement of microbubbles from the right to the left atrium, and the transesophageal echocardiography verifies the existence of a type secundum ASD measuring 1.7 cm (**Figure 1**). Cardiac computerized tomography (CT) with contrast is performed. Analysis of the area of ​​the right ventricular outflow tract (RVOT) shows that it is more slender so that at a distance of 12 mm distal to the pulmonary valve, the diameter is 11 mm, and at the level of the pulmonary valve 17 mm (Figure 1). Truncus pulmonalis diameter is 27 mm, the right pulmonary artery is 20 mm, and the left 19 mm. There are no definite CT signs of persistent ductus arteriosus, and slightly more prominent bronchial arteries, especially the upper right (diameter 5 mm). The patient presents himself to the cardiac surgery council and is accepted for cardiac surgical treatment. FIGURE 1. Display of atrial septal defect and dimensions of the right ventricular outflow tract. **Conclusion:** Follow-up of pulmonary stenosis in the pediatric population should also be performed with transesophageal echocardiography in order to evaluate the interatrial septum. (1, 2) Catheterization of the right heart with assessment of pressure in the pulmonary circulation forms the basis for cardiosurgical treatment.

Siniša Roginić, Tereza Knaflec, Vito Mustapić, Martina Roginić, Mladen Predrijevac, Krešimir Štambuk, Marija Čajko, Nikolina Mijač Mikačić, Domagoj Futivić

**Introduction**: Ventricular septal defect (VSD) is severe but fortunately rare mechanical complication of myocardial infarction with high mortality: 30-40% (1), ranging up to 87% if associated with cardiogenic shock (2). It has been reported to occur more frequently in the anterior than inferior/lateral wall infarction (70% versus 29%), but inferior infarcts are associated with complex VSDs a worse prognosis. Therapeutic options include medical management, surgical and transcatheter repair with variable success (1). **Case report**: 58-year-old male patient, smoker with hypertension presented with signs and symptoms of heart failure lasting for 3 days. He did not have chest pain, but 12-lead electrocardiogram showed Q waves in inferior leads with clearly positive troponin (hs trponin I 578.5 ng/l). Physical examination corresponded to Killip class II and peculiar holosystolic precordial murmur was noted which prompted urgent bedside echocardiography. Left ventricle was mildly dilated with akinesia of basal and mid segments of inferoposterior wall. Ejection fraction was estimated 55%, there was mild mitral regurgitation and moderate secondary tricuspid regurgitation due to pulmonary hypertension. Atypical parasternal and subcostal projections revealed large VSD located in inferior part of septum (approximate diameter 3 cm) with massive left to right shunt. Patient was immediately transferred to clinical institution with cardiac surgery capacity. Angiography revealed two-vessel disease, including thrombotic subocclusion of right coronary artery mid segment. During angiography his status has worsened further with development of cardiogenic shock despite pharmacological and intraaortic balloon pump support so he underwent urgent operation. Large septal defect was reconstructed combining pericardium with pledgets and double bypass was created (VSM-LAD, RCA). After releasing aortic clamp and restoring circulation there was rupture of myocardial wall next to suture; repeated attempts to stabilize patient failed and he soon died of shock and the multiple organ failure (Figure 1). FIGURE 1. A) Atypical parasternal projection showing large defect of basal inferoseptum. B) Massive turbulent left to right shunt. C) Remodeled left ventricle with inferior wall akinesia (A2C projection). D) Subcostal view of septal defect and corresponding shunt (E). F) CW Doppler of high velocity systolic flow through the defect. **Conclusion**: Mechanical complications of myocardial infarction are infrequently seen in the era of interventional cardiology (around 1/1000 STEMI patients) but bear significant morbidity and mortality despite early diagnosis. Urgent echocardiography is essential in acute coronary syndrome with signs of heart failure, especially if accompanied with new onset heart murmur.

Dijana Trninić

**Background:** Significant coagulopathy and hyperinflamation are found in patients with coronavirus disease 2019 (COVID-19). **Case report:** 44-year-old male patient was admitted to the Infective Clinic with a severe form of pneumonia COVID-19.The disease is complicated, with sub-segmental embolization of the lungs and pneumothorax, which are confirmed with laboratory and computed tomography (CT) lung with angiography. On the third day of hospitalization, an echocardiogram is performed, which shows global hypocontractility of the left ventricular walls with a reduced ejection fraction EF 45%. The right ventricle was borderline. After the applied therapy, the patient’s condition improves and he is discharged home with anticoagulant therapy. An echocardiography (**Figure 1**) performed as part of a cardiology examination one months after discharge from the hospital diagnosed a mass in the right atrium (35x27mm), which could correspond to a thrombus. Calcifications were visualized in the mass. The patient is readmitted to the Cardiology Clinic and intravenous heparin is indicated. Perform transesophageal echocardiography (**Figure 2**) and confirm the presence of a mass consistent with thrombus. Cardiac CT (**Figure 3**) shows mass in right atrium extending into the inferior vena cava, and CT of the abdomen shows thrombosis of the inferior vena cava. Cardiac magnetic resonance (**Figure 4**) showed a mass in the right atrium, which, corresponds to a thrombus adherent to the interatrial septum. After application of contrast, focal zones of higher intensity are visualized, zones of fibrosis on interventricular septum, anterior l and inferior and lateral wall of subepicardial and intramyocardial localization, which correspond to the sequelae of the inflammatory process, with ejection fraction 45%. The right ventricle has normal dimensions. After unsuccessful therapy with unfractionated heparin, the thrombus was successfully surgically extracted. FIGURE 1. Two-dimensional echocardiogram apical 4-chamber view showing a mass in the right atrium. FIGURE 2. Transesophageal echocardiography showing a mass in the right atrium. FIGURE 3. Cardiac computed tomography confirmed mass (thrombus) in the right atrium extending into the inferior vena cava. FIGURE 4. Cardiac magnetic resonance showed a mass in the right atrium, which corresponded to a thrombus adherent to the interatrial septum. **Conclusions:** Cardiac imaging modalities, including transthoracic or transesophageal echocardiography, cardiac magnetic resonance, cardiac computed tomography, and 18Ffluorodeoxyglucose positron emission tomography have a complementary and reinforcing role for the evaluation of cardiac masses.

Anamarija Plazonić, Krešimir Štambuk

**Introduction**: Commissural mitral valve prolapse is an infrequent form mitral valve prolapse (1) and it presents a diagnostic challenge for sonographers (2). However, with new tools such as 3D echocardiography it has become easier to define the proper mechanism of mitral valve regurgitation which is important for preoperative planning of surgical mitral valve repair. **Case report**: We present a case of a 65-years-old man with a history of arterial hypertension and a new onset of progressive exertional dyspnea and holosystolic murmur in clinical examination. A transthoracic 2D dimensional echocardiography showed severe eccentric mitral regurgitation due to prolapse of posterior mitral leaflet, as is initially described. As exact mechanism of mitral regurgitation was not clear we did 3D transesophageal echocardiography study, and it showed a posteromedial commissure and P3 scallop prolapse with chordal rupture (**Figure 1**) as the most likely mechanism of severe mitral regurgitation. Coronary angiography is performed, and it shows ectatic atherosclerotic coronary arteries with no significant stenosis and the patient is referred to Heart Team and is accepted for mitral valve repair. FIGURE 1. 3D echocardiography image shows the mitral valve with posteromedial commissural and P3 scallop prolapse with chordal rupture. **Conclusion**: 3D sonography could have a crucial role in defining the mitral valve mechanism, especially in cases of commissural prolapse as we showed in this case. It is important for planning treatment options especially in case of surgical mitral valve repair.

Vesna Pehar Pejčinović, Iva Uravić Bursać, Dijana Travica Samsa, Marijana Rakić, Damir Raljević, Viktor Peršić

Mitral annular disjunction (MAD) is the improper systolic acceptance of the posterior leaflet to the atrial wall (1). First line to the diagnosis is transthoracic echocardiography in the long axis view on the mitral valve using highest frame rate or sagittal view on cardiac magnetic resonance (CMR) (1). The distance between mitral-annulus and systolic bulge of ventricular myocardium range 5-10 mm is diagnostic and it is associated with morphological and functional remodeling of the left ventricular myocardium (1, 2). Prevalence of MAD varies due to the different cut-offs, imaging modalities and ec, and in a general population, is 8.7% (3). In patients with mitral valve prolapse the prevalence of MAD is 20-58% (1). While MAD with prolapse is common and associated with ventricular arrhythmias, isolated MAD without prolapse, has been described on CMR (2, 3). CMR can also identify prolapse using cine images, but the most important advantage of this technique is risk stratification of arrhythmias due to incremental prognostic value of late gadolinium enhancement over mitral valve prolapse severity (2). Several studies suggested association between late gadolinium enhancement (LGE) at the mid wall of papillary muscles and inferobasal region of left ventricle with complex arrhythmias (1-4). The origin of ventricular arrhythmia can be deduced according to the distribution of LGE on CMR (1, 2). CMR should be done in all patients who survived sudden cardiac death or sustained ventricular arrhythmia before devices, to clarify the etiology and also in patients with unexplained syncope or nonsustained ventricular arrhythmia, to assessment of left ventricle size and function, severity of mitral regurgitation, leaflet thickness and also when is poor echocardiographic window (1). CMR imaging provides excellent morphological information and helps in the assessment of fibrosis (4). Myocardial fibrosis determined according to LGE at CMR was associated with adverse outcome in patients with mitral valve prolapse without moderate-to-severe mitral regurgitation or left ventricular dysfunction (2).

Jelena Jovanić, Dijana Trninić, Miron Marjanović, Siniša Kovačević, Vojislav Vukašinović, Saša Lončar, Bojana Ilić, Nikolina Bosančić, Bogdana Menićanin

**Introduction:** Assessing the severity of tricuspid regurgitation (TR) remains a challenging task, and although echocardiography is the test of choice, significant limitations of the current recommendations exist (1). Recently updated guidelines from the American Society of Echocardiography suggest cardiac magnetic resonance imaging and computed tomography angiography may play a significant role. (2) Even more challenging is the quantification of TR following surgical or transcatheter repair devices. **Case report:** 52-year-old man was hospitalized in our institution, previously in a regional hospital a 24-hours Holter ECG and echocardiography were performed. At admission he complains of feeling fainting. He was treated for hypertension. At exam feeling well, eupneic, rhythmic heart action, clear tones, no noise. On repeated Holter ECG: atrial flutter with an average frequency of 56/min. Transthoracic echocardiography in our institution described: left ventricular with normal dimensions and ejection fraction. The right atrium markedly enlarged (RA area 45.7 cm2), the interatrial septum is directed to the left, the right ventricle also enlarged, with normal wall thickness. Tricuspid annulus dilated with severe tricuspid regurgitation (TR 4+, jet fills 2/3 of RA surface, TR ERO 1.3 cm2, TR RV 108 ml). The pulmonary artery normal. Cardiac magnetic resonance confirmed severe tricuspid insufficiency with no fat infiltration, and no fibrosis at late gadolinium enhancement sequences. Arrhythmogenic cardiomyopathy was excluded. Right heart catheterization was performed, right atrium 15/5/10 mmHg, right ventricle 35/7/15 mmHg, pulmonary artery 31/15/20 mmHg, mean pulmonary capillary wedge 11 mmHg, transpulmonary gradient 9 mmHg. On coronary angiography, coronary artery without narrowing, slow flow through the left anterior descending and right coronary artery. At proposal of pulmonologist, computed tomography of the chest and ventilation perfusion lung scintigraphy were performed. All findings were presented to the cardiological-cardiosurgical council, which indicated tricuspid valve surgery. **Conclusion:** Transthoracic echocardiography remains the first-line imaging modality in the guidelines as well as in clinical practice. Greater use of advanced imaging technology and techniques may improve the ability to accurately and reproducibly quantify this disease.

Anja Mandrapa, Ivona Mustapić, Paula Radić, Tea Domjanović Škopinić, Vedran Carević, Darija Baković Kramarić

**Introduction**: Ostium primum atrial septal defect (ASD) is a congenital cardiac malformation involving atrial septum contiguous with atrioventricular valve annulus, often resulting in associated atrioventricular valves malformations. It is typically repaired in the first years of life (1, 2). **Case report**: 32-year-old female asked for cardiology examination after she started to feel chest pain and dyspnea during mild activity. We found out that she is a child from an identical twin pregnancy and has been knowing for heart murmur since childhood. Except Arnold-Chiari malformation type 1, she had no other medical history. On transthoracic echocardiography (TTE) we found normal left ventricular size and function, enlarged right ventricle and significant primum ASD with possible cleft mitral valve leaflet (CMVL) as a cause of mitral regurgitation (MR) (**Figure 1**). Due to additional evaluation transesophageal echocardiography (TEE) was done and ASD was measured about 22-24 mm long with Qp:Qs ratio of 1,9 and a cleft of posterior mitral valve leaflet was confirmed (**Figure 2** and **Figure 3**). Right heart catheterization showed mildly increased pressures in pulmonary circulation, increased cardiac output with sinus tachycardia and significant left-to-right shunt. She was referred to a cardiac surgeon and operated; CMVL was repaired and ASD was closed with an autologous pericardial patch. Postoperative recovery was doing well and control TTE shows mild mitral regurgitation and no signs of shunt over good positioned interatrial patch. FIGURE 1. Parasternal short axis view. Mitral valve; white arrow is pointing at the cleft of the posterior mitral valve leaflet. FIGURE 2. Transesophageal 2D echocardiography. Ostium primum atrial septal defect. FIGURE 3. Transesophageal 3D echocardiography. Ostium primum atrial septal defect and cleft of posterior mitral valve leaflet. **Conclusion**: The diagnoses of ostium primum atrial septal defect and cleft mitral valve can easily and successfully be made by echocardiography. The long-term results with this congenital anomalies repair are excellent.

