Cardiologia Croatica. 2019;14(3-4)

Mario Ivanuša

Cardiovascular diseases (CVD) are the leading cause of mortality and account for 31% of total deaths globally according to data from the World Health Organization. An analysis of standardized mortality rates per 100,000 inhabitants found that in Italy, a country with one of the healthiest populations in the world, CVD mortality rate is 2.5 times lower than in Bosnia and Herzegovina. With this in mind, it is not surprising that the editors of the book “Kardiovaskularne bolesti i komorbiditeti” (eng. Cardiovascular Diseases and Comorbidities) activated as many as 36 experts – specialists and subspecialists from the fields of internal medicine, anesthesiology, surgery, and other fields. Most co-authors work at clinical hospitals in Tuzla and Sarajevo, and two authors are employed outside Bosnia and Herzegovina. Which novelties does this work bring in comparison with existing and well-known books and reputable handbooks? The 554 pages of text in the book can be grouped into three thematic units: patient states that represent an emergency and require immediate treatment, stratification of preoperative risk, and identification and evaluation of the significance of risk factors or comorbidities. Each of the 20 or so units are discussed using a trilateral approach – from the perspective of the patient, general practitioner, and consulting physician. Furthermore, important topics such as effective interpersonal communication, teamwork, and cooperation in medicine supplement this edition and provide a holistic approach to this topic. Knowledge, skills, and technological capabilities are important for clinical cardiology. One everyday example of both experience and optimal communication being crucial to prognosis are requests for medical consultations, i.e. consultant examinations. As more than half of hospitalized patients have two or more chronic diseases and are at an advanced age, medical consultations are most commonly performed by internists, cardiologists, and anesthesiologists, so it comes as no surprise that specialization in these fields is predominant among the coauthors for this book. The stratification of cardiovascular or respiratory risk, optimization of cardiologic therapy, or regulation of glycaemia represent the most common clinical questions. A skillful consulting physician will, like a virtuoso pianist brilliantly interpreting the opus of Frédéric Chopin, know how to properly approach the patient, understand the clinical question, determine the level of urgency, and be as available as necessary. Their recommendations on careful monitoring of the disease, performing additional diagnostic processing, and pharmacological, interventional, surgical, or rehabilitation treatment will be based on their acquired knowledge, experience, and competencies, but must also be adequately communicated. Regardless of the clinical scenario in which the consultant examination takes place, whether it is an intensive care unit, a department, hemodialysis, emergency room, or just telephone cooversation, a successful consultant examination benefits the patient and engenders positive feelings in the medical professional. The skill of performing a consultant examination is therefore worth developing and improving, which is not possible without adequate knowledge that is described in a practical fashion in this book. The publication of this book represents a great advancement for cardiology in Bosnia and Herzegovina and in the region as a whole, commensurate to the creation of the first all-encompassing course book in cardiology, published in 2006. The book “Kardiovaskularne bolesti i komorbiditeti” does not simply represent another expanded list of differential diagnoses in internal medicine/cardiology. By addressing issues from everyday clinical practice in a clear and conceptually developed way, this book instructs us on whether we should approach the patient probabilistically, prognostically, or pragmatically, and is very applicable in everyday clinical practice.

Mihajlo Kovačić, Marko Hranilović

**Introduction**: Stress echocardiography is the combination of 2D echocardiography with physical, pharmacological or electrical stress. The diagnostic endpoint of the detection of myocardial ischemia is the induction of a transient change in regional function during stress. (1) Myocardial ischemia results in a typical “cascade” of events in which the various markers are hierarchically ranked in a well-defined time sequence. Flow heterogeneity between subendocardial and subepicardial perfusion is the forerunner of ischemia, followed by metabolic changes, alteration in regional mechanical function, and only at a later stage by electrocardiographic (ECG) changes, global left ventricular dysfunction (LV) and pain. (2) Wall motion and perfusion or coronary flow reserve (CFR) changes are highly accurate, and more accurate than ECG changes for detection and location of underlying coronary artery disease. However, wall motion is more specific and requires ischemia; perfusion changes are more sensitive and may occur in the absence of true ischemia (microvascular disease, or LV hypertrophy). (1) The three most common ischemic stressor are exercise, dobutamine, and vasodilators (dipyridamole, adenosine). They are equally potent for inducing wall abnormalities in the presence of a critical epicardial coronary artery stenosis. Dobutamine and exercise manly act through increased myocardial oxygen demand. Vasodilators act by stimulating A2 adenosinergic receptors present on the endothelial and smooth muscle cells of coronary arterioles and induce ischemia due to reduced subendocardial flow supply subsequent to inappropriate arteriolar vasodilatation and steal phenomena. (1) Dipyridamole was the first pharmacological stress agent used for the diagnosis of coronary artery disease, with a pioneering indication proposed in Europe for the identification of ischemia during 12-lead ECG (3), and later in the USA by Lance Gould as hyperemic stress perfusion imaging. (4) Dipyridamole stress echocardiography pioneered in the year of 1985 (Picano et al). The safety record of high dose dipyridamole is well established on the basis of large scale multicentric studies with data from thousands of patients. The incidence of major complications, i.e. myocardial infarction, atrioventricular block, cardiac asystole, sustained ventricular tachycardia, is about 1/1500 cases. The mortality rate is about 1/10000 cases, similar to that of the exercise stress test. Dipyridamole has a better safety profile than when stress testing with dobutamine, where the incidence of major adverse reactions is about 1/300 studies. (5) Many centers from 1988 until today, have used high doses administered in a short time (syringe-based infusion of 0.84 mg/kg). Later, some studies were reproduced with these doses, and today, most centers worldwide use high dose delivered in 4-6 min. (6) **Center experience:** In 2017 we started to perform stress echocardiography in Čakovec County Hospital. The first method was exercise echocardiography using treadmill protocol, with imperative do accomplish post-exercise imaging as soon as possible (<1 min from the cessation of exercise) where patient moved immediately from treadmill to imaging bed. With this method we have information from exercise stress testing combined with echocardiographic wall motions analysis to make final interpretation. The second method and most used until recently in our echo lab is standard dobutamine stress protocol, starting with 5 ug/kg/min and increasing 10, 20, 30 and 40 ug/kg/min. If no endpoint is reached, atropine, usually in dose 0.25 mg, is added to maximal dobutamine dose infusion. We are using this method for coronary disease detection, but also low dose protocol, to asses myocardial viability, valvular pathology (low-flow aortic stenosis) and other indications. Recently we started vasodilator stress echocardiography using dipyridamole, and until today, we performed a test on 15 patients. Protocol diagram is showed in **Figure 1**. The dipyridamole dose employed for stress testing was 0.84 mg/kg administered in short time (syringe-based infusion at a rate of 0.21 mg/kg/min during 4 minutes). A fast protocol is embraced because is known that high dose protocols in short time causes a three- to fourfold increase in coronary blood flow in normals and give best sensitivity and specificity for coronary pathology detection (7). Peak vasodilatation occurs 1 to 2 minutes after the end of infusion and the dipyridamole elimination half-time is 40 minutes which enables us enough time for imaging in the period of maximum stress. After image completion we give the antidote aminophylline (blocks adenosine receptors) even in negative cases. The antidote can also be used for emergent reversal of adverse dipyridamole-related events which we have not encountered in this limited series of tests. If technically feasible due to image quality, we perform measurement of coronary flow reserve in mid to distal left anterior descending artery (LAD) using pulse doppler (**Figure 2** and **Figure 3**) before and after vasodilatation. CFR in many cases adds additional diagnostic value when combined with conventional wall motion analysis. Another tool to increase diagnostic accuracy, especially when image quality is impaired, are contrast agents (i.e. Optison) which help to delineate endocardial borders. FIGURE 1. Dipyridamole stress echo timeline. FIGURE 2. Distal left anterior descending artery – color doppler. FIGURE 3. Distal left anterior descending artery – PW max diastolic velocity. All caffeine-containing foods (coffee, tea, chocolate, bananas, cola drinks) should be avoided for 12 hours before test and all theophylline-containing drugs (aminophylline) should be discontinued for at least 24 hours before test. Very low dose protocols can be used (0.28 ug/kg) to identify myocardial viability and have high specificity (higher than dobutamine) but lower sensitivity (8). Absolute contraindications for dipyridamole stress test are active bronchospasm, 2nd or 3rd degree AV block and systolic blood pressure <90 mmHg. Relative contraindications are methylxanthine use and remote history of restrictive airway disease. **Conclusion**: Dipyridamole stress echocardiography can be used as a preferred stress test for the detection of coronary artery disease in patients unable to exercise and with contraindications to dobutamine (tachyarrhythmias, uncontrolled blood pressure). It is technically easier than exercise or dobutamine stress tests since image quality is less degraded by tachycardia, hyperventilation and hypercontractility. The test is equally accurate in comparison with dobutamine, but technically easier and safer (9). In the future, dipyridamole test is planned to be the default method for pharmacological stress in our laboratory supported with contrast agents when necessary. Contrast perfusion stress echocardiography is the next planned step in years to come.