Paula Radić, Anja Mandrapa, Vedran Carević, Tea Domjanović Škopinić, Ivona Mustapić, Darija Baković Kramarić

**Introduction:** Hypertrophic obstructive cardiomyopathy is the most common cardiac disease with genetic predisposition (1). We are presenting the case of a 57-year-old patient with broad, non-specific symptoms that was later diagnosed with hypertrophic obstructive cardiomyopathy. **Case report:** 57-year-old male presented to the Emergency Department after collapsing on the airport while waiting in line. He felt epigastric pain and constant exhaustion prior the event which he connected with Barrett’s esophagus, diagnosis he was previously diagnosed with. He felt palpitations and measured low blood pressure even though he was diagnosed with arterial hypertension. During medical history taking he stated he was the only family member living past 55 years of age since his siblings and father died due to heart condition with whom he was not familiar with. Auscultation revealed grade 3/6 systolic murmur over the heart apex. His blood work showed elevated N-terminal pro b-type natriuretic peptide (2054 pg/mL) and insignificantly elevated high sensitive troponin T. 12-lead electrocardiogram showed signs of left ventricle hypertrophy. Routine echocardiography visualized obstructive hypertrophic cardiomyopathy with high mean gradient in left ventricular outflow tract (LVOT mean PG 111.52 mmHg) (**Figure 1**), systolic anterior motion (SAM) (**Figure 2**) and severely thickened basal septum (IVSd 2.2 cm) (**Figure 3**. During his stay invasive coronary angiography was performed and significant coronary atherosclerosis was ruled out. A 24-hour heart monitor device was used with whom we ruled out rhythm disturbances. After clinical stabilization patient was referred to further medical evaluation in his country. FIGURE 1. Doppler flow in left ventricular outflow tract. FIGURE 2. Systolic anterior motion. FIGURE 3. Interventricular septum hypertrophy. **Conclusion:** Hypertrophic obstructive cardiomyopathy is not always a first diagnose that comes to our mind when patient presents with exhaustion and lightheadedness. Since it is a significant sudden cardiac death cause in younger people and often diagnosed after the fatal event we should think about it more often, even more since we have such a powerful and easily obtained diagnostic procedure like echocardiography.

Marija Brestovac, Blanka Glavaš Konja, Martina Lovrić Benčić, Vlatka Rešković Lukšić, Sandra Jakšić Jurinjak, Joško Bulum, Zvonimir Ostojić, Kristina Gašparović, Jadranka Šeparović Hanževački

**Introduction**: Cardiac resynchronization therapy (CRT) contributes to left ventricle ejection fraction and NYHA improvement in patients with severe heart failure. In literature, echocardiographic (ECHO) benefits of CRT were found in a wide spectrum of different ECHO parameters. (1-4) In this study we compared ECHO benefit of echocardiographic optimization of CRT device with the standard, ECG method in a spectrum of different ECHO parameters. **Patients and Methods**: An overall of 146 patients were analyzed according to the method of CRT optimization. The first group (OPT) was optimized by ECHO signs of dyssynchrony whereas the second group (ECG) was analyzed using QRS duration. Changes in QRS duration, NYHA, left ventricle end-systolic volume (ΔESV), end-diastolic volumen (ΔEDV), ejection fraction (ΔEF), global longitudinal strain (GLS), mean pulmonary artery pressure (PAP), mitral regurgitation (MR), ΔNTproBNP, septal flash (SF), atrioventricular dyssynchrony (AVd), interventricular dyssynchrony using difference in onset of pulmonary and aortic ejection (PV/AV) and quality of life using SF-36 questionnaire were compared between the groups in a follow up period of 6 months. **Results**: No additional benefit of one group over the other was found for QRS duration reduction (p=0.366), NYHA (p=0.221), ΔESV (p=0.093), ΔEF (p=0.218), GLS (p=0.665), MR (p=0.278), PAP (p=0.433) and QoL (p=0.213) whereas a faster and higher reduction in ΔEDV (p=0.045), ΔNTproBNP (p=0.037), SF (p=0.0014), AVd (p=0.002), PV/AV (p=0.041) was found in OPT group favoring ECHO optimization. **Conclusion**: Echocardiographic optimization of CRT contributes to an additional echocardiographic benefit over the standard method in reduction of NTproBNP, ΔEDV and SF, AVd and PV/AV.

Ana Marija Slišković, Vlatka Rešković Lukšić, Sandra Jakšić Jurinjak, Marija Brestovac, Jozefina Palić, Martina Lovrić Benčić, Jadranka Šeparović Hanževački

**Introduction:** Moderate and severe tricuspid regurgitation (TR) affects about 4% of people older than 75 years. Depending on etiology, it can be divided into primary, secondary (observed in more than 90% of cases) and TR due to pacemaker lead implantation (1, 2). Although severe TR exhibits high mortality, long-term cardiovascular risk in specific subgroups remains unknown. Purpose of this pilot study was to evaluate differences in survival among population with significant TR and variable clinical risk profiles. **Patients and Methods:** Single center retrospective study was conducted. Consecutive patients referred to UHC Zagreb for echocardiography, with moderate or severe tricuspid regurgitation diagnosed on initial exam in the period from 2011 to 2017 were included (**Figure 1**). Demographic characteristics, echocardiography findings and clinical outcomes were analyzed. Patients were categorized by TR etiology, left ventricular ejection fraction (LVEF), pulmonary artery hypertension, TAPSE (tricuspid annular plane systolic excursion), and tricuspid valve surgery (**Figure 2**). FIGURE 1. Severe tricuspid regurgitation. FIGURE 2. Specific subgroups of patients with tricuspid regurgitation. **Results:** 50 patients were enrolled (mean age 71.68±13.45 years, 62% female). There was no significant difference in cardiovascular (CV) mortality regarding TR etiology and LVEF. However, significant correlations between CV mortality and pulmonary artery hypertension (PAH) (p=0.039), TAPSE (p=0.049) and in TAPSE <17 mm (p=0.015) were observed. Besides, odds for CV mortality were 4 time greater in patients with TAPSE <17 mm (OR 4.3, 95% CI:1.3-14.5; **Figure 3**) and 5 times greater in patients to whom TV surgery was not performed during 6-years follow-up period (OR 5.3, 95% CI: 1.5-18.3; **Figure 4**). FIGURE 3. Differences in survival between patients with tricuspid annular plane systolic excursion, cut-off value 17 mm. FIGURE 4. Differences in survival between patients with and without tricuspid valve surgery. **Conclusion:** We found that pulmonary hypertension, right ventricular disfunction and lack of tricuspid valve intervention are associated with higher mortality. Appropriate timing for TV surgery, especially in patients referred for left-sided valve surgery could improve patient outcomes.

Jadranka Šeparović Hanževački, Viktor Peršić

CroEcho 2023 is organized by the Working Group on Echocardiography and Cardiac Imaging Modalities of the Croatian Cardiac Society and is endorsed by the European Association of Cardiovascular Imaging (EACVI), European Society of Cardiology. It is a three-day congress aimed at offering content for cardiologists and cardiology fellows dedicated to echocardiography and clinical decision-making. CroEcho 2023 addresses the essentials of the best practices in imaging and clinical contexts, with focus on interesting cardiology topics from the area of clinical decision-making where echocardiography and imaging plays a central role. It features key presentations from international expert speakers, plenary sessions covering clinical dilemmas, and advanced imaging in different clinical scenarios focusing on valvular heart diseases and interventions. The program is composed of plenary lectures, scientific oral presentations, debates, a teaching course, and interactive educational content (imaging workshops, practical work with a mentor, virtual practice on simulator), as well as a live anatomical-echocardiographic heart section. Come and join us, share your knowledge and experience, and learn in an entertaining and enjoyable way. Attend interactive workshops, hands-on sessions, and a basic teaching course, joining together beginners and experts. April 21-24, 2023 Hotel Parentium, Poreč, Croatia Predsjednici kongresa: / Congress Directors: Jadranka Šeparović Hanževački and Viktor Peršić

Petar Martinčić, Sandra Jakšić Jurinjak, Vlatka Rešković Lukšić, Marija Brestovac, Joško Bulum, Zvonimir Ostojić, Blanka Glavaš Konja, Martina Lovrić Benčić, Jadranka Šeparović Hanževački

**Introduction:** Right ventricular dysfunction (RVD) and tricuspid regurgitation (TR) are associated with poor outcomes in multiple valve disease (1). Degenerative valve disease mainly affects aortic and mitral valves (2). In case of significant aortic stenosis (AS) combined with the stenotic mitral disease, transmitral gradient (TMG) might be underestimated due to pressure load and assessment is more challenging. Less is known about the clinical impact of combined degenerative stenotic aorticomitral disease and RVD and TR. This pilot study aimed to evaluate the effect of combined aorticomitral degenerative disease and tricuspid regurgitation and right ventricular function on risk of hospitalization due to cardiovascular cause over four years follow-up and to evaluate potential prognostic value of the echocardiographic parameter for risk stratification in multivalvular disease. **Patients and Methods:** We retrospectively analyzed 39 eligible patients, admitted due to aorticomitral stenotic disease, with elevated transmitral gradient (TMG) defined as TMG >4 mmHg and mitral annulus calcification through 2017 and 2018. During the four-year follow-up, we examined the causes of hospitalization and mortality. Baseline demographic and echocardiographic data were extracted from the electronic medical record and analyzed from a digital database. **Results:** In total, 39 patient data were reviewed (average age 71.64±SD 9.39; 71% female), mean SVi 41.49±12.46 ml/m2, mean LVEF 61.28±9.8%, mean MV MPG (TMG) 5.92±1.92 mmHg, mean MVA 1.74±0.4 cm2, mean AVAi 0.81±0.36cm2/m2, mean TAPSE 16.23±5.1mm, mean RVFWS -18.43±6.5%, mean TR RV 13.33±12.4 ml, mean RVSP 33.01±15.24 mmHg. During four years of follow-up, the mean number of hospitalizations was 2.51±1.9 and the four-year survival rate was 79%. Paired quantile groups of mean RVFWS of -14.38±3.1% and mean RVFWS -24.9±4.9% were compared. The number of hospitalizations differed between groups, 2.88 ±1.3 and 2.14±1.4 respectively (p<0.0328). There was no difference in hospitalization between groups observed in TAPSE, TR or RVSP. Patients with RVD (RVFWS -18.4%) and significant stenotic aorticomitral disease had more hospitalizations due to cardiovascular causes. **Conclusion:** RVD increases the risk of hospitalizations, while RVFWS may serve as echocardiographic indicator of RVD in hemodynamically significant left side valvular disease and add value in risk stratification in multiple valve disease in this case aorticomitral stenotic disease.

Tereza Knaflec, Siniša Roginić, Ivan Jakšić, Martina Roginić, Marija Čajko, Nikolina Mijač Mikačić, Domagoj Futivić

Aim: To show a case of systemic wild-type transthyretin amyloidosis (wtATTR) combined with valvular and ischemic cardiomyopathy. Case report: 78-year-old man presented with worsening of congestive chronic heart failure. Medical history includes arterial hypertension, stage 3b chronic kidney disease, coronary artery disease, hypothyroidism, syndrome Raynoud (**Figure 1**) and implantation of ICD in secondary prevention. The electrocardiogram showed atrial fibrillation and right bundle branch block. Transthoracic echocardiography (**Figure 2****,****Figure 3**) showed reduced left ventricle ejection fraction, biventricular wall thickening (1), global and segmental hypokinesia of inferior wall and basal inferoseptum, decreased global longitudinal systolic function; moderate calcified aortic stenosis (2); biatrial enlargement, moderate mitral and severe secondary tricuspid regurgitation. Based on the absence of monoclonal protein, cardiac scintigraphy was performed with injection of technetium-based compound which confirmed the diagnosis of amyloid transthyretin cardiomyopathy (3) (**Figure 4 A and B**). Furthermore, recoronarography excluded progression of coronary artery disease. Peripheral polyneuropathy consistent with amyloidosis was also diagnosed. Guideline based heart failure management resulted in rapid recovery and after discharge patient was ambulatory (NYHA III). He was adherent to therapy without side-effects typical for cardiac amyloidosis (hypotension, etc.) probably due to combined aetiology of cardiomyopathy. Tafamidis (4) is the only medication approved for the treatment of wtATTR cardiomyopathy, slowing the dissociation of transthyretin and further progression of the disease, reducing all-cause mortality and cardiovascular-related hospitalizations compared to placebo. Unfortunately it is not indicated in advanced heart failure present in our patient. FIGURE 1. Raynaud’s syndrome; pallor 5th finger of the left hand and cyanotic hands. FIGURE 2. Parasternal long axis view: small left ventricle cavity, left atrial enlargement, biventricular wall thickening. FIGURE 3. Speckle tracking echocardiography: severely reduced global longitudinal strain (-9.0%) with an “apical sparing”/”cherry on the top” pattern. FIGURE 4. Cardiac scintigraphy and SPECT/CT; A. Static scintygraphy images showing heart/contralateral tracer uptake ratio >1.5. B. SPECT/CT confirmation that tracer is accumulating in myocardium.

Marija Brestovac, Ivana Sopek Merkaš, Vlatka Rešković Lukšić, Sandra Jakšić Jurinjak, Blanka Glavaš Konja, Martina Lovrić Benčić, Anica Milinković, Antonio Hanžek, Antonio Marić, Dominik Piršljin, Ana Čala, Jadranka Šeparović Hanževački

**Introduction:** Chronic aortic regurgitation (AR) results in left ventricular (LV) volume overload, leading to compensatory changes such as LV dilatation and hypertrophy. These adaptive mechanisms enable patients with severe AR to tolerate the increased blood volume for an extended period, even after the LV becomes enlarged and its function is reduced. In recent studies, aortic valve surgery has been shown to improve LV volumes. LV dimension can be used as a predictor of impaired left ventricular functional and structural recovery during follow-up after surgery. Furthermore, severe AR patients often present with coexisting tricuspid regurgitation (TR) and combined have a higher risk of adverse outcomes. (1, 2) The aim of this study was to explore the changes in LV end-diastolic volume (EDV), LV end-systolic volume (ESV), mean pulmonary artery pressure (PAP) and TR in patients with severe aortic regurgitation who underwent surgical treatment at the University Hospital Centre Zagreb. **Patients and Methods:** In this study 45 patients (87% male, 13% female) with severe AR that underwent aortic valve surgery were included. The average age was 54.8 year, and the average follow-up time was 38 months. The change in EF (%), EDV (ml), ESV (ml), PAP (mmHg) and TR was compared before and after aortic valve surgery. **Results:** The results show a statistically significant reduction in EDV (194.46± 80.51 vs. 142.55±56.94, p<0.001) and ESV (96.35±52.45 vs. 75.58±45.44, p<0.001) after AV surgery and change in pulmonary artery pressure (32.14 vs. 23.18). No significant differences were found in EF (53.26±10.92 vs. 52.40±12.53, p=0.612) or the degree of TR (p=0.785). The degree of TR was graded on a scale of 1-5. Prior to surgery, 13 patients (29%) had no TR (grade 0), 29 patients (64%) had grade 1 TR, 1 patient (2%) had grade 3 TR, and 2 patients (4%) had grade 4 TR, and none of the patients required surgical repair. Postoperatively, 12 patients (27%) had no TR (grade 0), 30 patients (67%) had grade 1(mild) TR, 2 patients (4%) had grade 2 (mild to moderate) TR, and 1 patient (2%) had grade 3 (moderate) TR. **Conclusion:** This study confirmed that EDV and ESV improved after surgery, as predictors of impaired LV functional and structural recovery. After successful AV surgery, mild TR does not worsen when there is no elevated PAP. However, the impact of TR on the outcomes of these patients requires further research in this area with larger and longer-term follow-up studies.

Iva Ladić, Renata Ivanac Janković, Ivana Petrović Juren, Gabriela Bašković, Matea Lukaš

Young female patient aged 42 who has been in intermittent hemodialysis program via Tesio catheter for 11 years following bilateral nephrectomy performed because of renal carcinoma, has been recently diagnosed with catheter sepsis (Enterococcus faecalis sepsis). Transthoracic echocardiography was performed and small left coronary aortic cusp vegetation on aortic side of the valve has been visualized (3x3 mm in size), with no signs of valve disfunction. Patient has been treated with vancomycin, without permanent catheter extraction since no other vascular access for hemodialysis was available and sepsis was very well medically controlled. Nevertheless, coincidentally, rare anomaly of tricuspid valve has been also demonstrated. One single, dominant hypertrophied, abnormal placed papillary muscle has been visualized having root attachment in the apex of the right ventricle with relatively short chordae tendineae (**Figure 1**, **Figure 2**). Only mild tricuspid regurgitation has been verified, with no sings od tricuspid stenosis. Right ventricle function and size seemed to be normal. These characteristic morphological features will likely present the case of a parachute tricuspid valve anomaly. Also, interestingly, significantly hypertrophied papillary muscles in small, hypertrophied left ventricle have been shown. Magnetic resonance of the heart was also performed, showing normal sized and shaped right ventricle (EDV 110 ml), having normal systolic function (EF 66%), with no signs of other congenital heart defects or abnormal masses in the cavity of the right ventricle. Parachute tricuspid valve anomaly is very rare congenital heart disease that has not yet been thoroughly investigated. (1-3) It is presented with one single dominant papillary muscle in the right ventricle, resulting in tricuspid valve regurgitation, stenosis or uncommonly, like in our case, normally functioning valve. FIGURE 1. Transthoracic two-dimensional right ventricular inflow view showing one single hypertrophied papillary muscle. FIGURE 2. Transthoracic two-dimensional apical 4-chamber view showing one papillary muscle.

Jasmina Ćatić, Ivana Jurin, Stanislav Glumbić

**Introduction**: Giant left atrium is a rare condition, with a reported incidence of 0.3%, and following mainly rheumatic mitral valve disease with mitral regurgitation being more prominent than mitral stenosis (1). It is believed that chronic volume and pressure overload created by mitral valve disease, in addition to weakening of left atrial wall because of rheumatic pancarditis, is responsible for the excessive enlargement of the left atrium (2). **Case report**: 62-year-old female patient presented complaining of dysphagia and dyspnea. She was diagnosed with severe mitral stenosis and mild regurgitation 10 years before admission to our hospital but refused cardiac surgery. Clinically, the patient had features of congestive cardiac failure. Chest X-ray revealed asymmetrical cardiomegaly with the right heart border extending to the right lateral chest wall. Echocardiography demonstrated giant left atrium with 11.2 x11.5 cm diameter and LAVI was 440 ml/m2 (**Figure 1**). ECG revealed atrial fibrillation which is always present in giant left atrium. It was interesting that there was no formatted thrombus in the giant left atrium although the patient did not take recommended oral anticoagulation therapy. Patient also had cardiac cachexia and inappetence due to dysphagia due to esophageal compression. Patient again refused cardiac surgery treatment. She died 6 months after due to heart failure. FIGURE 1. Giant left atrium due to severe mitral stenosis. **Conclusion**: Nowadays, giant left atrium is rarely seen due to decreasing incidence of rheumatic heart disease. Dilatation of the left atrium in our case was the left atrial compensation mechanism due to the chronic pressure overload in mitral stenosis, to balance pulmonary capillary wedge pressure. To the best of our knowledge, this is the only case reported in the recent literature of the giant left atrium due to severe mitral stenosis and without formatted thrombus.

Nikolina Jupek, Antun Car

**Introduction**: Hemolytic anemia is well-known, but rare complication of mitral valve repair. The mechanism of intravascular hemolysis in patients with mitral regurgitation after mitral valve surgery is due to high shear stress of the erythrocytes caused by the regurgitation flow. (1) **Case report**: We report a case of a 74-year-old female who presented with post-operative hemolytic anemia after mitral valve repair with a biological prosthesis. The patient’s medical history was significant for bilateral mastectomy (breast carcinoma, radiation therapy), congestive heart failure, permanent atrial fibrillation, implantation of the biological mitral valve, aortic valve insufficiency and hyperthyroidism. Short after surgery, laboratory studies showed a significantly decreased hemoglobin (83 g/L), elevated levels of lactate dehydrogenase (1540 U/L) and bilirubin (45.5 μmol/L). The patient received multiple blood transfusions. Direct Coombs testing and DAT were negative. The findings were suggestive of hemolytic anemia. A transthoracic echocardiogram revealed a normal ejection fraction, mitral valve annuloplasty with regurgitation and tricuspid valve with regurgitation. The closure of the paravalvular leak on the biological mitral valve prosthesis and annuloplasty of the tricuspid valve was done. After reoperation, the patient’s symptoms gradually resolved and her hemoglobin, lactate dehydrogenase and bilirubin levels normalized. After the procedure, the patient is transferred to the Intensive Care Unit. On the first postoperative day, she is weaned from the respirator and extubated 14 hours after admission. The patient enters a total AV block rhythm further guided by a temporary pacemaker. On electrocardiogram, sinus rhythm gradually recovered, and no permanent pacemaker was indicated. The patient was discharged on day 30 postoperatively in good condition. **Conclusion**: Following mitral valve repair, clinically significant hemolysis is highly uncommon. The optimal treatment remains surgical repair or replacement because hemolysis rarely resolves spontaneously.

Krešimir Šutalo, Ana Šutalo, Renato Filjar

**Introduction:** Assessment of left atrial size and function is a routine part of any echocardiographic examination. These parameters reflect volume and/or pressure load, pathology of the left ventricle, arrhythmias, storage diseases, and congenital heart defects (1). The presented research aims to evaluate the significance of the atrial stiffness index (ASI) and left atrial strain (LAS) in assessing the function and structure of the left atrium. **Patients and Methods:** The analysis was performed using statistical methods applied on the observations of 57 male individuals split into three groups: G1 (22 healthy individuals), G2 (25 patients with arterial hypertension), and G3 (10 patients with heart failure with reduced ejection fraction). All subjects were in sinus rhythm and without a significant degree of calcification of the mitral annulus. Patients underwent classical echocardiographic examination along with 2D speckle tracking analysis.Variable observations related to group pairs (G1-G2, G1-G3, G2-G3) were statistically analyzed using t-test for differences of means and F-test for analysis of variance. **Results:** Statistically significant difference (p<0.05) between means was found for all group pairs for ASI and reservoir LAS, while latter showed p<0.05 for difference between variance as well. Median ASI value was 0.28 (interquartile range (IQR) 0.2-0.31), 0.44 (IQR 0.35-0.7), and 1.66 (IQR 0.91-2.77) for G1, G2 and G3 respectively, measured in mmHg/ml. Median reservoir LAS value (average of two and four chamber views) was 29.75% (IQR 27.87-33.73), 24.13% (IQR 20.32-27.57), and 14.39% (IQR 9.12-18.24) for G1, G2 and G3 respectively. **Conclusion**: ASI and reservoir LAS showed significant difference in patient subgroups. Taking that into account, it is reasonable to include them in everyday echocardiographic practice for diagnosis both clinical and subclinical cardiac dysfunction.

Petra Zebić Mihić, Grgur Dulić, Dijana Dumančić, Iva Jurić, Matija Drinković, Anto Stažić, Sandra Šarić

**Introduction**: Primary cardiac tumors are approximately 20 times rarer than nonprimary cardiac tumors. Cardiac papillary fibroelastomas are the 2nd most common type of cardiac tumors, with greater frequency only by myxomas. (1) The clinical manifestations of this entity are not well described. Cardiac papillary fibroelastomas are associated with substantial complications that are secondary to systemic embolism. Based on studies cardiac papillary fibroelastomas are generally small and single, occur most often on valvular surfaces, may be mobile, resulting in embolization. Because of the potential for embolic events, symptomatic patients, patients undergoing cardiac surgery for other lesions, and those with highly mobile and large ones should be considered for surgical excision. Valve-sparing excision produces good long-term results. (2) **Case report**: We present 66-years-old asymptomatic female who was diagnosed an incidental cardiac mass on echocardiography. Cardiac mass was attached to aortic side of aortic valve and was 22 x12 mm in dimension. Due to the large size of cardiac mass the patient underwent urgent cardiac evaluation, to evaluate the consistency and the extent of tumorous mass. MSCT coronarography showed Calcium score of coronary arteries 63, suggesting non-significant coronary disease. Inside of noncoronary sinus with expansion to left coronary sinus, postcontrast imbibition defect was visualized, irregular in contour, dimensions 20x13mm parallel to aortic anulus. Transesophageal echocardiography was done to ensure the exact location and attachment to cardiac structures, as well as mobility. She was referred to Heart Team. Considering the size and mobility of tumor, the decision was to do surgical resection. Surgical resection was done, with complete extraction of tumorous mass, great result and sparing of aortic valve structure. During surgery the pathohistological material of tumorous mass was send to pathohistological evaluation. It showed the tumor was papillary fibroelastoma, respectively benign primary cardiac tumor. At 2 month follow up patient was clinically stabile, except for the occurrence of atrial fibrillation, as a possible postoperative complication. **Conclusion**: This case report is the example of comprehensive diagnostic evaluation and treatment possibilities in patient with cardiac papillary fibroelastoma.

Vlasta Soukup Podravec, Kristina Milevoj Križić, Ivana Petrović Juren, Andreja Čleković-Kovačić, Sandra Prša

**Introduction**: Coronary intramural hematoma is defined as an accumulation of blood within the media of artery without intimal disruption. (1-4) It is a rare cause of acute coronary syndrome. Several factors such as the pre- and postpartum periods, trauma, hypertension, vasculitis, fibromuscular dysplasia, and the use of contraceptives may potentially relate to this phenomenon, yet this patient did not possess any of them. The most common profile is a middle-aged woman with few cardiovascular risk factors. **Case report**: 60-year-old woman, smoker with history of untreated arterial hypertension, hyperlipidemia presented with retrosternal chest pain duration for 2 hours. Initial 12-lead electrocardiogram (ECG) showed elevated ST-segment in leads V4 to V6 and D1 and aVL. Patient received a loading dose of aspirin and ticagrelor and was transferred to a tertiary hospital for a coronary angiography which showed a subocclusively altered distal segment of the left anterior descending artery (LAD) up to the apex. Based on coronary angiography, it was concluded that it is most likely an intramural hematoma possibly caused by fibromuscular dysplasia. It was recommended to do a recoronarography in two months and to continue with dual antiplatelet therapy. A transthoracic echocardiogram showed normal global systolic function of the left ventricle with hypocontractility of the lateral wall. The patient was discharged after a few days in a very good condition. After two months recoronarography was performed which showed complete recanalization of the apical LAD. CT angiography of the renal arteries and supraaortic branches were also performed where no changes were found that would indicate fibromuscular dysplasia. The patient does not have chest pain anymore and she tolerates physical exertion well. **Conclusion**: Diagnosis and treatment of intramural hematoma of the coronary artery is very challenging, because it is an overlooked diagnosis. Catheter-based angiography is validated for the diagnosis of coronary intramural hematoma. In a stable patient conservative approach is preferred because an intramural hematoma heals spontaneously. Dual antiplatelet therapy with aspirin and clopidogrel for one year is recommended followed by using aspirin alone indefinitely.