Marko Hranilović, Branko Ostrički, Mihajlo Kovačić

**Introduction:** Assessment of myocardial viability can be done with a variety of imaging techniques, including nuclear, magnetic resonance and echocardiographic methods. Stress echocardiography offers higher specificity for postoperative ejection fraction improvement than perfusion techniques due to the requirement of viable myocardium with preserved contractile reserve. (1, 2) Low dose dobutamine is the preferred method for viability assessment. Myocardial revascularization without detected viability may be considered according to guidelines but portends worse prognosis (3) and in this case, if feasible, left ventricular assist devices and heart transplantation may be considered. Dipyridamole in very low doses can be used for viability detection and may be considered due to a better safety profile than dobutamine in patients with uncontrolled hypertension or tachyarrythmias (4). **Case report:** 64-years-old male patient, formerly treated for hypertension, type 2 diabetes mellitus and peripheral vascular disease (conservative therapy) was admitted to coronary care unit due to chest pain with modest troponin elevation. Coronary angiography was urgently performed and revealed highly significant stenoses in proximal parts of all coronary vessels with high syntax score (**Figure 1**, **Figure 2**). Immediate referral to the cardiac surgeon was suggested by an interventional cardiologist but upon completion of diagnostic procedure pulmonary edema was induced due to contrast infusion and hypertensive reaction (RR 180/100). Despite urgent treatment with parenteral nitrate, loop diuretics, morphine and non-invasive ventilation significant lactacidosis with pH 7.1 and acute respiratory failure ensued (SO2 72%). Bedside echocardiography showed left ventricular ejection fraction (LVEF) 30% with hypo/akinesia in all vascular territories. Patient was sedated, intubated and mechanically ventilated for a few hours. When he was clinically stabilized heart team opted for viability assessment in order to plan treatment (in case of no viability detected left ventricular assist device as a bridge to heart transplantation would be an option). Serial troponins showed only modest elevation above reference values. We opted for a low dose dobutamine protocol (up to 10 mcg/kg/min) when the patient was normotensive and clinically stable and verified LVEF increase to 54% (by Simpson method measuring 2- and 4-chamber with auto LVEF by speckle tracking) (**Figure 3**). The patient was transferred to cardiac surgery and had received LIMA to LAD and RIMA to PD which was a part of the right dominant coronary artery. Subsequently we documented good functional recovery (stress ECG test 4 months later was stopped after 6 minutes of Bruce protocol due to peripheral claudication with no chest pain or dyspnea and without significant ECG changes). Echocardiography 10 months after revascularization showed LVEF of 55% with normokynesia in all segments of myocardium. MSCT angiography verified bilateral femoral artery occlusion. With walking distance of 500 meters he has not been scheduled for operative/interventional treatment at this point in time and has completed hospital cardiac rehabilitation programme. FIGURE 1. Coronary angiography (LAO caudal). FIGURE 2. Coronary angiography (LAO cranial). FIGURE 3. Left ventricular ejection fraction in 2-chamber apical view after 10 mcg/kg/min dobutamine infusion. **Conclusion:** Different imaging modalities can be used for viability detection in severely depressed left ventricular function. Pharmacological stress imaging has higher specificity for improvement of ejection fraction after revascularization than perfusion-based tests and may be done if the risk is perceived acceptable as it was in our patient after clinical stabilization.

Josip Vincelj, Sandra Jakšić Jurinjak, Ida Vuglec

**Introduction:** Cor triatriatum sinister (CTS) is a very rare congenital cardiac malformation in which the left atrium (LA) is divided into two chambers by a fold of tissue, a membrane, or a fibromuscular band. The anomaly is usually diagnosed in childhood, but in adult age is less common. Clinical symptoms can mimic mitral stenosis. (1-5) **Case report:** We report a case of 54-year-old woman referred to our hospital for transesophageal echocardiography (TEE). She had in history of dyspnea, headache, dizziness and effort intolerance for five years. Physical examination and laboratory values were unremarkable. Two-dimensional and three-dimensional transesophageal echocardiography revealed fibromembranous structure in the dilated LA (**Figure 1** and **Figure 2**). The membrane attached laterally to the junction of the left upper pulmonic vein and left atrial appendage, and medially to the interatrial septum. The membrane divided LA into two chambers (proximal chamber and distal chamber). Proximal chamber was receiving the pulmonary veins, and distal chamber contained left atrial appendage and mitral valve orifice. We found few fenestrations connecting the two chambers (**Figure 3**). Multislice computed tomography (MSCT) confirmed diagnosis of CTS (**Figure 4**). Coronary angiography revealed normal coronary arteries. The patient was referred to surgery following a TEE and MSCT diagnosis of CTS. The atrial membrane was excised around its periphery. Recovery from the surgery was uneventful and she was asymptomatic on further hospital stay and follow-up. FIGURE 1. Two-dimensional transthoracic echocardiogram parasternal long-axis view showing a fibromuscular membrane dividing the dilated left atrium into two chambers. FIGURE 2. Transesophageal echocardiogram demonstrating a fibromuscular membrane in the left atrium. FIGURE 3. Three-dimensional transesophageal echocardiogram showing membrane in the left atrium. FIGURE 4. Multislice computed tomography showing a membrane dividing the left atrium into two chambers. **Conclusion:** The diagnosis of cor triatriatum sinister is paramount because of possibility of surgical repair with excellent long-term prognosis. 3D TEE is noninvasive method for comprehensive imaging and correct diagnosis of this rare congenital cardiac malformation. Surgical repair is an easy and definitive treatment choice of CTS should be considered in patients with left heart chamber obstruction symptoms.

Ante Matana, Teodora Zaninović Jurjević, Slavica Kovačić

Left ventricular (LV) hypertrophy in patients with aortic stenosis (AS) is an adaptive mechanism that tries to maintain left ventricular systolic function despite obstruction. (1) The appearance of myocardial fibrosis (MF) is a turning point from adaptive hypertrophy to maladaptation, that changes ventricular function and represents the basis for development of ventricular arrhythmias. There are two types of MF: reversible interstitial MF in early stage, and irreversible replacement MF in the later stage of disease. MF occupies LV midwall, usually in LV posterobasal part. Cardiac magnetic resonance (CMR) by late gadolinium enhancement (LGE) is the „gold standard“ in diagnosis of replacement MF. Positive LGE results indicate significantly greater risk of adverse outcome than it is in patients without MF. Echocardiography suggests the presence of MF with significant reliability, because global longitudinal strain (GLS) shows good correlation with results of CMR. Consequently, GLS can serve as a surrogate marker of MF and an indicator of adverse events in AS. (2-4) MF mostly influences LV longitudinal function, while circumferential and radial deformation are less modified. In severe compensated AS circumferential deformation and apical rotation (ApR) are increased, what is likely a compensative mechanism for keeping cardiac output. The increased ApR is also associated with worse survival. With disease progression these compensative changes disappear. Mechanical dispersion (MD) is an indicator of contractile heterogeneity caused by fibrosis. Increased MD demonstrates significant association with mortality and can serve as an additional prognostic parameter. Along with more extensive MF the risk for sudden cardiac death increases what raises the question of the benefit of cardioverter-defibrillator. In conclusion, impaired GLS suggests MF, what must be confirmed by CMR. MF classifies the patients with severe asymptomatic AS into the group with increased risk of adverse outcome. Therefore, it is to be expected that these new facts will result in new guidelines for treatment of severe asymptomatic AS.

Fatmir Ferati, Anida Ferati, Mentor Karemani, Ardian Preshova

**Introduction**: The purpose of the study is, analysis of the right ventricular (RV) function in patients with pulmonary embolism proven by the clinical presentation and CT angiography of the pulmonary arteries, which are positive in this disease. Verification of echocardiography changes in these emergency cases, enables early diagnosis of this condition as well as its efficient treatment. (1, 2) **Methods and Results**: A total of 20 patients with pulmonary embolism, 59 years old (59 +/- 11 years) were screened, based on the clinical observation and visualization of thrombotic formations in the pulmonary arteries, with CT angiography. The group of persons without cardiovascular disease is consisted of a total of 50 people, 55.3 years old (55.3 +/- 17), that were selected randomly. In **table 1** results of the RV function in the population without a verified cardiovascular disease have been taken. A significant increase of EDV of RV (157ml vs. 79ml) was recorded, registered by 3D measurements, in the group with PE (**Figure 1**). Reduction in the EF of RV in the PE group (57.3% vs 27.8%), was recorded in the first days. TAPSE of RV, is reduced in the PE group (18.6 mm vs. 10.5 mm) along with the reduction of (FAC) of RV (47.66 vs. 22.11%). LSS (septal wall) shows its reduction from -18.9% to -12.8%, while the free wall LS (LSF) function is much more pronounced in the group of pulmonary PE patients, and it has been reduced from -31.5% to -11.6% in the PE group, which is one of the echocardiographic characteristics of the PE (**Figure 2**). ### TABLE 1: Results of the right ventricular function in the population without a verified cardiovascular disease. | | **EDV of RV in ml** | **EF of RV** **(%)** | **TAPSE RV mm** | **Fac** **(%)** | **Septal LS (-%)** | **Free wall LS (-%)** | **Basal** **dimension of RV (mm)** | | --- | --- | --- | --- | --- | --- | --- | --- | | b.o | 79.14 | 57.25 | 18.6 | 47.66 | 18.89 | 31.49 | 31.24 | | TE pulmonum | 157.367 | 27.86 | 10.57 | 22.11 | 12.81 | 11.668 | 39.72 | FIGURE 1. Pulmonary TE in acute phase (3D RV function). FIGURE 2. The same case in a chronic phase and with improvement in the hemodynamic condition. **Conclusions:** Based on the data obtained from our study, we can conclude: **1**) In pulmonary PE patients, there is a dilatation RV, which is more accurately quantified with 3D echocardiography, than with 2D echocardiography. **2**) Decrease of EF to RV in patients with pulmonary PE was registered. **3**) In patients with pulmonary PE, there is a decrease in TAPSE and FAC of RV. **4**) The longitudinal strain (LS) of septal and free wall of RV are decreased. **5**) We have a much more significant reduction in the LS free wall of RV(LSF), which is one of the PE characteristics. **6**) The 2D strain decreases in the middle and apical level of the free wall LS, which is one of the characteristics of the pulmonary PE. **7**) In cases of suspicion of pulmonary presence, the regional kinetics of VD, especially the Free wall should be analyzed, which would enable early diagnosis with a high probability.