Gordana Bačić, Ivana Smoljan, Vjekoslav Tomulić, Davorka Lulić, Teodora Zaninović-Jurjević

Mitral valve surgery is the recommended first-line treatment option for the majority of patients with severe mitral valve regurgitation or stenosis. Despite the major progress achieved in surgical techniques and prosthesis characteristics over the past two decades, reoperation is needed in 20% to 35% of patients after mitral valve replacement or repair during 10-year follow-up. Redo mitral valve surgery is associated with high early mortality, especially in elderly patients with multiple comorbidities. Transcatheter mitral valve implantation has emerged as a valuable and promising alternative for redo surgery in symptomatic patients with degenerated mitral bioprosthesis (transcatheter mitral valve-in-valve implantation, TMVI-VIV) or failed mitral annuloplasty rings (transcatheter mitral valve-in-ring implantation, TMVI-VIR) who are judged by a Heart Team to be at high or greater surgical risk. Transcatheter mitral valve implantation has also been performed in patients with native mitral valve dysfunction due to extensive mitral annular calcification (TMVI-MAC). The most commonly implanted devices are Edwards SAPIEN balloon-expandable valves (SAPIEN 3, SAPIEN XT) through the transfemoral or transapical approach. Recent data from registries show favorable outcomes, including technical, device, procedure and patient success in VIV and VIR groups. (1) Patients with extensive mitral annular calcification are the most challenging group with the lowest device success and the highest in-hospital and 30-day mortality as compared to VIV or VIR procedures. We present our centre experience with transcatheter mitral valve implantation. From May 2021 to June 2022 four patients at high surgical risk underwent TMVI at our site. Two procedures were performed as TMVI-VIV in failed mitral valve bioprosthesis and two procedures as TMVI-MAC. The predominant type of mitral valve dysfunction in all patients was severe stenosis. All procedures were performed in general anesthesia, using transfemoral approach. An Edwards SAPIEN 3 valve was implanted in all patients under 3D-TOE and fluoroscopic guidance. TMVI was technical successful in three patients. Conversion to surgery and valve embolization was observed in one patient with extensive MAC. Three patients with technical successful TMVI had good hemodynamic parameters without left ventricular outflow obstruction detected. Clinical success with functional improvement was achieved in two patients. One patient was hospitalized for worsening heart failure six weeks after the procedure. One patient died three weeks after hospital discharge due to sepsis. TMVI is a promising but still challenging method of treatment for patients with either failing mitral bioprosthesis, failing annuloplasty rings or in patients with native mitral valve dysfunction due to extensive MAC. Careful planning and Heart Team approach is required to improve both patient selection and procedural technique, especially in TMVI-MAC group.

Marin Boban, Mladen Jukić, Robert Bernat, Antun Zvonimir Kovač

**Introduction:** Hypertrophic cardiomyopathy (HCM) is a genetic cardiac disorder characterized by the thickening of the myocardial walls of the heart. (1) It can lead to structural and functional abnormalities of the heart and is a common cause of sudden cardiac death. Treatment for HCM typically includes medications, lifestyle modifications, and sometimes surgical interventions, including Transcoronary Ablation of Septal Hypertrophy (TASH). This case report presents a patient who underwent a TASH procedure to manage symptoms of the HCM. **Case report:** A man born in 1983, without previous known heart pathology, working as a chief engineer in a shipyard in South Korea presented himself in November 2019 due to occasional palpitations, discomfort in the chest, and presyncope during exertion. The family history indicated that the patient’s father had undergone heart valve surgery. Patient previously underwent cardiological examination - echocardiogram showed thickening of the proximal interventricular septum with a gradient in the left ventricular outflow tract of 45 mmHg. MSCT coronary angiography ruled out significant atherosclerotic changes in the coronary arteries but revealed deep bridging in the left anterior descending artery. The patient was recommended medication treatment (bisoprolol 2.5 mg) and advised to undergo further testing including echocardiogram. In February 2020, the patient developed severe symptomatic presyncope and syncope, and the diagnosis of hypertrophic cardiomyopathy was made. The patient underwent a TASH procedure in Bad Neustadt. The pre-procedural gradient at rest was 100 mmHg (up to 200 mmHg post-VES/Valsalva). Patient reported significant improvement in symptoms and could now tolerate moderate physical exertion. Follow-up examinations showed no significant changes or abnormalities. The patient is to this day without anginal symptoms, feeling well, and tolerating moderate physical activity. **Conclusion:** This case report demonstrates the successful use of TASH in managing symptoms of HCM in a patient who presented with palpitations and presyncope. While the procedure can be invasive, it may provide significant symptom relief for patients with HCM who do not respond to other forms of treatment. Regular follow-up examinations are essential for monitoring the patient’s condition and ensuring optimal long-term outcomes.

Ivana Sopek Merkaš, Nenad Lakušić, Jurica Šalković, Alen Hodalin

**Introduction**: Primary cardiac tumors are rare, with an incidence estimated to be approximately 100 times lower than that of secondary tumors (1). Autopsy studies have indicated a prevalence of primary cardiac tumors ranging from 0.001% to 0.03% (1). Myxomas are the most common type of primary cardiac tumor, accounting for 50-70% of cases (2). Among myxomas, left atrial myxomas are the most frequent subtype, constituting about 75% of cases (2). Although myxomas can occur in people of all ages and genders, they are more prevalent in women and typically arise in adults aged between 30 and 60 years. (1) While the majority of myxomas occur sporadically (90%), some may be associated with genetic syndromes such as Carney complex or familial myxomatous syndrome (1). Left atrial myxomas can cause various adverse outcomes due to multiple pathophysiological mechanisms. These include inducing intracardiac obstruction leading to symptoms of dyspnea, fatigue, and angina, embolization resulting in cerebral, limb, or organ ischemia, disrupting normal cardiac electrophysiology leading to arrhythmias, and inducing heart failure (1, 2). **Case report**: We present a case of a 49-year-old woman who was referred for an echocardiographic examination due to newly developed symptoms of NYHA class II-III heart failure. The patient had a previous medical history of a stroke at the age of 32, and there is no documented medical evidence of a transthoracic echocardiogram or transesophageal echocardiogram being performed during the initial evaluation to assess for possible cardiac sources of embolism. She was discharged with a diagnosis of antiphospholipid syndrome and was on warfarin therapy for the next six years. However, this diagnosis was later excluded, and oral anticoagulant therapy was discontinued. Transthoracic echocardiography revealed a tumor mass within the left atrium, fixed to the interatrial septum, measuring approximately 2x4 cm (**Figure 1**). The mass dynamically moved and prolapsed into the left ventricle during diastole, causing obstruction of the mitral valve with hemodynamic parameters resembling severe mitral stenosis (peak gradient 20 mmHg, mean gradient 14 mmHg) and resulting in moderate pulmonary hypertension (pulmonary artery pressure around 60 mmHg). A cardiac surgical procedure was subsequently performed, during which the tumor mass was completely excised. The pathology report described the tumor as a myxoma. FIGURE 1. Visualization of a left atrium myxoma from the long parasternal axis view.

Tomo Svaguša, Dubravka Šušnjar, Stjepan Cvetković, Matija Drinković, Grgur Dulić

In patients with an implanted aortic valve, the importance of obtaining the maximum values of velocity and flow over the valve is extremely important to obtain adequate data on hemodynamics. However, the variability between the obtained values of ultrasound measurements in the same patients and among different ultrasound specialists has been known for a long time. Also, differences in hemodynamics between different manufacturers of heart valves are known, with a note that the larger the valve, the more favorable the hemodynamic effect on the valve is. By measuring the average internal diameter (ID) of the biological aortic valve in comparison with the body surface area (BSA) and the body mass index (BMI), an adequate instrument can be obtained for the comparison of different cardiac surgical centers. ID could also be a parameter that would allow us to compare different manufacturers of prosthetic aortic valves. (1, 2) The aim is to show the results of one center over a year, the average ID of the aortic valve and its relationship with body surface area and body mass index. The study included patients from University Hospital Centre Osijek, who were treated for severe isolated aortic valve stenosis from January to December 2022, and who have had a biological aortic valve implanted. Patients who had dilatation of the ascending part of the aorta, who had an implanted mechanical aortic valve, and who had significant aortic regurgitation in addition to aortic stenosis were excluded from data analysis. For adequate assessment and comparison with other centers, the average UP is expressed in relation to body surface area and body mass index. Patient’s data and measured values are shown in **Table 1**. Data are shown as average ± standard deviation. The presence of a bicuspid aortic valve, the left ventricular ejection fraction, and the presence of postoperative aortic regurgitation were not examined in the presentation of the results. The above should be examined in more detail in future research. ### TABLE 1: Patient data. | N | 54 | | --- | --- | | MALE | 35 | | AGE (year) | 68.07 ± 7.10 | | HEIGHT (cm) | 167.68 ± 9.59 | | MASS (kg) | 81.58 ± 15.41 | | BSA (m2) | 1.94 ± 0.21 | | BMI (kg/m2) | 29 ± 4.77 | | INTERNAL DIAMETER (mm) | 21.4 ± 1.87 | | INTERNAL DIAMETER/BSA (mm/m2) | 11.13 ± 0.89 | | INTERNAL DIAMETER/BMI (mm/kg/m2) | 0.76 ± 0.12 | [†] BSA = body surface area, BMI = body mass index Due to the different sizes of biological aortic valves used in clinical practice, we believe that the average internal diameter of the valve (the part through which blood passes) indexed by body surface area or body mass index would be adequate data for comparing different types of aortic valves. Also, it could become one of the parameters for comparison with different cardiac surgery centers.

Vanja Nedeljković, Ana Marija Slišković, Dejan Došen, Marko Siroglavić, Petra Angebrandt Belošević

**Introduction**: AV block of varying severity is the most common disorder in Lyme carditis. However, myocarditis, pericarditis, and heart failure have all been described as possible manifestations. The clinical course of Lyme carditis is generally mild, and in most cases, completely reversible. (1-3) **Case report**: 32-year-old male patient was admitted for one week history of shortness of breath and chest pain. Physical exam showed normal findings. Resting 12-lead ECG showed signs of pericarditis and laboratory tests showed mild signs of inflammation. Echocardiography (**Figure 1** and **Figure 2**) showed circular pericardial effusion maximum size of 22 mm next to the right atrium and ventricle with otherwise normal findings. For first day of admission patient was treated with high doses of ibuprofen and colchicine. After 2 days of treatment patient developed fever with significant rise in inflammatory markers and therefore antimicrobial therapy was initiated (first amoxicillin + clavulanic acid and azithromycin). Since patient had a fever that lasts longer than 5 days with rise in inflammatory markers (maximum value of CRP was 285 mg/L) antimicrobial therapy was escalate to piperacillin-tazobactam. Due to clinical course of disease, we performed all blood test, including viral and bacterial serology, and other tests to exclude autoimmune condition, tuberculosis, and neoplasm. We performed CT scan which revealed bilateral pleural effusion. All blood tests were negative except serology of Lyme borreliosis (Lyme disease) where IgM antibodies for Borrelia burgdorferi infection were detected with Enzyme-Linked Immunosorbent Assay (ELISA) and confirmed with Western Blot Analysis (WB). After consulting with infectologist, antimicrobial therapy was changed to ceftriaxone. Soon after, fever stopped, patient had no more chest pain and markers of inflammation, CRP and leukocyte count, decline. After 5 days of antibiotic therapy echocardiography revealed no signs of pericardial effusion (**Figure 3** and **Figure 4**). FIGURE 1. Long parasternal view of circular pericardial effusion. FIGURE 2. Subcostal view of pericardial effusion. FIGURE 3. Long parasternal view of resolution of pericardial effusion after antibiotic treatment. FIGURE 4. Subcostal view of resolution of pericardial effusion after antibiotic treatment. **Conclusion**: Lyme disease is a common disease that uncommonly affects the heart. Because of its rarity and the often absence of other clinical manifestation, consideration of Lyme carditis demands a high level of suspicion. Therefore, we believe that with atypical clinical presentation, this was rare case of Lyme carditis manifested as acute pericarditis.