Sandra Jakšić Jurinjak, Ivana Jurin, Josip Vincelj, Diana Delić-Brkljačić

**Introduction**: Right ventricular (RV) function and cardiac involvement in systemic sclerosis (SSc) is important factor for the prognosis of SSc but often remains undetectable despite echocardiographic screening. (1, 3) Speckle derived strain (2D-STE) of the right ventricle (RV GLS) was utilized to detect subclinical abnormalities in regional and global contractility in SSc patients with no echocardiographic signs of pulmonary arterial hypertension. Aim of pilot study was to study the advantages of 2D speckle-tracking echocardiographic derived parameters in identifying RV dysfunction in SSc patients for quantifying myocardial deformation and conventional RV indexes in patients with SSc and to investigate whether these could be indicative of right heart failure or can be used as non-invasive methods of screening in SSc. **Methods and Results**: 27 SSc patients (mean age, 54.3 years; 96% female) with technically adequate echocardiograms were studied. Standard morphological measurements of RV chamber function, tricuspid annular plane systolic excursion (TAPSE), fractional area change (FAC), tricuspid tissue Doppler annular velocities (TDIs,), right ventricular myocardial performance index (RIMP) and global longitudinal right ventricular free wall strain (RV FW GLS) were obtained. Twenty-two patients without pulmonary arterial hypertension (PAH) due to systemic sclerosis were studied. When we used the cutoff value recommended by the American Society of Echocardiography Guidelines to identify abnormal RV function (4), patients were determined to have normal RV function, TAPSE 21.9 (±2.21), RV FAC 49.4 (±3.45), RIMP 0.44 (±0.08) respectively. Global longitudinal strain (LS) of the RV was calculated by averaging the LS value of 3 segments of the RV free wall in RV focused apical 4-chamber view (**Figure 1**). 40.9% of those patients had abnormal RV FW GLS (-14.8%) more pronounced in the basal regional longitudinal strain. FIGURE 1. Global longitudinal strain (LS) of the right ventricle (RV) calculated by averaging the LS value of 3 segments of the RV free wall in RV focused apical 4-chamber view. **Conclusion**: Right ventricular strain reveals a diverse pattern of regional strain in SSc that is not detected by conventional measures of function, suggestive of subclinical RV myocardial disease and could be used as non-invasive method in screening for PAH in SSc to select patients eligible for right heart catheterization or to monitor the effects of PAH therapy. (1-3)

Silvija Miletić Gršković, Vedran Buršić, Adrian Trobonjača, Viktor Peršić

**Case report**: 46-years-old patient with positive family anamnesis of cardiovascular diseases was presented in Special Hospital Magdalena in January 2017 with fatigue and chest pain. Cardiology work up revealed dilated cardiomyopathy (EF 20%), moderate mitral regurgitation and thrombus in left ventricle. On a second day of hospitalization patient was diagnosed with left popliteal artery embolism. After the treatment with low molecular heparin, thrombus was dissolved, and popliteal artery was recanalized. He continued anticoagulation therapy with warfarin. During hospitalization another complication occurred. Left sided pneumonia was successfully treated with azithromycin and ceftriaxone. Beside his cardiological problems, hepatic lesion was also verified. The etiology of hepatic lesion was probably alcohol abuse as it was for dilated cardiomyopathy (1). In February 2018. invasive coronarography showed anomalous origin of left anterior descending artery from right coronary sinus with intact coronary arteries. Echocardiography was performed and there was an improvement of left ventricular ejection fraction (50%) and a sign of mild mitral regurgitation. In our hospital, MSCT coronarography verified anomalous origin of one left anterior descending artery from right coronary sinus, at the level of the exit of right coronary artery, with „bridging“ along the whole proximal segment. Left main coronary artery exits from left coronary sinus from where arises (except circumflex artery) another left anterior descending artery running in normal anatomic position along left ventricle with all developed branches, septals and diagonals (2). Magnetic resonance imaging showed dilated cardiomyopathy with ejection fraction 40%; nonspecific, stationary deficit of perfusion in intraventricular septum and inferior wall with typical pathological imbibition of myocardium, “midventricular type“, in post-contrast sequences. This sign is typical for toxic, dilated cardiomyopathy. This heart with coronary arteries is 3D printed.

Tatjana Zekić Varljen, Damir Raljević, Vesna Pehar Pejčinović, Viktor Peršić, Vladimir Peša, Ingrid Buljanović

**Background**: Tachycardiomyopathy is an illness that is caused by accelerated and/or irregular cardiac rhythm, which result in myocardial damage and heart failure. There are no clear criteria for diagnosing this condition, but patient have weakness of the heart muscle with presence of some forms of heart rhythm. Non-compaction cardiomyopathy is disease which is result of a disturbance in the early phase of myocardial left ventricular development with deep recessions between trabeculae and abnormal trabeculation/hyperproliferation. This disease is associated with left ventricular failure, cardiac arrhythmias, embolic events, and sudden cardiac death. (1, 2) **Case report**: 39-year-old man with history of palpitation, ventricular arrhythmias, now complaining of tachycardia, palpitation and fatigue, was hospitalized in our Hospital for treatment of episode of paroxysmal supraventricular tachycardia which was converted in sinus rhythm with adenosine. Patient was not treated with any medical therapy before. Electrocardiography was shown episode of supraventricular tachycardia (PSVT) with short RP interval which was encouraged with ventricular extrasystole with retrograde conduction. We were suspected at atrioventricular circular tachycardia with retrograde conduction with aberrant beam. Echocardiography detected non-compaction cardiomyopathy with low left ventricular ejection fraction (LVEF), without element of coronary artery disease. Considering the earliest echocardiography findings with preserved LVEF, we conclude that with the existence of non-compaction cardiomyopathy, the tachycardiomyopathy was followed up to an additional reduced LVEF. With administered drug therapy followed by a significant reduction in the number of episodes of supraventicular tachycardia, the patient was discharged with the recommendation of taking verapamil, sacubitril/valsartan, eplerenone, dabigatran and we recommended ablation. After ablation, echocardiography findings that has been done, have shown global hypocontractility with preserved contractility posterolateral and better LVEF. After three months of ablation we made control magnetic resonance wich has shown discretely dimension reduction of left ventricle and still non-compaction cardiomyopathy with global hypokinesia but discreet recovery of LVEF and better feeling of patient. **Conclusions**: The current clinical case have shown that the tachycardia weakened the global LVEF in this young man, who also have non-compaction cardiomyopathy, which was slightly improvement when we manage the fast tachycardia with drugs and ablation.

Marija Mance, Ivan Bitunjac, Richard Matasić, Kristina Gašparović, Petra Lepetić, Sandra Večerić, Martina Lovrić Benčić

**Introduction**: Patients with symptomatic chronic heart failure (sCHF) and implanted cardiac resynchronization device (CRTD) were included in this retrospective study in order to evaluate possible differences in mitral (MR) and tricuspid (TR) valvular regurgitation severity, NYHA class and left ventricle ejection fraction (LVEF) between male and female CRTD patients during follow up of 6 months after CRTD implantation. **Patients and Methods**: We included 135 patients (89 men, 46 women) with sCHF due to any cause with implanted CRTD according to guidelines with optimal medical therapy regardless of atrial fibrillation or in sinus rhythm. Clinical (NYHA class) and echocardiographic data (valve pathology, LVEF) were assessed before and 6 months after CRTD implantation. Using color and continuous wave Doppler, PISA, regurgitation volume and vena contracta measurements, mitral regurgitation (MR) and tricuspid regurgitation (TR) were stratified in 3 grades. Echocardiography was recorded always by the same echo-technician to avoid inter-observer variability. **Results**: Mean age of patients was 60 (±10) years. The results showed improvement in LVEF from 25% up to 36% (p=0.006) in all patients regardless of sex and age, as well as worsening of TR (p<0.001) and no difference in MR (p=0.195). In male patients (N=89), significant worsening in MR and TR was present (p<0.05) while there was no significant worsening in MR (p=0.42) nor TR (p=0.06) in female patients (N=46). In patients older than 60 years (N=82, female 27 (33%), male 55 (67%)), as well as younger than 60 years (N=53; female 19 (36%), male 34 (64%)) significant worsening of TR grade was observed (p<0.05), while the grade of MR remained the same (p=0.255 and p=0.534). An improvement in NYHA class was observed in 75 patients (71%), no change in 25 patients (24%) and worsening in 5 patients (5%). Overall improvement in NYHA class was statistically significant (p<0.001). **Conclusion**: Six months after CRTD implantation the severity of MR and TR remained the same in female patients while significant worsening of MR and TR severity was found in male patients. TR worsening might be due to implanted electrodes. The age of patients had no impact on LVEF, TR and MR change. Despite improvement in clinical status and NYHA class, the echocardiography results did not meet our expectations. (1-4) Due to limited number of patients the results were not divided according to the etiology of CHF.