Marija Tomac Stojmenović, Vlatka Rešković Lukšić, Irena Ivanac Vranešić, Velena Radošević, Tamara Žigman, Maja Hrabak Paar, Jadranka Šeparović Hanževački

**Introduction**: FLNA gene provides instructions for producing protein filamin A. It plays a role in regulating skeletal and brain development, formation of heart tissue and blood vessels, maintenance of lung tissue, function of digestive system, etc. It is found on the X chromosome and has X linked dominant inheritance (**Table 1**). Pregnancy with aortic aneurysm is rare but potentially fatal. Aortic dissection in pregnancy accounts for 0,1-0,4% of all dissections. Maternal mortality is high. (1-4) Currently there are no guidelines for the management of vascular, cardiac, and connective tissue problems during pregnancy with FLNA mutation. ### TABLE 1: Clinical findings in patients with FLNA mutation. | **CARDIOVASCULAR** | **CENTRAL NERVOUS SYSTEM** | **GASTROINTESTINAL** | **OTHER** | | --- | --- | --- | --- | | -Aortic dilatation -Outflow tract malformation -Patent ductus arteriosus -Atrial/ventricular septal defect -Vascular malformation | -Periventricular nodular hyperplasia -Seizures -Mental retardation -Cerebellar hypoplasia -Early hypotonia -Spasticity -Polymicrogyria | -Intestinal malrotation -Congenital short small intestine -Pseudo-obstruction/ severe chronic constipation -Anal stenosis | -Joint hypermobility -Thrombocytopenia -Dysmorphic facies -Respiratory infections -Hypospadias | **Case report**: We present a family (**Figure 1**) with heterozygous pathogenic variant of FLNA (c.2191-2192insGT (p.Tyr731Cysfs *12)) and aortic aneurism. FIGURE 1. Genetic tree. Mother: double miscarriages (16th week), two deliveries (vaginal), died at age 60, due to aortic dissection. Last known size of ascendent aorta was 43-45mm. She had mild/moderate aortic regurgitation, mild mitral and tricuspid regurgitation, coronary artery disease and underwent percutaneous coronary intervention at the age of 56. Sister: one miscarriage (8th week), moderate aortic regurgitation, dilatation of ascending aorta (42mm), pulmonary artery dilatation (40mm), mild pulmonary regurgitation, bilateral periventricular heterotopia, hypoplastic body of corpus calosum. In 2021. gave birth (C-section) to a baby girl GA 34+4 weeks. Baby girl is FLNA mutation positive and has subependymal heterotopia, echocardiography is normal. Sister: double miscarriage (13th, 8th week), mild mitral and aortic regurgitation, aortic dilatation (2020/2022. 46mm/48mm), joint hyperlaxity, hypoplastic back third of corpus callosum, and bilateral periventricular heterotopia. In 2022 she gave birth (C-section) to a healthy boy GA 34+3 weeks (genetic results pending). Grandmother: one delivery, three miscarriages, died at the age of 64. We don’t have genetic confirmation of mutation. She had sister who has a healthy son, further details are unknown. Father has dilatation of ascending aorta (41mm), he is not a carrier of FLNA mutation. **Conclusion**: Both sisters were advised against pregnancy, because there is still no exact data on this mutation and its influence on aneurism progression and childbirth. The pregnancies were high-risk and required team approach - frequent monitoring by cardiologist and gynecologist (during pregnancy and 6 months after delivery). ## Acknowledgments Patient consent: Obtained from family.

Maria Nicole Sičaja, Mario Sičaja

**Introduction**: Intramyocardial dissection hematoma (LV-IDH) is a rare complication of myocardial infarction. It is usually caused by hemorrhagic dissection among the spiral myocardial fibers. LV-IDH consists of a cavity filled with blood, the outer wall of which is the myocardium and pericardium, and the inner wall, facing the ventricular cavity, is part of the myocardium and endocardium. (1-3) To date there is no establish protocols and clear guidelines for management in such cases. **Case report**: We hereby report the case of a 80-year-old male patient with left ventricular intramural dissecting hematoma (LV-IDH) who presented with a severe breathlessness with heart failure (NYHA IV) complicated with cardiorenal syndrome (Type 1). Transthoracic echocardiography (TTE) was performed showing LV-IDH, confirmed with cardiac magnetic resonance imaging (cMRT). Selective coronary arteriography was performed showing significant obstructive coronary artery disease (CAD). Patient management first included conservative approach followed with complex PCI. **Conclusion**: LV-IDH is an extremely rare and diagnostically challenging condition that occurs most often as a complication of CAD. The diagnostic algorithm includes multimodal imaging in the entire spectrum of cardiac imaging (primarily TTE and cMRT). The main differential diagnosis includes intracavitary thrombosis. The distinction from intracavitary thrombus relies on the clear identification of the endocardial layer surrounding the neoformation and its systolic expansion. Therapy is complex and individualized and should follow through the Heart Team Protocol.

Alma Mehić, Sanela Halilović Šuškić, Đemil Hrnjić, Aida Brzika, Emina Alispahić-Husić, Hatidža Omerdić, Belma Šakić

**Introduction**: Infective endocarditis (IE) is rare but potentially fatal disease. Right-sided IE accounts for 5% to 10% of all IE cases, and compared with left-sided IE, it is more often associated with congenital heart desease (1). Among congenital heart disease, ventricular septal defect (VSD) is the most frequent anomaly in right-sided IE (2). It is rarely diagnosed in adults owing to correction of large VSDs and the spontaneous closure of smaller ones during the patient’s early years (3). **Case report**: 66-year-old man was admitted to Internal Department with symptoms of general weakness, palpitations and shortness of breath which aggravated over the past two weeks. His physical examination showed blood pressure of 140/90 mmHg, irregular heart rate of 203 beats per minute and axillar temperature of 37.0 C. Systolic/diastolic murmur was heard over the left upper sternal border. Laboratory investigation showed mild leukocytosis with white blood count of 10.89x 109/L, raised C-reactive protein (CRP) of 47 mg/L and positive rheumatoid factor. The transthoracic echocardiography (TTE) revealed left-to-right shunt perimembranous VSD with enlarged all four heart chambers (**Figure 1**). There were visible vegetation on aortic and tricuspid valves, dilated mitral annulus and severe aortic, mitral and tricuspid regurgitation with signs of pulmonary hypertension (**Figure 2****,****Figure 3**). Left ventricular systolic function was normal with ejection fraction of 55%. Series of blood cultures were taken but they remain sterile. A computed tomography pulmonary angiogram (CTPA) did not show any signs of pulmonary embolization but there were bilateral pleural effusion. The patient was diagnosed as possible infective endocarditis with modified Duke clinical diagnostic criteria (one major and two minor criteria). He was treated with intravenous empirical antibiotics, diuretics, antiarrythmics and other supportive treatment. Despite intensive care and treatment his clinical condition was worsened with signs of heart, renal and liver failure. The patient unfortunately died. FIGURE 1. Transthoracic echocardiography showed a ventricular septal defect. FIGURE 2. Parasternal long axis view showed vegetation on aortic valve with severe aortic and mitral regurgitation. FIGURE 3. The apical four chamber view showed large vegetation attached to the tricuspid valve. **Conclusion**: Infective endocarditis still remains diagnostic and therapeutic challenge. Echocardiography is the primary imaging technique used to detect right-sided IE as it provides valuable information because of anterior location of right-sided structures that are close to the TTE transducer. Prompt diagnosis is necessary to prevent its complications including multi organ failure and death.

Jure Samardžić, Vedran Velagić, Vedran Pašara, Ivan Prepolec, Daniel Lovrić, Mislav Puljević, Borka Pezo-Nikolić, Ivo Planinc, Maja Čikeš, Davor Puljević, Davor Miličić

**Introduction:** Pulmonary vein isolation (PVI) is a well-established method for treating paroxysmal and persistent atrial fibrillation (Afib) to maintain sinus rhythm. Left atrium size is an important factor which can determine rate/rhythm control strategy. Normal, mildly, and moderately increased left atrial volume index (LAVI) favors rhythm control. We sought to evaluate periprocedural characteristics and outcomes (freedom from Afib reccurence and effect on left atrium size) of Afib patients with severely enlarged left atria (LAVI > 48 mL/m2) who underwent cryo PVI in our institution. **Patients and Methods:** We analyzed data from our hospital’s PVI registry from February 2015 to November 2021. Patients with LAVI > 48 mL/m2 prior to PVI were enrolled in the analysis. Follow up was performed through hospital’s information system to assess potential Afib reccurence and control LAVI. **Results:** Out of 611 patients, 222 had no information on left atrial size. Seventeen patients (2.78%) had LAVI > 48 mL/m2 (mean 61 mL/m2; range 49-85 mL/m2). Patients were mostly men (n=10; 58.8%), in persistent Afib (n=10; 59.8%) and underwent PVI in sinus rhythm (n=12; 70.6%). Mean CHADS-VASc score was 2.11 (range 1-4). Fourteen patients had preserved ejection fraction (82.3%). Mean procedure time was 69.5 min and all PV were isolated in 15 patients (88.2%). There was no significant periprocedural complications (only two patients had transient phrenic nerve palsy). Three patients were lost to both clinical and echo follow up. Median follow up of was 11.5 months (range 5-29). Only two patients had Afib reccurence. LAVI measurement in follow up was available for seven patients and was statistically lower compared to index LAVI (44.6 mL/m2 vs 55.7 mL/m2; p=0.03). **Conclusion:** Our results indicate that PVI can be a safe and effective method to maintain sinus rhythm and reduce LAVI in selected Afib patients with severely increased left atrium.

Dijana Trninić, Jelena Jovanić, Bojana Ilić, Nina Žmarić, Jelena Đoković

**Background:** Moderate or severe tricuspid regurgitation is observed in 0.55% of the general population and its prevalence increases with age, affecting about 4% of the patients aged 75 years or more. Etiology is secondary in ≥90% of cases. Tricuspid valve prolapse (TVP) is rare on transthoracic echocardiography and of uncertain significance. Over 18 years is TVP was present in 0.3% of individuals. Seventy–five percent of those with TVP had associated mitral valve prolapse. (1-4) **Case report**: 66-year-old patient presented with worsening condition in the form of frequent palpitations and a decrease in exercise tolerance. She has a history of moderately severe tricuspid regurgitation, ventricular premature ventricular contractions, and drug-controlled hyperthyroidism. On examination a pansystolic murmur was audible at the left lower sternal edge. The electrocardiogram showed a sinus rhythm with partial right bundle branch block. In the Holter 24-hours EKG-monitoring, 3560 VES, medium long coupling. Echocardiography shows severe tricuspid regurgitation (**Figure 1**). The jet of tricuspid regurgitation is directed in the largest part towards the interatrial septum. PISA radius 9mm, effective regurgitant orifice area (EROA) was 50 mm2 and a regurgitant volume 60 mL. The right ventricle is borderline in size, TAPSE 22mm. The right atrium is slightly dilated (area 17cm2). Transesophageal echocardiography visualizes tricuspid valve prolapse (**Figure 2**) with rupture of the secondary chordae of the septal leaflet (**Figure 3**) and eccentric jet of tricuspid regurgitation (**Figure 4**). The following hemodynamic parameters were obtained by catheterization of the right heart: RA 13mmHg, RV 55/2/18mmHg, PA 38/19/29mmHg, PCWP 18mmHg CO 6,04L/min, SV 86ml/beat PVR 1,8WOOD Units. The patient was referred to the cardiothoracic surgery department. Heart surgery was performed. FIGURE 1. Transthoracic echocardiography: Apical four-chamber view. Severe tricuspid valve regurgitation jet. FIGURE 2. Transesophageal mid-esophageal four-chamber view; tricuspid valve prolapse. FIGURE 3. (A, B) Transesophageal mid-esophageal four-chamber view: rupture of the secondary chordae of the septal leaflet. FIGURE 4. Transesophageal mid-esophageal four-chamber view: jet of tricuspid regurgitation. **Conclusions**: Severe tricuspid regurgitation is associated with impaired survival and worsening heart failure. Appropriate timing of intervention is crucial to avoid irreversible RV damage and organ failure with subsequent increased surgical risk. When surgery is performed, valve repair is preferred over replacement. Valve replacement is associated with a higher mortality rate. Surgical repair is possible in the vast majority of cases of tricuspid valve and chordal anomalies. For patients at increased surgical risk novel percutaneous interventions may offer an alternative treatment.

Dubravka Šušnjar, Tomo Svaguša, Davor Barić, Daniel Unić, Josip Varvodić, Marko Kušurin, Nikola Slišković, Savica Gjeorgjievska, Gloria Šestan, Igor Rudež

Established surgical treatment of aortic stenosis, especially nowadays using a minimally invasive approach through ministernotomy, is still the gold standard in the treatment of the calcifying degenerative disease of aortic stenosis. Considering of the choice of artificial aortic valves, regarding the size and type of prosthesis, available today, it is crucial to monitoring their impact on hemodynamics, to avoid the prosthesis patient mismatch (PPT). Prosthesis patient mismatch occurs when the effective orifice area (EOA) of the inserted prosthetic valve is too small in relation to body size. It is necessary the calculate the patient’s body surface area (BSA) from weight and height and determined of the minimal valve EOA required to ensure an indexed EOA >0.85 cm2/m2, select the type and size of prosthesis that has reference values for EOA greater or equal to the minimal EOA value. (1, 2) Aim: To present hemodynamic impact of different aortic valve prosthesis in a cohort of patients treated with surgical aortic valve repair in our institution, to avoid prothesis-patient mismatch, which may have a significant impact on the long-term results of valve surgery. Study included 115 patients (75 male, 40 female) who underwent isolated aortic valve replacement surgery in our center in the period of January 2022 until January 2023. Patient’s ages ranged from 32 to 81 (M=67.7), average BSA 1.96 m2. Average EuroScore logistic was 6.15%. Biological prosthesis was implanted at 108 patients, while a mechanical prosthesis was used in 7 patients. Biological prosthesis that are used are Carpentier-Edwards Magna Ease, Edwards Inspiris Resilia, Medtronic Avalus, in range of 19-29 mm, and mechanical prosthesis St Jude Mechanical, CarboMedics Mechanical TopHat, On-X valves in range of 21-27 mm. The average velocity V max was 2.57 m/s±0.39, and average PPG was 26.92 mmHg±8.34. 17 patients (15.6%) who underwent surgical replacement had postprocedural velocity over the valve >3m/s.