Vlatka Rešković Lukšić, Marijan Pašalić, Jadranka Šeparović Hanževački

**Background:** Hypertensive patients with basal septal hypertrophy (BSH) are known to have high filling pressures comparing to healthy controls, eventually leading to diastolic heart failure and poor outcomes. (1-3) Aim: To investigate if occurrence of basal septal hypertrophy is related to diastolic disfunction in younger patients with uncomplicated arterial hypertension (AH) and no comorbidities compared to hypertensive patients with no BSH. **Patients and Methods**: A total of 138 patients aged 18-65 years (mean 48.51±11.69 years), 47.8% men were enrolled. Complete 2D transthoracic echocardiography according to standardized protocol was performed, as well as deformation study using 2D speckle tracking echocardiography. All patients included in the study had a preserved left ventricular ejection fraction (mean 61.35±5.07%). Basal septal hypertrophy was defined as basal/mid septal thickness ratio >1.3, or thickness of the basal interventricular (iv) septum >2mm compared to mid iv septum, along with visual BSH notification. **Results**. There were altogether 74 patients (53.6%) with BSH. Patients in this group were older (51.41±9.37 vs 45.16±13.19 years, p=0.006), with longer duration of AH (9.01±9.67 vs 5.95±8.51 years, p=0.009). Echocardiographic measurements revealed normal left ventricular (LV) geometry and left atrial (LA) volumes, as well as normal values of standard indices of diastolic function in the whole population. However, patients with BSH showed incipient concentric LV remodeling and relaxation impairment (RWT 0.38±0.11 vs 0.3±0.08, p2, p<0.001; E/A 0.96±0.28 vs 1.16±0.39, p=0.001; LAVI 32.68±9.98 vs 30.02±11.37, p=0.05). There were no differences among groups according to global deformation measurements. Longitudinal strain was more impaired in three basal LV segments (basal iv septum -11.38±4.37% vs -14.69±2.54%, p<0.001; mid iv septum -16.44±3.54 vs -18,16±2.9%, p=0.01; basal anteroseptum -13.38±4.19 vs - 15.24±3.23%, p=0.01), correlating with indices of diastolic function shown in **Table 1**. ### TABLE 1: Correlations between regional longitudinal strain and parameters of diastolic function. | | **E/A** | **E’ sept** | **E/E’sept** | | --- | --- | --- | --- | | **LSBAS** | r=-0.249, p=0.011 | r=-0.363, p=0.001 | r=0.228, p=0.021 | | **LSBS** | r=-0.329, p=0.001 | r=-0.387, p=0.001 | r=0.26, p=0.007 | | **LSMS** | r=-0.211, p=0.029 | r=-0311, p=0.001 | r=0.224, P=0.022 | [†] LSBAS=basal anteroseptum longitudinal strain, LSBS= basal septum longitudinal strain, LSMS=mid septum longitudinal strain. **Conclusion**. BSH is shown to be an early sign of LV relaxation impairment, along with incipient geometry and regional longitudinal deformation changes even in younger patients and in early stages of AH.

Jana Ljubas Maček, Marija Mance, Marijan Pašalić, Vlatka Rešković Lukšić, Ivo Planinc, Nina Jakuš, Boško Skorić, Jure Samardžić, Daniel Lovrić, Maja Čikeš, Hrvoje Jurin, Dora Fabijanović, Davor Miličić, Jadranka Šeparović Hanževački

**Background:** A new hemodynamic environment is set up after heart transplantation (HTx). (1, 2) Our aim was to assess changes in diastolic function of the left (LV) and right (RV) ventricle in HTx patients (Pts) during the 1st year and the influence of donor age, graft ischemic time (GIT) and acute right ventricular failure (ARVF). **Patients and Methods:** In 55 “healthy” HTx Pts echo was performed 1- and 12 months after HTx. Data on mitral valve (MV) E- (Ew) and A-wave velocity (Aw), MV and tricuspid (TV) E/A ratio, MV and TV E’ and A’ by tissue Doppler, septal E/E’, pulmonary vein diastolic velocity (PVd), systolic pulmonary pressure (sPAP), left atrial indexed volume (LAVI), right atrial pressure (RAP) were collected. **Results:** Ew significantly decreases during the 1st year (0.80 vs. 0.73 m/s; p=0.036) as well as E/E’ (11.5 vs. 9.1; p=0.009), PVd (0.67 vs. 0.55 m/s; pst month have higher Ew (0.90 vs. 0.75 m/; p=0.021), E/A (2.2 vs. 1.7; p=0.034) and E/E’ (14 vs. 10; p=0.047) but after 1-year only elevated mitral E/A (2.2 vs. 1.6; p=0.014), without change in LAVI (p=0.095). Higher donor age (> mean of 41 years) caused initially lower MV-E’ (r=-0.390, p=0.010) and higher sPAP (r=0.285; p=0.045). GIT had no influence on diastolic parameters. ARVF significantly correlated with lower TV-A’ acutely and after 1 year (ARVF 5 vs. no-ARVF 8 cm/s; r=-0.600, p= 0.011) and higher RAP (r=0.414, p=0.003). **Conclusion:** During the 1st postransplant year there is improvement in LA diastolic function with better early passive LV filling and reduction in PAH, without change in active filling phase or LA volume. In Pts with significant PAH mean E/E’ ratio within 1st month was 14, but after a year in Pts with persistent PAH it declines to only 10, preserving the higher E/A ratio. Higher donor age could impair the early LV ventricular filling with a trend toward more PAH but MV-E’ improves with time. ARVF reduces active RV filling (TV-A’) throughout the 1st year. GIT does not have influence on diastole.

Zvonimir Ostojić, Vlatka Rešković Lukšić, Marija Mance, Blanka Glavaš Konja, Martina Lovrić Benčić, Maja Strozzi, Joško Bulum, Jadranka Šeparović Hanževački

**Aim**. To investigate early outcomes after balloon aortic valve valvuloplasty (BAV) performed as palliative procedure in patients with decompensated unstable end-stage aortic stenosis (AS). **Patients and Methods**. A retrospective observational study was conducted in University Hospital Centre Zagreb. It included all patients who underwent lifesaving BAV, and had no options for surgical or transcatheter aortic valve replacement at the time, between 2015 and 2018. Clinical and echocardiographic characteristics before and after BAV procedure were recorded. **Results**. Study included 29 patients, mean age 80.2 (51-92) years, 65.5% women. Mean logistic Euroscore II was 24.1±21.1% and STS score 16.9±14.5%. Comorbidities included: coronary artery disease (48.28%), diabetes mellitus (31.4%), chronic renal insufficiency (58.6%), peripheral artery disease (17.2%) and atrial fibrillation (55.2%). All patients were dependent on parenteral diuretics and 24.14% on parenteral inotropic support. Echocardiographic parameter before and after BAV are presented in **table 1**. There was significant increase in indexed aortic valve area and decrease in pulmonary artery systolic pressure in overall population. There was no significant change in severity of aortic insufficiency. In-hospital mortality was 20.1% (N=6). Periprocedural complications included: 1 (3.4%) myocardial infarction, 1 (3.4%) major bleeding, 1 (3.4%) acute kidney injury and 2 (6.9%) vascular access complications. All other patients have been successfully weaned of parenteral therapy within 4 days and discharged. ### TABLE 1: Echocardiographic parameters before and after aortic valve balloon valvuloplasty. | | Before BAV (n=29) | After BAV (N=25) | P value | | --- | --- | --- | --- | | Ejection fraction (%) | 35.6±12.3 | 36.9±13.8 | 0.691 | | E/E´ | 25.7±8.8 | 25.1±13.2 | 0.821 | | Maximal pressure gradient - mmHg | 71.1±42.1 | 56.8±31.1 | 0.134 | | Mean pressure gradient – mmHg | 41.7±24.1 | 31.1±14.9 | 0.055 | | Indexed aortic valve area | 0.341±0.008 | 0.399±0.12 | **0.030** | | Any aortic insufficiency N (%) | 22 (75.9) | 21 (84) | 0.459 | | More than mild mitral insufficiency N (%) | 18 (62.1) | 17 (68) | 0.649 | | Pulmonary artery systolic pressure - mmHg | 64.8±27.1 | 46.8±17.5 | **0.001** | [†] BAV = balloon aortic valve valvuloplasty **Conclusion**. BAV represents palliative treatment option in critically ill hemodynamically unstable patients with end-stage AS, who are not candidates for valve replacement. (1) BAV provides clinical improvement due to increase AVA and decrease in PAPs, with acceptable mortality and complications rate.