Paula Radić, Anja Mandrapa, Vedran Carević, Tea Domjanović Škopinić, Ivona Mustapić, Darija Baković Kramarić

**Introduction:** Takotsubo cardiomyopathy (TTS) or stress cardiomyopathy is a transient dysfunction of left ventricle often caused by emotional or physical stress. It is often misdiagnosed as myocardial infarction (1). Bronchogenic TTS was proposed as a special form of TTS occurring during severe dyspnea in COPD and asthma (2). **Case report:** We are presenting a 73-year-old female brought to the Emergency Department after sudden onset of dyspnea. During medical history taking she stated shortness of breath for the last four months. Because of severe respiratory distress and high D-dimer levels (22.46 mg/L) multislice CT pulmonary angiography was arranged and pulmonary embolism was ruled out after which she was admitted in the Intensive Care unit (ICU). Initial blood work showed severe metabolic acidosis and high troponin T level (193.3 ng/L) with normal N-terminal pro b-type natriuretic peptide (464 pg/mL). 12-lead electrocardiogram showed poor R wave progression through precordial leads. Cardiologist was consulted and emergent invasive coronary angiography was performed but showed no significant findings. Patient was transferred to the Cardiology Intensive Care Unit and echocardiography verified stress cardiomyopathy with reduced left ventricle ejection fraction (2D LVEF 42%), lower global longitudinal strain (GLS -7%), dyskinetic circumferential apical region of the left ventricle (**Figure 1**) and indirect signs of pulmonary hypertension. Echocardiography was repeated 8 days after the initial one and showed improvement of LVEF (60%), still lower GLS (-10,7%) (**Figure 2**), and hypokinetic apicoseptal region. Pulmonary function tests were done, and chronic obstructive pulmonary disease (COPD) was diagnosed. A final diagnosis of transitory manifestation of stress cardiomyopathy due to COPD exacerbation was made. After that, patient was transferred to the Clinic for Lung Disease for further pulmonary status assessment. FIGURE 1. Apical ballooning pattern. FIGURE 2. Global longitudinal strain. **Conclusion:** Sometimes, severe respiratory distress due to COPD or asthma can cause stress cardiomyopathy. Echocardiography is a noninvasive pivotal imaging modality used to diagnose it.

Hrvoje Falak, Petra Bistrović, Vanja Ivanović Mihajlović, Mario Udovičić, Antonio Bulum, Davor Barić, Šime Manola

**Introduction**: Rheumatoid arthritis can cause a variety of cardiac manifestations. Pericarditis can be found in about 30% of the patients, however it is usually acute and asymptomatic. (1) Constrictive pericarditis is a rare complication of rheumatoid arthritis with major complications. (2) **Case report**: We present a case of a 70-year-old woman who presented to the emergency room with signs and symptoms of right ventricular heart failure. The patient has a 30-year-old history of seropositive rheumatoid arthritis and is currently treated with steroids and ebetrexat. Also, cardiac workup was performed a few years prior due to microvoltage in the ECG, however other than lamellar pericardial effusion with no effects on hemodynamics, no pathology was found. Initial workup showed right sided pleural effusion and cranial redistribution on chest X-ray, elevated natriuretic peptide level, as well as slightly elevated bilirubin and liver enzymes. A small amount of ascites was noted on the abdominal ultrasound. A circumferential 8-millimeter pericardial effusion, lower lateral e’ wave velocity than septal e’ wave velocity and a dilated vena cava were noted on the echocardiogram. Right heart catheterization showed possible signs of constriction. Treatment with non-steroid antirheumatics was initiated and prior corticosteroid therapy was escalated, however the patients’ symptoms worsened, and the effusion progressed. Further magnetic resonance imaging revealed a thickened pericardium with post-contrast imbibition and septal bounce in early diastole, findings suggestive of constrictive pericarditis. Patient was referred to cardiac surgeons and radical pericardiectomy was performed with a good outcome. **Conclusion**: Constrictive pericarditis is a rare and easily overlooked diagnosis that should be kept in mind when assessing rheumatological patients with signs of right-sided heart failure.

Petra Bulić, Ivana Grgić Romić, Ana-Marija Flego Bojić, Ana Antonić, Teodora Zaninović Jurjević

**Introduction**: Qvadricuspid aortic valve (QAV) represents an extremely rare congenital heart disease with an incidence of 0.01-0.04%. It is generally an isolated anomaly, but 18-32% of patients present with another congenital heart disease. (1, 2) QAV is frequently associated with progressive aortic regurgitation (AR) with the development of significant valvulopathy in the fifth to sixth decade. There are a few classifications of QAV based on leaflet size and distribution. (1-3) Transthoracic echocardiography (TTE) and transesophageal echocardiography (TEE) are the main methods of QAV detection. Computed tomography (CT) may accurately show the status of QAV, dimensions of the aorta, and the location of coronary ostia. (2, 3) **Case report**: A totally asymptomatic 49-year-old woman was referred to our echo lab after an accidental finding of a diastolic heart murmur. A cardiovascular examination did not show signs of heart failure. TTE showed normal left ventricular dimensions, wall thickness, normal 3D-derived left ventricle volumes and ejection fraction (60%). The size of the aortic root was normal, and ascending aorta was slightly dilated. The parasternal short-axis view showed an aortic valve characterized by 4 cusps of equal size and moderate aortic regurgitation with a central aortic jet, without any further anomalies. She did not agree to the proposed TEE, but the acoustic window on TTE was accurate to see closely the aortic valve. We did a CT of the aorta and aortic valve, which showed the QAV without calcification, normal measurements of the aortic annulus and an annulus circumference, as well as the normal distance of the annulus from the coronary arteries. The ascending aorta was slightly dilated. **Conclusion**: A QAV is a rare congenital heart disease whose clinical manifestations depend on the functional status of the valve and the presence of any associated anomalies. The presence of severe aortic regurgitation, severe aortic stenosis, or QAV with valvular dysfunction associated with other clinically significant abnormalities are indications for surgery. (1, 2) The surgical options are replacement or valve repair. Aortic valve tricuspidalization is the most common repair technique. (1-3) Our patient was totally asymptomatic with normal ventricular function and moderate aortic regurgitation, so further TTE follow-up is indicated in this case.

Josipa Josipović, Klara Brčić

Undiagnosed or inadequately controlled arterial hypertension (AH) is the leading independent factor of cardiovascular (CV) morbidity and mortality. In addition, the grouping of multiple risk factors in patients with AH contributes to the severity of this “silent but deadly” disease. Assessment of the total CV risk and treatment of each possible risk factor are extremely important in reducing the total CV risk. The use of fixed-dose combinations of medicinal products with good pharmacodynamic, pharmacokinetic, metabolic, and safety profiles has been proven to simplify the treatment and improve the outcomes and adherence of patients, which must be lifelong. One such combination that contains a long-acting ACE inhibitor – perindopril, a metabolically neutral long-acting diuretic from the thiazide class – indapamide, and a potent statin – rosuvastatin, is the fixed-dose combination Roxiper® – a triple combination for two diseases in a single tablet.

Tea Domjanović Škopinić, Darija Baković Kramarić, Andrija Matetić, Frane Runjić, Ivica Kristić, Vedran Carević

**Introduction:** Transcatheter aortic valve implantation (TAVI) is recommended in elderly patients with severe aortic stenosis and high surgical risk (1). This procedure offers survival benefit, but the development of valve degeneration is associated with impaired prognosis. There are several mechanisms for valve degeneration, including the under-expansion, leaflet damage, enhanced calcification, and valve thrombosis. Recent studies have shown an emerging phenomenon after TAVI, which can be seen in up to 13% of patients, that is characterized by hypo-attenuated leaflet thickening (HALT). This imaging feature can be associated with reduced leaflet motion (RELM) leading to valve failure in particular patients (2). Studies have shown that introducing anticoagulation therapy with warfarin can lead to partial, and sometimes even complete resolution of valve thrombosis (2, 3). It is important to be aware of this potential complication of the TAVI procedure and to know how to proceed with further evaluation of patients who present with valve dysfunction early after TAVI. **Case report:** We present an 80-year-old patient that underwent TAVI procedure with balloon-expandable valve. Early postprocedural transthoracic echocardiogram (TTE) confirmed good valve function with a maximal velocity of 1.04 m/s. However, five months later, he was scheduled for another control TTE which revealed an impaired anterograde flow at the aortic valve with maximal velocity over 5 m/s and a mean pressure gradient of 62 mmHg (**Figure 1**). The patient noticed exertion intolerance and dyspnea. He was admitted to the hospital for further evaluation, including focused multi-slice computed tomography (MSCT). Detailed analysis revealed substantial crescent-shaped thickening of all aortic leaflets with impaired motion, referring to HALT and RELM (**Figure 2**). After the initial parenteral anticoagulation, the patient was discharged with warfarin and is scheduled for control TTE after one month. FIGURE 1. Continuous doppler flow across the aortic valve. FIGURE 2. A and B. Short axis view of the aortic valve. Arrows are pointing to crescent-shaped thickening of all aortic leaflets. C and D. 2-chamber view. **Conclusion:** HALT and RELM are important factors for early valve dysfunction. Timely diagnosis with focused MSCT is crucial to initiate anticoagulation therapy. Further studies should delineate the exact underlying mechanisms for HALT, including the importance of patient, prosthesis, and hematologic factors.

Ivona Mustapić, Andrija Matetić, Paula Radić, Anja Mandrapa, Tea Domjanović Škopinić, Vedran Carević, Darija Baković Kramarić

**Introduction:** Transcatheter edge-to-edge mitral valve repair (TEER MV) is recommended for symptomatic patients with secondary mitral regurgitation (MR) (class IIa) and with primary MR (class IIb) without concomitant coronary artery or other heart disease when they are not eligible for surgery (1). Nevertheless, there are limited data on the percutaneous approach for acute primary mitral regurgitation. **Case report:** We report the case of an 82-year-old woman who was hospitalized for orthopnea and pulmonary congestion. She had previously had a left hip arthroplasty and suffered from tuberculosis in her youth. On admission, transthoracic echocardiography (TTE) was performed, showing a normal-sized left heart with preserved systolic function (LVEF 60%) and restrictive mitral inflow. The mitral valve was myxomatous degenerated with prolapse and flail of the posterior leaflet and therefore eccentric regurgitation jet. Transesophageal echocardiography revealed systolic prolapse and flail of the posterior MV leaflet due to rupture of the primary chordae tendineae **(****Figure 1****)**. Percutaneous coronary angiography showed normal epicardial coronary arteries. Considering the rapid onset of symptoms and acute primary MR, the patient was referred to the Heart Team. Due to advanced age and functional status NYHA III, the patient was considered high surgical risk and TEER was recommended. TEER MitraClip™ was successfully performed with two clips in the region of the regurgitant jet under control of TOE: A2-P2 segment; XTW x1 and XT x1. Intraprocedural success was measured by echocardiographic reduction of MR from severe to trivial with concomitant reduction in left atrial pressure. Postprocedural TTE showed a significant reduction of MR **(****Figure 2****)** with a marked improvement in functional status to NYHA I. FIGURE 1. Transesophageal echocardiography. Left image: a blue arrow pointing at prolapse and flail posterior mitral valve leaflet. Right image: color Doppler flow over mitral valve with eccentric regurgitant jet toward the septum. FIGURE 2. Transthoracic echocardiography. Left image 3D-echocardiography, a blue arrow pointing at good position of implanted clips. Right image: three-chamber view, color Doppler flow over mitral valve showing absence of mitral regurgitation. **CONCLUSION:** Acute mitral regurgitation requires rapid and adequate assessment of surgical risk with good visualization of mitral valve anatomy. If necessary, TEER is a life-saving treatment with immediate relief of symptoms.

Tea Domjanović Škopinić, Vedran Carević, Ivona Mustapić, Paula Radić, Anja Mandrapa, Darija Baković Kramarić

**Introduction:** Apical hypertrophic cardiomyopathy (HC) is a rare type of cardiomyopathy characterized by hypertrophy involving the left, and sometimes right ventricular apex. It is more prevalent in the Asian population where it accounts for 25% of patients with HC. In the non-Asian population, it accounts for 1 to 10% of hypertrophic cardiomyopathy cases (1, 2). The echocardiographic diagnostic criteria for apical HC include a demonstration of apical hypertrophy, apical wall thickness ≥ 15 mm, and a ratio of maximal apical to posterior wall thickness ≥ 1.5 (2). We present a case of apical HC in a 68-year-old patient. **Case report:** 68-year-old female patient was brought to the emergency department with symptoms of chest pain and dyspnea. 12-lead electrocardiogram (ECG) showed sinus rhythm with negative T waves in the anterolateral and inferior leads and voltage criteria for left ventricular hypertrophy. Her bloodwork was unremarkable, except for slightly increased values of N-terminal brain natriuretic peptide which was 1107 pg/ml. The high sensitive troponin T level was 36.1 ng/L. She was admitted to the Cardiac Intensive Care Unit for further observation and diagnostics. The coronary angiogram was normal. Ventriculography revealed a spade-like-shaped left ventricular cavity (**Figure 1**). Echocardiography confirmed the same shape of the left ventricle due to hypertrophy of apical segments of the LV with maximum wall thickness of 19 mm (**Figure 2**). The global longitudinal strain was reduced in apical and middle segments **(****Figure 3**) and LVEF was 61%. The patient was scheduled for cardiac magnetic resonance imaging, but she refused the imaging due to claustrophobia. FIGURE 1. Ventriculography in patient with apical hypertrophic cardiomyopathy. FIGURE 2. 4-chamber echocardiography view of apical hypertrophic cardiomyopathy with apical wall thickness of 19 mm and spade-like shaped left ventricular cavity. FIGURE 3. Global longitudinal strain in apical hypertrophic cardiomyopathy. **Conclusion:** Although apical HC is not so common in the European population, it should be considered as a differential diagnosis in patients with typical ECG changes. These patients can present with a broad range of symptoms, including palpitations, dyspnea, syncope, exercise intolerance, and chest pain. Although it is not associated with increased cardiovascular mortality, up to one-third of patients with apical HC can develop serious complications, e.g., arrhythmias, myocardial infarction, and stroke.