Zina Lazović, Nermir Granov, Behija Hukeljić Berberović, Omer Perva, Lejla Divović

**Introduction**: Ascending aortic pseudoaneurysm is a rare, sometimes fatal complication after aortic surgical procedures. Contrast computed tomographic scan is the investigation of choice. Transesophageal echocardiography is helpful before, during and after treatment of pseudoaneurysm. Surgical treatment of pseudoaneurysm is often considered like treatment of choice but is associated with high morbidity and mortality. Sometimes transcatheter closure may be an effective treatment in selected patients. (1, 2) We describe the successful management of a pseudoaneurysm of the ascending aorta which was united with fistula between superior vena cava and aortic pseudoaneurysm. **Case report**: 71-year-old male came to hospital with superior vena cava syndrome which manifested in facial swelling, neck distension, and enlarged veins of the upper chest, which developed two days before admission. One year ago, he had aortic valve replacement with biological valve, mitral valve repair, plastic of tricuspidal valve and implantation of the pacemaker. Chest computed tomography showed pseudoaneurysm dimension 85x57x65 mm on right lateral contour of the ascending aorta. The neck of pseudoaneurysm was 17 mm in diameter. In the area of ​​dorsal contour of pseudoaneurysm sachets was communication with a vena cava superior in the sense of fistula. A transesophageal echocardiography exam shows pulsatile flow between aorta and pseudoaneurysm. After a heart time discussion, the percutaneous approach was undertaken. In the Hybrid operating room under transesophageal echocardiography and fluoroscopic guidance the Amplatzer duct occluder device was placed in the neck of pseudoaneurysm, but day after procedure control transesophageal echocardiography showed flow right next to device between pseudoaneurysm and aorta. The high velocity blood flow move Amplatzer device. Next day patients has open chest surgery with pseudoaneurysmectomy, reconstruction of ascending aorta and reparation defect of the superior vena cava. Several days after surgery the facial and neck swelling was disappeared, and patient felt better.

Alma Sijamija, Nermir Granov, Alma Agačević, Omer Perva, Nedžad Hadžić

**Background**: Patent foramen ovale (PFO) is an anatomical interatrial communication with potential for right-to-left shunt. It is detected in 10-15% of the population by transthoracic echocardiography (TTE) and up to 27% by autopsy. Most patients with isolated PFO are asymptomatic. Patients may have a history of stroke or transient ischemic event of undefined etiology. (1, 2) The recurrence rate of stroke or TIA has been reported to be as high as 3.4-3.9% per year. In patients with atrial septal aneurysm and PFO, the risk of first recurrent stroke within 2 years has been reported to be as high as 9%, whereas the rate of subsequent stroke or TIA recurrence within 2 years increases to 22%. No consensus exists on treatment of PFO in patients with transient ischemic attack (TIA) or stroke. Traditional treatment has been antiplatelet therapy alone in low-risk patients or combined with warfarin in high-risk individuals to prevent cryptogenic stroke. (3, 4) **Case report**: We represent three cases of PFO diagnosed in our hospital within past 14 months. Case 1: 34-year-old man hospitalized at the Neurology Department due to transient loss of consciousness. Echocardiography showed interatrial septal aneurysm, and two septal defects – subaortal, 11mm in diameter with left-to-right shunt and second one, 3mm in diameter. There was also visible smaller thrombotic mass in left auricle. Patient underwent surgical closure. Case 2: 39-year-old woman presented at the routine echocardiographic examination after she was discharged from Neurology department where she was treated for stroke. Her brain MRI showed multiple ischemic infarctions with no focal deficit on neurological examination. TTE showed small PFO, 3mm in diameter with right-to-left shunt (**Figure 1****,****Figure 2**). Patient received percutaneous surgical closure. Case 3: 63-year-old man presented to the internist after loss of consciousness. ECG showed atrial fibrillation and TTE was subsequently performed. It showed atrial septal aneurysm, a septal defect, 14mm in diameter with left-to-right shunt (**Figure 3**, **Figure 4**). The patient is on the waiting list for surgical treatment. FIGURE 1. Patent foramen ovale. FIGURE 2. Patent foramen ovale with right-to-left shunt. FIGURE 3. Atrial septal aneurysm. FIGURE 4. Atrial septal defect with left-to-right shunt. **Conclusion**: Surgical closure PFO has resulted in elimination of residual shunt across the PFO. Advantages include the following: permanent closure of the defect, prevents future paradoxical emboli, no long-term anticoagulation and its risks.

Antonia Melada, Ivona Mustapić, Mijo Meter, Josip Katić, Viktoria Lišnić, Zora Sušilović Grabovac, Ante Anić, Darija Baković Kramarić

**Introduction:** The left atrial appendage (LAA) is the most common place of thrombosis in patients with atrial fibrillation (AF). Numerous studies have shown that oral anticoagulation (OAC) significantly reduces the risk of thromboembolism. However, there are no recommendations regarding how to treat cardioembolic recurrent strokes when patients are well anticoagulated. (1-3) **Case report:** 68-years-old male with permanent non-valvular AF, currently taking apixaban, was hospitalized for the second time due to recurrent ischemic cerebrovascular stroke. At the time of his first presentation six months ago, he was well anticoagulated with warfarin (international normalized ratio was 3.56) and have had a CHA2DS2VASc score 2. He had no significant carotid disease or mobile aortic arch atheroma. Transesophageal echocardiography (TEE) revealed a significantly dilated left atrium (LA) with dense spontaneous echo contrast (SEC). There was no organized thrombus in the LA nor in the LAA. The contractile function of the LAA was severely decreased, with peak systolic velocity of 33 cm/s on Doppler evaluation. Despite taking effective anticoagulant medications for both times our patient experienced recurrent ischemic stroke and yet again had dense SEC in the LA and LAA. In order to prevent upcoming cardioembolic event, we decided to preform percutaneous LAA closure with Amplatzer Amulet device under TEE guidance. Successful LAA closure was confirmed by color Doppler imaging and a single postocclusion angiography. The patient was discharged with OAC (warfarin) in addition of 100 mg/day of acetylsalicylic acid to prevent thrombus formation on device. Follow up TEE was performed one month after the procedure. Good position of LAA occluding device was confirmed with no evidence of thrombus formation on the left atrial face of the device. **Conclusion:** In general, after implantation of LAA occluding device, OAC is not indicated. However, combination therapy with indefinite OAC plus LAA closure in patients with AF with recurrent strokes despite good anticoagulation should be considered in order to prevent a new stroke.

Ljupka Dimitrovska, Bor Antolič, Rok Zbačnik, Matjaž Šinkovec, Jana Ambrožič

**Background:** Intracardiac echocardiography (ICE) is an established imaging modality for guiding electrophysiology procedures, with a rapidly expanding role also in other percutaneous structural interventions. Endomyocardial biopsy (EMB) is another potential application where ICE can be used to guide diagnosis of cardiac tumors. (1-3) Here we present our first case of a cardiac tumor in which EMB was guided by ICE. **Case report:** 62-year-old man with a history of non-Hodgkin lymphoma treated with radiation and chemotherapy 10 years ago was admitted due to acute coronary syndrome. Coronary angiogram showed a subtotal occlusion of already collateralized RCA. Due to induced ischemia on myocardial perfusion imaging postponed percutaneous coronary intervention of RCA was performed. During diagnostic work-up at admission when pulmonary embolism was at first suspected, CTA was performed. It showed a heterogeneous formation in the right ventricle (RV) with a thickness of up to 16 mm, that extended from the base towards the apex and into the interventricular septum at the level of the tricuspid ring (**Figure 1**). According to the CT appearance the mass was suspected to be a liposarcoma. Transthoracic echocardiography showed a hypoechogenic form in the RV, clearly defined after application of contrast for left ventricular opacification. Due to previous history of malignancy PET CT was done that revealed hypometabolic RV mass without any sign of distant metastasis. Percutaneous biopsy of the cardiac mass for histopathological diagnosis was performed guided by ICE and fluoroscopy. ICE allowed continuous imaging of intracardiac structures and clear visualization of the cardiac mass in the RV outflow tract under the pulmonary valve (**Figure 2**). On angiography there was no evidence of flow restriction. A cardiac biopsy catheter was percutaneously inserted into the RV and with the guidance of ICE accurate positioning of the bioptome was achieved. Three tumor specimens were taken for analysis. The histology revealed lipoma, with no evidence of malignancy. In the absence of any cardiac functional disturbances surgical therapy could be avoided and follow-up was proposed. FIGURE 1. CTA revealed a heterogeneous formation (arrow) in the RV that extended from the base towards the apex and into the interventricular septum at the level of the tricuspid ring. FIGURE 2. Intracardiac echocardiography reveals tumor mass (arrow) in right ventricle outflow tract. View from right ventricle towards right ventricular outflow tract. **Conclusion:** Cardiac masses are rare and often incidental findings. Histology provides definite diagnosis and ICE could be a valuable tool for guiding biopsy procedure. Our first experience confirmed the benefits of ICE that are real-time visualization of cardiac structures, facilitating proper positioning of biopsy catheter, good patient tolerability and the lack of need for general anesthesia. Therefore, with ICE guided percutaneous biopsy the more invasive diagnostic open-heart procedures could be avoided.