Mirjana Isailović-Keković, Predrag Keković

**Introduction**: Sacubitril/valsartan (S/V) is a treatment for heart failure with reduced ejection fraction (HFrEF), which impressively impact cardiovascular prognosis by reducing major adverse cardiovascular events such as cardiovascular mortality and sudden death but the precise mechanism underlying the beneficial effect on reducing cardiovascular mortality is still not clear. S/V blocks harmful effects of renin-angiotensin-aldosterone system activation and raising levels of natriuretic peptides that are degraded by neprilysin. S/V can have among patients living with HFrEF by improving imbalance in the cardiac autonomic nervous system (CANS). Data from an international investigation show the benefits that this imbalance has been linked to mortality in HFrEF, noted the study authors. (1-4) The present study aims to assess effects of S/V on heart rate variability parameters (HRV) in symptomatic patients with HFrEF. **Patients and Methods**: Patients diagnosed as a HFrEF with left ventricular ejection fraction (LVEF)<40%. 24 patients with HFrEF according to NYHA II-IV classification who aged 49 to 82 years were enrolled in the study. HRV parameters SDNN (standard deviation of normal-to-normal intervals) and RMSSD (mean square of differences between consecutive R-R intervals) were reordered before starting S/V and after one two months of the therapy. S/V treatment increased SDNN (67.22±17.7 ms vs. 88.81±16.5 ms, P <0.05) and RMSSD (31.52±12.4 ms vs. 42.9±16.1 ms) in the study participants. **Conclusion**: In our study we concluded also that LVEF improved, and plasma N-terminal-pro hormone brain natriuretic peptide fell in accordance with HRV changes.

Tea Domjanović Škopinić, Andrija Matetić, Anja Mandrapa, Paula Radić, Ivona Mustapić, Darija Baković Kramarić

**Introduction:** The most important imaging modality in diseases of the aorta is multi-slice computed tomography (MSCT) angiography. However, echocardiography can often help distinguish acute aortic syndrome from other causes of chest pain (1, 2). Here, we present a case of a 45-year-old female patient who was admitted to the emergency department (ED) with acute ST-segment elevation myocardial infarction and neurological deficit in form of left-sided hemiparesis. **Case report:** 45-year-old female patient with no previous medical history presented to the ED with chest pain that lasted for 2-3 hours before the ED visit. 12-lead electrocardiogram revealed ST-segment elevation in inferior and lateral leads. During the physical examination, it was also noted that she had left-sided hemiparesis and facial paresis. Point-of-care echocardiography was performed where the wall of the available descending part of the aorta could not be clearly visualized. This directed further diagnostic process to MSCT of the brain which showed no ischemic zones and MSCT aortography where multiple thrombi were visualized at the brachiocephalic trunk origin, aortic arch, and the descending aorta (**Figure 1A**). Furthermore, perfusion defects were verified in the right kidney. Due to the high risk of thrombotic masses dissemination, invasive coronary angiography was not performed. Instead, alteplase was administered (0.9 mg/kg). Complete neurological recovery and ST-segment elevation resolution were achieved. Her blood workup showed mildly elevated levels of high sensitive troponin T (61.8 ng/L), N-terminal pro-BNP (335 pg/mL), and very high levels of D-dimer (>35.2 mg/L). Echocardiography revealed hypokinesis of the inferior and posterior wall with preserved systolic and diastolic function of the left ventricle. Control aortography showed regression in the size of aortic thrombi (**Figure 1B**) and kidney perfusion defects. Extended laboratory work-up revealed mildly elevated levels of anticardiolipin antibodies and β2-glycoprotein (both class IgM). Since the patient was a foreign citizen, she was transported to her country to continue further medical evaluation. FIGURE 1. A. Initial MSCT of the aorta showing multiple thrombi. B. Control MSCT of the aorta 1 day after thrombolysis showing significant regression in sizes of thrombi. **Conclusion:** Echocardiography can be a valuable tool in directing the diagnostic process for diseases of the aorta. This points to the importance of point-of-care ultrasonography availability in the ED, as it speeds up the process of establishing the final diagnosis.

Ivana Grgić Romić, Lea Skorup, Petra Bulić, Ana Antonić, Tin Nadarević, Teodora Zaninović Jurjević

**Introduction**: Mitral annular disjunction (MAD) is a structural abnormality defined as the separation of the ventricular myocardium between the mitral valve annulus and the left atrial wall during systole. Multiple studies have demonstrated a high prevalence of MAD in the setting of myxomatous mitral valve disease. MAD has exceedingly variable clinical course that patients may present with. It has been associated with a risk of malignant ventricular arrhythmias and sudden cardiac death, consequently recognition of this diagnosis and risk stratification are greatly important. (1, 2) **Case report**: 23-year-old male with a history of myxomatous mitral valve disease was admitted to Cardiology Department because of 90 minutes long chest pain. High-sensitive troponin was mildly elevated (307 ng/L), same as NTproBNP (1077 n/L). Inflammation parameters and D-dimer were normal. CT coronary angiography excluded obstructive coronary artery disease (CAD). A transthoracic echocardiogram (TTE) showed normal left ventricular ejection fraction and evidence of Barlow disease with a mild to moderate mitral regurgitation (MR). Additionally, cardiac event monitoring showed no ventricular arrhythmias. Hospitalisation was uneventful, and he was discharged with a diagnosis of myocardial infarction with non-obstructive coronary arteries (MINOCA) with an instruction to do a heart CMR (myocarditis was suspected). CMR showed no signs of oedema or myocardial fibrosis, but it did describe a few crypts in the basal inferoseptal segments of the left ventricle (**Figure 1**). The missing puzzle was found one year later, when an evaluation TTE described moderate bileaflet mitral valve prolapse (MVP) and prominent MAD, measuring 8 mm in end-systole, together with earlier known mild-moderate MR (**Figure 2**). On outpatient follow-up, he has no therapy and has not had further chest pain. FIGURE 1. TTE parasternal long axis demonstrating scallop billowing and mitral annular disjunction (blue arrow). FIGURE 2. Steady-state free precession cine sequence, 3ch view. Separation of posterior mitral valve leaflet insertion and left ventricle myocardium of >5 mm. Mitral annular disjunction (blue arrow). **Conclusion:** Although MAD is easy to diagnose, it can be overlooked in daily practice, like it happened in our case. The authors speculate that MVP and MAD were aetiology of MINOCA, and it should be considered in the work-up. The current guidelines recommend implantable cardioverter defibrillator (ICD) for secondary prevention, but the role of primary prevention ICD in MVP and MAD is unclear and decisions should be made on an individual basis. (3) Providing the case, we wanted to increase the awareness of this anatomic variation of the mitral valve.

Filip Lončarić, Xabier Morales Ferez, Gaspar Delso, Ana Garcia-Alvarez, Jordi Mill Tena, Adelina Doltra, Marta Sitges, Oscar Camara, Bart Bijnens

**Introduction**: Transthoracic echocardiography (TTE) is the gold standard in the assessment of diastolic dysfunction, however, measurement of diastolic markers such as the pulmonary vein (PV) inflow velocities is often challenging. 4D flow cardiac magnetic resonance imaging (CMR) enables quantification of velocities in any target volume without the constraints faced by TTE. We explored the use of 4D flow CMR in generating Doppler-like velocity spectrograms of mitral and PV inflow and comparing them to actual echo-based pulsed-wave (PW) Doppler velocity curves. **Patients and Methods:** A total of 6 HCM patients were included (P1-6). TTE was performed with a GE E95 system. Mitral and PV inflow PW Doppler was performed in the apical 4-chamber view by placing the sample volume at the level of the leaflet tips and at the inflow of the PV. The CMR data was acquired through a GE SIGNA Architect 3.0T scanner. The left atrium (LA), ventricle and outflow tract (LVOT) were segmented from a 4D flow phase contrast angiogram. The sample volume of PW Doppler was imitated using spheres of 4 mm radius placed in the same positions as in TTE. Velocities were projected in the direction typically captured by the echo transducer generating Doppler-like images. The duration of the pulmonary Ar and the mitral A wave was measured using both methods. **Results:****Figure 1** shows volumetric renderings of velocity at mid-systole. Compared to P1 and P2, P3-6 present an accelerated LVOT flow due to obstruction caused by systolic anterior motion, causing posterolateral mitral regurgitation disrupting LA hemodynamics. **Figure 2** demonstrates the mitral and pulmonary inflow patterns obtained with TTE and CMR. Peak velocities and the duration of the A/Ar waves were comparable when quantified using both modalities (**Table 1**). Ar wave duration could be quantified in all CMR studies, showing heterogeneity between the four PVs, whereas AR could be measured in only 3/6 of the echo studies. FIGURE 1. Volumetric rendering of the 4D flow MRI velocity fields mid-systole, including arrows pointing in the direction of the blood flow. A clear LVOT obstruction can be observed in patients 3-6 caused by systolic anterior motion. The distinctive rotational flow pattern in the LA is a consequence of the mitral regurgitation jet. FIGURE 2. The synthetic 4D flow derived PW Doppler velocity curves alongside the authentic PW Doppler. The sample volume for the PV acquisitions was located in the right superior PV, while the mitral valve profiles were extracted from the leaflet tips. ### TABLE 1: AR and A wave duration measurements performed from cardiac magnetic resonance and transthoracic echocardiography images, respectively. | Patient | | 1 | 2 | 3 (AF no A wave) | 4 | 5 | 6 | | --- | --- | --- | --- | --- | --- | --- | --- | | Ar Duration | RS-4D (ms) | 198 | 136 | – | 188 | 180 | 260 | | | RI-4D (ms) | 168 | 97 | – | 229 | 193 | 200 | | | LS-4D (ms) | 168 | 58 | – | 229 | 138 | 260 | | | LI-4D (ms) | 137 | 233 | – | 313 | 346 | 421 | | | RS-Echo (ms) | 185 | 206 | – | – | 220 | – | | Mitral A Duration | MV-4D (ms) | 150 | 191 | – | 123 | 136 | 158 | | | MV-Echo (ms) | 145 | 181 | – | 140 | 156 | 166 | [†] RS - right superior PV; RI – right inferior PV; LS – left superior PV; LI – left inferior PV **Conclusion**: 4D CMR shows potential for blood flow assessment previously feasible only via TTE. CMR could provide a more reproducible assessment of PV inflow velocities, relevant in assessing diastolic function, and potentially reveal new insights about LA hemodynamics.

Vlatka Rešković Lukšić, Karla Pavlović, Marijan Pašalić, Jadranka Šeparović Hanževački

**Background**: Basal septal hypertrophy (BHS) is one of the first signs of concentric left ventricular remodeling in chronic pressure overload such as arterial hypertension (AH) (1). Aim: To investigate if the appearance of BHS in the early course of AH correlates with outcomes in the long-term follow-up. **Patients and Methods**: A total of 138 patients with primary AH, aged less than 65 years and with no comorbidities were included during 2014-2017. Patients were divided into two groups according to BSH presence on the transthoracic echocardiography. Follow-up was performed by checking patients’ hospital data charts and telephone interview. Data concerning antihypertensive drug therapy and cardiovascular morbidity was collected. **Results:** Basal septal hypertrophy was found in half of the patients (53.6%). Mean follow-up period was 91.92±7.20 months. At the time of follow-up, mean age was 56.09±11.68 years, patients with BSH were older (p=0.004). In the whole cohort, mean number of antihypertensive drugs at baseline was 2.01±1.29, in the follow up 1.81±1.14 (**Figure 1**). BSH patients were altogether taking more antihypertensive drugs (2.10±1.26 vs 1.53±0.94, p=0.032), more diuretics (p=0.014), angiotensin converting enzyme inhibitors (p=0.007) and beta-blockers (p=0.004). In the follow-up period, hospitalizations, or referrals to emergency department due to cardiovascular events, stroke or transient ischemic attack, intracranial hemorrhage and newly diagnosed coronary artery disease and atrial fibrillation were noted in both groups, **Figure 2**. Even though those outcomes were more frequent in the BSH group, there was no significant difference, probably due to a small number of included patients and relatively short follow-up period. FIGURE 1. Distribution of antihypertensive drug therapy in the whole cohort. ACEi = angiotensin converting enzyme inhibitors, ARB = angiotensin receptor blockers, CCB = calcium channel blockers, BB = beta-blockers. FIGURE 2. Outcomes in patients with and without basal septal hypertrophy. BSH = basal septal hypertrophy, CV = cardiovascular, CVI = stroke, TIA = transient ischemic attack, SAH = subarachnoid hemorrhage, AFib = atrial fibrillation. **Conclusion:** Appearance of BSH is found to be a macroscopic marker of the incipient regional and global left ventricular remodeling and dysfunction in chronic pressure overload, but it could also be a potential marker of adverse outcomes in the long-term follow-up. Lower total amount of antihypertensive therapy in the follow-up may imply lower patient’s compliance.

Amer Iglica, Edin Begić, Edin Medjedović, Nirvana Šabanović Bajramović, Alen Džubur, Alden Begić, Ivana Lalović

**Aim:** Presentation of a patient with diagnosed eclampsia and peripartum cardiomyopathy (PPCM) and delivered in the intensive coronary care unit (ICCU). **Case presentation:** 27-year-old patient in the 38th week of pregnancy was admitted to the Intensive Coronary Care due to the suspected acute coronary syndrome and clinical picture of pulmonary edema with headache and severe hypertension (180/110 mmHg). An increase in markers of myocardial necrosis was noted, along with proteinuria (+). Reduced ejection fraction of left ventricle (LVEF 40%) with hypokinesis of the inferoseptal, basal, and mid segments of the inferior and inferolateral, wall and symptomatic severe mitral and tricuspid regurgitation was verified by transthoracic echocardiography (TTE). The patient was treated by non-invasive mechanical ventilation and delivered by a gynecologist during the night. Six hours after hospital admission, the patient had convulsions and, due to respiratory arrest, was intubated and connected to controlled mechanical ventilation with midazolam sedation. The patient received therapy with magnesium sulfate to stop convulsions and continued antihypertensive therapy with diuretics. After 24 hours, the patient was successfully extubated after the T-tube test. The post-extubation period passed neat. After 15 days treatment with bromocriptine and beta blocker, angiotensin-converting enzyme (ACE) inhibitor and mineralcorticosteroid antagonist, TTE showed mild decreased systolic function (LVEF 50) % with mild mitral and tricuspid regurgitation. Three months after discharge, control TTE showed global longitudinal strain (GLS) of -19,7%, preserved systolic function (LVEF 63%) and proper valvular function (**Figure 1**). FIGURE 1. Global longitudinal strain of presented patient. **Conclusion:** Eclampsia in peripartum cardiomyopathy can mimic acute coronary syndrome**.** (1, 2) GLS can be a tool for diagnosis confirmation.

Ana Antonić, Koraljka Benko, Tamara Hlača Caput, Ivana Smoljan, Tin Nadarević, Slavica Kovačić, Teodora Zaninović Jurjević

**Case report**: We present a case report of an 82-year-old male patient with known valvular and ischemic heart disease and a history of paroxysmal atrial fibrillation, iatrogenic hypothyroidism and rheumatoid arthritis who was admitted to our Department in August 2018 due to chest pain. Routine echocardiography revealed a large pericardial effusion and pericardiocentesis was performed with instillation of bleomycin. Cytology of the pericardial effusion showed elements of peripheral blood, but there was no evidence of malignant disease. Ten months later he was admitted again due to non-ST elevation myocardial infarction with clinical symptoms and signs of heart failure. Coronary angiogram revealed atherosclerotic disease of left and right coronary artery with significant stenosis of the left main, left anterior descending, left circumflex and right coronary artery. Echocardiography revealed an enlarged right atrium and ventricle with severely thickened interventricular septum and anterolateral wall of the right ventricle which was akinetic and seemed infiltrated as well as adjacent pericardium. A small pericardial effusion was also noted. There were no regional wall abnormalities of the left ventricle, and its ejection fraction was preserved. Additionally, severe aortic stenosis was determined. Magnetic resonance imaging of the heart was performed which showed diffuse myocardial and pericardial infiltrative mass along the right ventricular free wall, right ventricular apex, interventricular septum, and right ventricle papillary muscles. Morphological features were indicative of a cardiac lymphoma. Endomyocardial biopsy was performed. Histopathological analysis of right ventricle samples obtained at endomyocardial biopsy confirmed the presence of a diffuse large B-cell lymphoma. Chemotherapy was initiated with R-mini-CHOP protocol. After the seventh cycle the patient developed febrile neutropenia with signs of heart failure. Echocardiographic findings showed thinner myocardial walls and global hypokinesia with reduced left ventricular ejection fraction. Patient died 7 months after the diagnosis. **Conclusion**: Primary or secondary lymphomas are rare malignancies of the heart, often presenting with atypical symptoms and signs (1, 2). Imaging includes echocardiography, computed tomography and magnetic resonance imaging with final diagnosis that is based upon histopathological analysis of the heart tissue obtained on endomyocardial biopsy. The prognosis is poor, and diagnosis is often obtained post mortem (1, 2).

Ivona Mustapić, Vedran Carević, Anja Mandrapa, Paula Radić, Tea Domjanović Škopinić, Darija Baković Kramarić

**Introduction:** Infective endocarditis (IE) is a life-threatening disease with poor prognosis and high mortality if not diagnosed promptly and intervened early (1). Perianullary extension accounts for nearly 40% of all native valves IE, most commonly the aortic valve, but formation of an intracardiac fistula occurs in less than 1% of all cases (2). **Case report:** We report the case of a 64-year-old man admitted to the intensive care unit because of acute respiratory failure with high fever, high inflammatory blood reactants and electrolyte disbalance. He had previously been extensively evaluated for microcytic anemia due to hemorrhoids, and had also suffered from epilepsy since his youth. A series of blood cultures were obtained and Streptococcus oralis was positive. Because of systolic-diastolic murmur and second-degree atrioventricular conduction disturbance on electrocardiography, transthoracic echocardiography (TTE) was performed. TTE showed the aortic valve with a hyperechogenic mass and severe aortic regurgitation with a jet directed toward the septum and moderate aortic stenosis. However, a 1.5 x 2.2 cm hyperechogenic mass was noted in the right atrium adjacent to the aortic annulus **(****Figure 1****).** Transesophageal echocardiography (TOE) showed a deformed aortic valve with three degenerative leaflets and hyperechogenic mobile vegetations, a circumferential abscess of the aortic annulus with extension of infection toward the right atrium just above the tricuspid septal leaflet and extension of infection toward the left atrium with formation of a fistula detected by color Doppler flow **(****Figure 2****).** The patient was treated with vancomycin and benzilpenciline and referred to cardiac surgery, where the aortic valve was replaced with a biological prosthesis and the aortic root was patched. The postoperative course was complicated by the COVID -19 infection. A series of control blood cultures were sterile. After two months of treatment, the patient was discharged home with normal TTE function of the biological aortic valve **(****Figure 3****)**. FIGURE 1. Transthoracic echocardiography. Three-chamber view with a hyperechogenic mass in the right atrium measuring 1.5 x 2.2 cm. FIGURE 2. Transoesophageal echocardiography. Color Doppler frow from aorta to left atrium. FIGURE 3. Postoperative transthoracic echocardiography: four-chamber view. **Conclusion:** TTE and TOE are invaluable for rapid and accurate diagnosis of the anatomic involvement of IE and its extent, leading to appropriate treatment and thus a better prognosis.

Petra Angebrandt-Belošević, Dejan Došen, Ana Marija Slišković, Eduard Margetić

**Introduction**: Takotsubo cardiomyopathy (TCM), also known as stress cardiomyopathy, or “broken heart” syndrome, occurs in the setting of catecholamine surge from an acute stressor. This syndrome mimics acute myocardial infarction in the absence of coronary disease. The classic feature of this syndrome is regional wall motion abnormalities with characteristic ballooning of the left ventricle. The etiology of the stressor is often physical or emotional stress. Echocardiography identification of wall motion abnormalities is crucial in diagnostic approach. Apical, typical type is most common, but some fewer common variants have been reported as well. (1, 2) **Case report**: We will present 3 patients with unusual clinical presentation of TCM. The first patient had characteristic apical type with depressed mid and apical segments and hyperkinesis of the basal walls (apical ballooning). The second patient had reverse or inverted TCM with hypokinesis of the base with sparing of the midventricle and apex. This type is present in only 2.2% of patients. (1) The third patient manifested as cardiogenic shock where echocardiography revealed typical TCM with left ventricular outflow tract obstruction, which caused reduced cardiac output and signs of sock. (**Figure 1**) All three patients were female and had initially significantly reduced global systolic function of the myocardium. One patient had secondary TCM due to malignancy, and the other two had primary TCM due to extreme emotional stress right before developing symptoms. In follow up period two of three patients had recovered completely in global systolic function and with no other cardiac complications. Control echocardiography for third patient has not jet been made. FIGURE 1. Apical four chamber view of left ventricular outflow tract obstruction. **Conclusion**: There are many aspects of TCM that are still not completely understood, and even though medical society knows about this phenomenon over 30 years, it is only recently gained increased recognition. Although TCM is thought to be a benign condition and most patients recover completely, the recent observation data suggest that cardiogenic shock and and death rates are comparable to patients with acute coronary syndrome. (1) The aim of this abstract was to show less common clinical presentations of TCM and its complications.

Dubravka Šipuš, Luka Perčin, Anica Milinković, Dora Fabijanović, Ivo Planinc, Marijan Pašalić, Nina Jakuš, Hrvoje Jurin, Jure Samardžić, Boško Skorić, Maja Čikeš, Ida Hude Dragičević, Davor Miličić, Daniel Lovrić

**Introduction:** Virus associated hemophagocytic syndrome (VAHS) is severe complication of numerous viral infections that is associated with “cytokine storm” and the accumulation of activated T-lymphocytes and macrophages in various organs, frequently resulting in multiorgan failure and death (1, 2). We present a case report of VAHS caused by Influenza A infection. **Case report:** 50-years old, previously healthy male presented to Emergency Department with fever and respiratory failure. Initial arterial blood gases showed global respiratory failure with acidosis (pH 2 9.3 kPa, pO2 8.7 kPa, lactates 13.5 mmol/L, HCO3- unmeasurable). Computed tomography showed left sided pneumonia, and initial laboratory workup showed severe leukopenia, elevated C-reactive protein, and mild renal lesion (**Table 1**). Polymerase Chain Reaction (PCR) was positive for Influenza A, and Streptococcus Pyogenes was isolated from bronchoalveolar lavage. After initial workup patient arrested and cardiopulmonary reanimation (CPR) with intubation was performed. Post-CPR echocardiography showed severely reduced left ventricular systolic function (LVEF 9) | **0.8** (3.4 - 9.7) | | Neutrophiles (x109) | **0.49** (2.06 - 6.49) | | Lymphocytes (x109) | **0.27** (1.19 - 3.35) | | Platelets (x109) | 120 (158 – 424) | | Troponin I (ng/L) | 8.5 (0 - 34.2) | | Urea (mmol/L) | 8.6 (2.8-8.3) | | Creatinine (umol/L) | **138** (60 – 104) | | C reactive protein (mg/L) < 5 | **268.7** (<5) | | Bilirubin (umol/L) | 12 (3 – 20) | | Alanine-aminotransferase (U/L) | 24 (12 – 48) | | D-Dimers (mg/L) | 4.35 (0-0.50) | FIGURE 1. Imaging methods after admission: A) Chest X-ray after VA ECMO placement showing bilateral extensive confluent, homogeneous infiltrates of the lung parenchyma; B) Computed tomography showing extensive zones of consolidation in the lower lobes of the lungs and large zone of destruction of left lower lung lobe; C) Echocardiography (subcostal view) showing thrombus formation in the left ventricle. **Conclusion:** VAHS is one of rare and potentially lethal complications of Influenza A which can lead to multiorgan failure that can require mechanical circulatory support. Echocardiography plays crucial role in diagnostics and management of critical ill patients.

Dubravka Šušnjar, Igor Rudež

**Introduction**: Unicuspid aortic valve (UAV) is a very rare congenital malformation, with prevalence of 0.02% in the adult population, usually diagnosed already in childhood. It is most often associated with other cardiovascular abnormalities such as aortic stenosis and aortic insufficiency. UAV are classified into two types: the unicommissural type and the acommissural type described based on the absence (or presence) of a lateral attachment of the commissures to the aorta at the level of the orifice (1). **Case report**: 18-year-old female patient with the diagnosis of bicuspid aortic valve (BAV) confirmed in childhood. She reported no family history of cardiac disease or sudden cardiac death. She was without symptoms such as chest pain and syncope, but in last half year she presented with dyspnea during exercise. The patient was under regular cardiological controls with repeated cardiac MRI, which showed a moderate bicuspid AV stenosis, with maximum velocity > 3 m/s, with mild insufficiency. The diameter of the aortic root is 34mm, STJ 29mm, ascending aorta 35mm. The left ventricle is of adequate size with preserved global systolic function (EF 60%). There were no other complications, such as ascending aortic dilatation, patent ductus arteriosus, and coarctation of the aorta which are often accompanied by UAV. Transthoracic echocardiography (TTE) showed marginally changed aortic valve leaflet (**Figure 1**), visible cuspis raphae with lateral commissure, confirmed to be unicuspid, with maximum velocity 3.48 m/s, and maximum gradient PPG 48.49 mmHg. The aortic stenosis was moderately severe with a valve area AVA (VTI) of 1.4 cm2. Moderate aortic insufficiency, with a vena contracta of 7 mm and a pressure half time of 350 milliseconds, was found with eccentric regurgitant flow to the left ventricle’s posterior wall. Findings on the transesophageal echocardiographic confirmed UAV stenosis with insufficiency. Patient underwent aortic valve surgery, intraoperative finding confirmed UAV. Bioprosthetic aortic valve (Edwards Inspiris Resilia 23 mm) was implanted, with good function on control echocardiography. FIGURE 1. The parasternal long and short axis view showed an unicuspid aortic valve. **Conclusion**: The diagnosis of UAV can be made with use of two-dimensional or three-dimensional TTE or TEE, cardiac computed tomography, or cardiac magnetic resonance imaging. With echocardiography, a UAV is best imaged during systole, because the absence of cusp separation during aortic valve opening reveals the classical eccentric “teardrop” opening (2). In our case, transesophageal echocardiographic TEE proved to be superior to MR.

Amer Iglica, Edin Begić

**Aim**: To present a patient diagnosed with Yamaguchi syndrome (apical nonobstructive hypertrophic cardiomyopathy). (1, 2) **Case presentation**: The patient was referred for transthoracic echocardiography with suspected ischemic heart disease, with electrocardiographic findings of left ventricular hypertrophy and T wave inversion in the chest as well as the limb lead. Echocardiographic finding of left ventricle showed regular dimensions. From the mediobasal segment to the apex interventricular septal end diastole diameter was 3.1 cm, with left ventricular posterior wall end diastole diameter was 3.2 cm, with preserved ejection fraction of left ventricle of 50%, while diastolic function was altered by the type of prolonged relaxation (tissue Doppler-derived E/ e’ ratio 15.2) (**Figure 1**). There was no evidence of ventricular outflow tract obstruction, even after the Valsalva maneuver. Multi-Slice Computed Tomography coronary angiography showed no obstructive coronary artery disease. FIGURE 1. Yamaguchi syndrome. **Conclusion**: Electrocardiographic Yamaguchi syndrome is characterized by signs of left ventricular hypertrophy and giant (>10 mm in amplitude) negative T waves most prominent in V4-5 chest lead. Concentric hypertrophy of the apex is treated with beta-blockers or calcium channel blockers to control the heart rate and angiotensin-converting enzyme (ACE) to reduce left ventricular after-load.