Siniša Roginić, Jelena Zajec Gorički, Marija Čajko, Krešimir Štambuk

**Introduction**: Secondary mitral regurgitation (MR) is a dynamic myocardial disease accompanying cardiomyopathy and coronary artery disease. (1-4) **Case report**: We present a case of 63-year-old patient with long standing cardiomyopathy after breast cancer chemotherapy. Her left ventricle (LV) is slightly dilated with moderately impaired systolic function (estimated EF 30%) and severe diastolic disfunction. Significant MR (**Figure 1**, **Figure 2**) has been present for years but patient’s condition deteriorated rapidly with frequent admissions for heart failure despite optimal medical therapy. Upon last discharge echocardiography showed slight improvement in LV systolic function and persistent severe secondary MR. She has no significant coronary artery disease or indication for CRT. FIGURE 1. Parasternal long axis view showing dilated left ventricle and significant mitral regurgitation into dilated left atrium. FIGURE 2. Mitral quantification using the proximal isovelocity surface area (PISA) method – effective regurgitant orifice area (EROA) 0.5 cm2, right ventricle (RV) 57 ml. **Discussion and Conclusion**: Severe secondary MR is in most cases treated conservatively, especially in the absence of other surgical indication. According to guidelines mitral valve intervention (surgery or transcatheter procedure) may be considered in refractory cases after heart team discussion. Our patient was subsequently scheduled for mitral valve replacement.

Mario Milun

**Case report**: Female patient was admitted to the Cardiology Department with the symptoms of inappetence, dyspnea, and acute heart failure. She was 83-year-old woman who had single chamber right ventricular pacemaker (PM) implanted in 2006 because of a complete AV block, and reimplanted again in 2014. She had a long history of hypertension, atrial fibrillation, and was diagnosed with pulmonary embolism in 2015. Upon admission she had a radiologically and clinically verified signs of heart failure. The ECG showed a permanent pacing. Echocardiogram verified preserved global and segmental left ventricular (LV) systolic function, with mild LV diastolic dysfunction. Systolic function of the right ventricle was normal, with dilatation of the right chambers of the heart. Tricuspid valvular ring was dilatated, and there was a „loop“ of the pacemaker lead moving freely through the valve, with massive tricuspid regurgitation. Systolic pulmonary artery pressure was 45 mmHg. Patient was treated with diuretics and was soon clinically better. Because of her fragile health it was decided not to pursue cardiosurgical intervention, or to change the pacemaker lead. **Conclusion**: A mild or moderate TR is a common echocardiographic finding in patients with PM and has been reported as high as 53%. Such a prevalence of mild or moderate TR is expected since the lead crosses the tricuspid valve and may slightly impair its closure. Severe lead induced tricuspid regurgitation (LITR) is very rare. Usually results from implantation damage to tricuspid valve (perforation or laceration of the tricuspid leaflets) or lead interference with the tricuspid valve leaflets. It can also be due to an abnormal activation of the right ventricle with a delayed activation of the apex and the papillary muscles. Fibrosis adherences between lead and tricuspid valve have been described and may cause delayed severe TR. Diagnosis is based on symptoms, clinical examination, echocardiogram, cardiac CT, or MRI. Therapeutic approach can be conservative or surgical. (1-3)

Ivana Lukić, Marul Ivandić, Sandra Makarović

**Introduction**: An ascending aortic aneurysm is defined as localized dilatation of aorta. We classify thoracic aortic aneurysms into aortic root or ascending aortic aneurysms (most common, ≈60%), followed by descending aortic aneurysms (≈35%) and aneurysms of aortic arch (<10%). It is a disease of insidious progression which is initially mostly asymptomatic. Clinical presentation depends on the size and rate of growth of the disease itself, which sometimes makes it difficult to recognize until first complications arise, and it is unfortunately in many cases initially presented by aortic dissection. (1, 2) **Case report**: 53-year-old patient without previous medical history reports to the hospital because of shortness of breath and occasional chest tightness, which he notices for the past two months. Clinically there is audible diastolic murmur next to the left edge of sternum and peripheral congestion. Laboratory tests excluded acute coronary syndrome, NT-proBNP was elevated at 2013 ng/L. Echocardiographically we verified significant dilated ascending aorta size (75-84-82-80 mm) with consequently severe aortic regurgitation (AR PHT 112 ms) and dilated left ventricle with reduced systolic function (LVEF 36%). CT angiography excluded dissection and confirmed echocardiographic test findings of ascending aorta. Coronary angiography was performed, epicardial blood vessels were without stenosis. This patient was presented to cardiac surgeon and upon completion of preoperative assessment, Bentall procedure was performed – reconstruction of ascending aorta and a replacement of the aortic valve with a composite graft. Postoperative progress went without complications, and the subsequent pathohistological test result was unremarkable. **Conclusion**: Our case presentation is interesting and unusual because of the clinical presentation of the gigantically dilated aorta which is often manifested by dissection. Here it was camouflaged by clinical picture of the initial heart failure which in its basis had critical phase of the underlying disease. These findings should be taken into consideration and carefully looked into in everyday medical practice.

Josip Katić, Mijo Meter, Antonia Melada, Ivona Mustapić, Viktoria Lišnić, Darija Baković Kramarić

**Introduction:** Electric shock is the set of pathophysiological events resulting from the action of an electric current on the human body (1). Electrical injuries involve both direct (caused by contact with electricity arc) and indirect mechanisms (mechanical trauma due to falls). The most common sequels after electrical injury in the heart are arrhythmias and myocardial contusion (2). Large arteries like aorta are rarely affected because their rapid flow allows them to dissipate the heat produced by the electric current. However, they are susceptible to medial necrosis, with aneurysm formation and rupture at a later point of time. There is one case in the literature which described aortic valve rupture due to high-voltage electrical injury (3). **Case report:** 47-year-old male patient was admitted to our echo lab for ultrasound examination of the heart. In the last two months he had been complaining about exertional dyspnea. His medical history was quite poor: he had never been admitted to the hospital until two months ago when he had been observed for 24 hours after he had suffered a high-voltage electric injury. The transthoracic echocardiography was performed, and we discovered moderately enlarged left ventricle (LVED volume 180 ml) due to severe aortic regurgitation on bicuspid aortic valve with holo-diastolic retrograde flow. The regurgitation jet that filled the two thirds of left ventricular outflow tract with laceration and flail of bicuspid cusps confirmed on transesophageal ultrasound. The patient underwent surgery. The aortic valve was removed and replaced with a mechanical prosthesis. **Conclusion:** Cardiac symptoms and echocardiographic findings that initiated two months after a high-voltage electric injury led us to conclusion that bicuspid valve was probably injured by electrical shock. To the authors’ knowledge, this is the first reported case of valvular rupture due to electrical injury in Croatian population.

Marija Mance, Vlatka Rešković Lukšic, Blanka Glavaš, Martina Lovrić Benčić, Joško Bulum, Zvonimir Ostojić, Jadranka Šeparović Hanževački

**Introduction**: Age and gender may influence the incidence of aortic regurgitation (AR) and its severity. Significant aortic regurgitation (sAR) is often treated surgically especially when symptomatic or when systolic function declines. (1-3) The aim of this study was to evaluate the outcomes in patients with sAR according to treatment strategy, age and gender differences in our study population. **Methods**: In this retrospective descriptive single-center study an overall of 107 patients (22 female, 85 male) with significant AR in the last 5 years were analyzed. Patients were treated according to valid recommendations, surgically (SUR) or conservatively (CON), except for 5 patients who refused surgery. Baseline and follow up (FU) data (AR severity, left ventricular ejection fraction (LVEF), ascending aorta diameter (AA), treatment, comorbidities and major adverse cardiac events during FU), from documented medical history and digital imaging data were collected and analyzed. Additional sub-analysis was performed according to sex and age differences (above vs. below the age of 50). For statistical analysis a Chi-Square test was used. **Results**: In the overall study population, during an average FU of 3.8 years, 16 patients (15%) developed major adverse cardiac events (MACE) with no statistically significant difference between gender (p=0.846). Forty-six (43%) patients were surgically treated (87% male, 13% female) and 61 (54%) conservatively. LVEF did not worsen in FU period (54.1%, vs. 53.8%). In SUR, median age was 54 years, severe AR was present in 93%, incidence of MACE was 21.7%, 80.4% patients were symptomatic and 14.5% had dilatation of AA more than 50 mm. In CON, MACE was present in 9.8% during FU (p=0.87), median age was 64 years. Intermediate AR (48% vs 6.5%) and AA from 40-49 mm (80 vs 35%) was present more frequently as well as arterial hypertension (82 vs 70%) and chronic renal disease (23.2 vs 16.6%). The incidence of MACE was not found to be age-related (p=0.426). **Conclusion**: In patients with sAR treated by either surgery or medication therapy only, during 3.8 years of FU, LVEF remained unchanged, while incidence of MACE was not found to be related to treatment strategy nor gender. In surgically treated patients, as expected, AR was more severe and AA was more dilated, while neither age nor gender had an impact on the incidence of MACE.

Mario Ivanuša, Domagoj Ivanuša, Vlatka Rešković Lukšić, Jadranka Šeparović Hanževački

Comparably to almost every scientific discipline or field of study, medicine has also drastically revolutionized its methods and diagnostics throughout the last centuries. One of the most important milestones in the field of cardiology was the development of cardiological diagnostic test and procedures. The conceptualization of transthoracic echocardiography (TTE) was brought forward already 65 years ago when Edler and Hertz noted down movements of the heart walls “in the normal and in the diseased heart” by using 2,5MHz transducer in vivo connected to the cathode-ray-tube screen. (1) The penetration of TTE method has significantly changed the course of cardiology in the XX and XXI century, due to the vast amount of data gathered through non-invasive and non-harmful applicability used for creating a diagnostic and therapeutic impact. Nowadays, TTE and its use have been framed through a standard protocol (2), indications (3) and guidelines (4) published on a constant basis by different professional societies (**Table 1**). In the upcoming years, alongside the increase of the volume, the development of TTE will be further stimulated by: a) growing demand for handheld or portable echocardiography devices, b) increased use of automated function imaging and c) emergence of 5D imaging technology. ### TABLE 1: State-of-the-art overview of standards/clinical practice guidelines created by professional societies for transthoracic echocardiography. | • EACVI Compendium – comprehensive compilation of all its recommendations, position papers and consensus statements published in the EHJ-CVI journal. EACVI E-Store. https://www.escardio.org/Sub-specialty-communities/European-Association-of-Cardiovascular-Imaging-(EACVI)/Education/E-Store | | --- | | • Mitchell C, Rahko PS, Blauwet LA, Canaday B, Finstuen JA, Foster MC, et al. Guidelines for Performing a Comprehensive Transthoracic Echocardiographic Examination in Adults: Recommendations from the American Society of Echocardiography. J Am Soc Echocardiogr. 2019 Jan;32(1):1-64. https://doi.org/10.1016/j.echo.2018.06.004 | | • Galderisi M, Cosyns B, Edvardsen T, Cardim N, Delgado V, Di Salvo G, et al; 2016–2018 EACVI Scientific Documents Committee; 2016–2018 EACVI Scientific Documents Committee. Standardization of adult transthoracic echocardiography reporting in agreement with recent chamber quantification, diastolic function, and heart valve disease recommendations: an expert consensus document of the European Association of Cardiovascular Imaging. Eur Heart J Cardiovasc Imaging. 2017 Dec 1;18(12):1301-1310. https://doi.org/10.1093/ehjci/jex244 | | • Wharton G, Steeds R, Allen J, Phillips H, Jones R, Kanagala P, et al. A minimum dataset for a standard adult transthoracic echocardiogram: a guideline protocol from the British Society of Echocardiography. Echo Res Pract. 2015 Mar 1;2(1):G9-G24. https://doi.org/10.1530/ERP-14-0079 | | • Jeanrenaud X, Seiler C, Attenhofer Jost C, Kaufmann B, Gruner C et al; Board of the Working Group Echocardiography and Cardiac Imaging of the Swiss Society of Cardiology. What is a standard transthoracic echocardiogram performed by a cardiologist? MÉDECINE CARDIOVASCULAIRE. 2015;18(4):146–151. http://www.swisscardio.ch/DOCS_PUBLIC/Empfehlungen_Richtlinien/What_is_a_standard_transthoracic_echo_2015.pdf | | • Lancellotti P, Płońska-Gościniak E, Garbi M, Bucciarelli-ducci C, Cosyns B, Cardim N, et al. Cardiovascular imaging practice in Europe: a report from the European Association of Cardiovascular Imaging. Eur Heart J Cardiovasc Imaging. 2015 Jul;16(7):697-702. https://doi.org/10.1093/ehjci/jev116 | | • Popescu BA, Stefanidis A, Nihoyannopoulos P, Fox KF, Ray S, Cardim N, et al. Updated standards and processes for accreditation of echocardiographic laboratories from The European Association of Cardiovascular Imaging: an executive summary. Eur Heart J Cardiovasc Imaging. 2014 Nov;15(11):1188-93. https://doi.org/10.1093/ehjci/jeu057 | Current research shows that there is still room for improvement regarding the optimal use of TTE when it comes to patients with acute myocardial infarction, heart failure, arrhythmias, stroke, sepsis or in critically ill patients. This could be partially regarded to the fact that 35-50% of patients will repeat their TTE within one to three years from the first examination but this will yield any significant change in the outcome of patients only in less than a third of cases. (5) Even though the TTE is a low-risk, the consequences of over-use could be false positive tests, downstream utilization of resources, and an increase in overall costs. There are a few methods of addressing over-testing: a) clinical practice guidelines (available in both hard copy form and on the Internet; **Table 1**), b) appropriate use criteria (6-8) (the nomenclature for appropriate use categorization: appropriate, may be appropriate, and rarely appropriate procedures; **Table 2**), c) changes in financial incentives, d) education and training, e) national campaigns and public awareness, f) change in assessment of the value of diagnostic testing, etc. Recently in order to improve all aspects of the overall health care service, British Society of Echocardiography has published The Echocardiography Quality Framework (9). Alongside, the quality of echocardiography these methods also include reproducibility and consistency, education and training, and customer feedback. ### TABLE 2: An overview of top articles on the topic of the appropriate use of the criteria for echocardiography. | • Doherty JU, Kort S, Mehran R, Schoenhagen P, Soman P, Dehmer GJ, et al. ACC/AATS/AHA/ASE/ASNC/HRS/SCAI/SCCT/SCMR/STS 2019 Appropriate Use Criteria for Multimodality Imaging in the Assessment of Cardiac Structure and Function in Nonvalvular Heart Disease. J Am Coll Cardiol. 2019 Feb 5;73(4):488-516. https://doi.org/10.1016/j.jacc.2018.10.038 | | --- | | • Doherty JU, Kort S, Mehran R, Schoenhagen P, Soman P. ACC/AATS/AHA/ASE/ASNC/HRS/SCAI/SCCT/SCMR/STS 2017 Appropriate Use Criteria for Multimodality Imaging in Valvular Heart Disease. J Am Coll Cardiol. 2017 Sep 26;70(13):1647-1672. https://doi.org/10.1016/j.jacc.2017.07.732 | | • Fonseca R, Jose K, Marwick TH. Understanding decision-making in cardiac imaging: determinants of appropriate use. Eur Heart J Cardiovasc Imaging. 2018 Mar 1;19(3):262-268. https://doi.org/10.1093/ehjci/jex257 | | • Steeds RP, Garbi M, Cardim N, Kasprzak JD, Sade E, Nihoyannopoulos P, et al; 2014–2016 EACVI Scientific Documents Committee; 2014–2016 EACVI Scientific Documents Committee. EACVI appropriateness criteria for the use of transthoracic echocardiography in adults: a report of literature and current practice review. Eur Heart J Cardiovasc Imaging. 2017 Nov 1;18(11):1191-1204. https://doi.org/10.1093/ehjci/jew333 | | • Chambers JB, Garbi M, Nieman K, Myerson S, Pierard LA, Habib G, et al. Appropriateness criteria for the use of cardiovascular imaging in heart valve disease in adults: a European Association of Cardiovascular Imaging report of literature review and current practice. Eur Heart J Cardiovasc Imaging. 2017 May 1;18(5):489-498. https://doi.org/10.1093/ehjci/jew309 | | • Douglas PS, Garcia MJ, Haines DE, Lai WW, Manning WJ, Patel AR, et al. ACCF/ASE/AHA/ASNC/HFSA/HRS/SCAI/SCCM/SCCT/SCMR 2011 Appropriate Use Criteria for Echocardiography. J Am Soc Echocardiogr. 2011 Mar;24(3):229-67. https://doi.org/10.1016/j.echo.2010.12.008 | In March 2019, Nucleus of the Working Group on Echocardiography and Cardiac Imaging Modalities of the Croatian Cardiac Society has investigated the frequency of the echocardiography follow-ups in different clinical case scenarios. The anonymous online questionnaire was distributed by e-mail to subscribers of the kardio.hr newsletter, who were voluntarily requested to answer on 10 questions. The questionnaire was comprised of two parts – routine procedures in the echocardiography laboratory (first five questions) and the perceived frequency of the need for repeating TTE follow-ups (second five questions). The routine procedures have been analyzed with the length of the echocardiography experience, order for procedures, indications for the first and the control examination and the frequency of follow-ups as explanatory variables. Moreover, the perceived frequency of the need for repeating the TTE check-ups has been investigated through several possible clinical scenarios such as patients with arterial hypertension, mitral valve prolapse, after an acute myocardial infarction as well as patients with the aortic stenosis or implanted aortic valve.

Alma Sijamija, Nermir Granov, Omer Perva, Lejla Granov Aladjuz, Berka Begović, Nedžad Hadžić

**Introduction:** Subvalvular aortic stenosis, which may have a dynamic component, may be due to a fibrous membrane, muscular obstruction, or a combination of the 2 (1, 2). About 50% of patients with a subaortic membrane also have leakage of the aortic valve. Left ventricular outflow tract (LVOT) obstructive lesions account for approximately 6 percent of cases of congenital heart disease in children; the incidence was estimated to be 6 in 10,000 live births (3, 4). Subvalvar aortic stenosis (AS) is the second most common form of AS. Among children with congenital AS, subvalvar AS accounts for 10 to 14 percent of cases (4). Subvalvar AS is more common in males, who account for 67 to 75 percent of cases (4). Patients with severe or untreated subaortic stenosis may be at risk for sudden cardiac death. An echocardiogram will show the level and severity of the obstruction. It will also show if the left ventricle is thickened or enlarged. The progression is often very slow. This is especially true in people whose obstructions are not detected until they are adults. Surgery may be necessary to stop the progression of subaortic stenosis. **Case 1:** 25-year-old female patient was admitted on our department, resuscitated and afterward intubated due to ventricular fibrillation. Her condition was additionally complicated by allergy reaction and signs of acute respiratory distress syndrome. In the age of 6 verified CHA. TTE show hypertrophic obstructive cardiomyopathy (HOCM) with PGmean 76mmHg and PGmax 142mmHg in LVOT. Two months later she underwent surgical operation of septal myectomy. 10 years later she is healthy and gave two births. **Case 2.** 36-years-old female patient, growth with developmental difficulties, adipose with amaurose. Within the preoperative preparation for cholecystectomy operation was diagnosed for HOCM (**Figure 1**, **Figure 2**), subvalvular subaortic membrane (**Figure 3**), with high gradients of subaortic stenosis, PG mean 54mmHg in the LVOT and mild aortic valve regurgitation (**Figures 4-6**Figure 5Figure 6). After cholecystectomy operation, she was scheduled for surgical correction of HOCM. FIGURE 1. The concentric left ventricular hypertrophy; IVSd: 1.7cm. FIGURE 2. The concentric left ventricular hypertrophy; IVSd: 2.3cm. FIGURE 3. The presence of a subaortic membrane (small white arrow). FIGURE 4. A subaortic membrane with severe stenosis gradient, moderate aortic regurgitation. FIGURE 5. The presence of aortic regurgitation. FIGURE 6. Quantification of aortic regurgitation using continuous wave Doppler. **Conclusion:** Although the development of left ventricular hypertrophy or aortic regurgitation is a clear indication for operation, the timing of intervention in the otherwise asymptomatic patient remains a point of controversy.

Dubravka Šušnjar, Sandra Jakšić Jurinjak, Boris Starčević, Josip Varvodić, Davor Barić, Daniel Unić, Robert Blažeković, Igor Rudež

**Case report:** 29-year-old male underwent aortic root reconstruction with root remodeling technique and external ring annuloplasty (Corneo Extra Aortic Ring A 29, Gelweave graft 30 mm) in 2015 due to bicuspid aortic valve with significant aortic regurgitation and aortic root dilatation. In 2017 due to pseudoaneurysm of aortic root and severe aortic regurgitation, the patient was reoperated and mechanical aortic valve was implanted (Carbomedics Mechanical A 25) with patch plastic of the pseudoaneurysm. A year later he was admitted again, now due to fever and high inflammatory markers. Multimodality imaging, transthoracic echocardiography, transesophageal echocardiography, MSCT aortography and abdominal CT described aortic /perivalvular root abscess with significant paravalvular leak in terms of hemodynamically significant regurgitation with high flow velocity over the mechanical valve, peak velocity > 4 m/s. TEE (2D+3D) showed the septate hyperechogenic formation with hypoechogenic cavities which seemed to touch a part of trigonum, approximately 15 mm thick, extending from annulus ascending to the entire visible part of the aortic root, ascending more than 4 cm. It appeared to affect > 50% of the annulus, with visible paraannular leak and massive aortic regurgitation. Previously implanted patch plastic on aortic root was hypermobile depending on heart cycle. Left ventricle showed normal contractility. MSCT of thorax and aortography confirmed the finding (**Figure 1**). The patient was diagnosed with endocarditis of the mechanical aortic valve and aortic root abscess, thus the antimicrobial therapy was started. Patient had to undergo urgent surgical reoperation. Aortic root replacement with coronary artery reimplantation was performed (sec Bentall, BioIntegral Surgical A 23). There were no signs of paravalvular leak on the control MSCT aortography, with proper flow through graft, coronary artery and supra-aortal branches. Control echocardiography showed a good function of the mechanical valve. Patient recovered successfully and was sent home after antimicrobial therapy protocol was finished. FIGURE 1. A MSCT aortography showing complex aortic root abscess, B 3D TEE of aortic root showing patch plastic protruding into aortic lumina, C, D multiplane transoesophageal echocardiography showing paraannuluar aortic abscess with paraannular regurgitant jet. **Conclusion**: Cooperation of the heart team (cardiologist, heart surgeon, radiologist) and multimodality imaging is a paramount for accurate diagnosis and management of patients with complex aortic pathology (1, 2).

Dubravka Šušnjar, Sandra Jakšić Jurinjak, Ilko Vuksanović, Mario Udovičić, Josip Vincelj, Igor Rudež

**Case report:** 56-year-old male patient was admitted due to fever of unknown origin. Upon admission, the patient was in bad general condition with high values of inflammatory markers in laboratory results and signs of right side heart failure. 12-lead ECG showed nonspecific conduction disorders. No signs of systemic disease have been found with extensive internal and diagnostic treatment. Scintigraphy with labeled leukocytes, as attempt to find origin of infection did not show any pathological accumulation. Coronarography excludes atherosclerotic changes in epicardial vessels. Transthoracic (TTE) and transesophageal (TEE) echocardiography described a visible hyperechogenic formation in a right ventricular outflow tract (RVOT), oriented towards pulmonic valve, 1.6 cm long and 0.3 cm wide (**Figure 1**). Right ventricle (RV) showed milder reduced systolic function, with signs of right-side congestion. There was moderate pulmonary valve regurgitation (PR 2+), and mild tricuspid regurgitation with estimation of right ventricular systolic pressure of 27 mmHg. Preserved left ventricular fraction of 58% was observed. MSCT of thorax identified a strange metal body, resembling to sewing needle in the RV area. The patient initially refused the surgical procedure of foreign body extraction, until the clinical condition deteriorated. He was hospitalized again with fever, hem culture positive on Escherichia coli. Transthoracic echocardiography now showed a formation of 8 mm, possibly vegetation, on the ventricular surface of the pulmonary valve with severe pulmonary insufficiency, severe tricuspid regurgitation and moderate right-side heart failure. Diagnosis of pulmonary valve endocarditis has been established. The patient was successfully operated, the bioprosthetic pulmonary valve was implanted combined with tricuspid valve repair and the foreign body was removed from the right ventricle. Postoperative recovery went well, and control echocardiography showed a good function of the bioprosthetic pulmonary valve and tricuspid valve repair. Foreign body was a sewing needle, but the patient could not remember how did it get there. FIGURE 1. A, B 3D-Transoesophageal echocardiography of foreign body in right ventricular outflow tract. B, C 3D-Transthoracic echocardiography revealing foreign body in right outflow tract. **Conclusion**: Isolated right ventricular heart failure can be caused by pulmonary valve insufficiency (1, 2), as in our case by foreign body in RVOT finally complicated by endocarditis of pulmonary valve.

Ivana Petrović Juren, Sandra Prša, Andreja Čleković-Kovačić, Vlasta Soukup Podravec, Kristina Milevoj Križić, Renata Ivanac Janković, Gabrijela Bašković

**Introduction**: Takotsubo (stress cardiomyopathy/transient apical ballooning/broken heart syndrome) is disorder associated with transient left ventricular disfunction. It usually occurs in older women, 50% of them with neurologic or psychiatric conditions, or after emotional or physical stress, but also in absence of those triggers, and in younger population and men too. An excessive release of catecholamines seems to be pivotal role in the development of stress cardiomyopathy. Usually patients present with acute heart failure or acute coronary syndrome. Echocardiographically is almost undistinguishable from acute myocardial infarction due to left descending coronary artery occlusion. (1, 2) **Case report:** We herein present the case of 50-years-old female with nonregulated hypertension, dyslipidemia and obesity, presented with renal colic accompanied with nonspecific chest pain and dyspnea within 36 hours before admission. Initially she was presented to urologist due to dilation of channel system of the right kidney; radiographically possible stone in prevesical part of right ureter. Since her electrocardiogram showed subacute myocardial infarction with ST-segment elevation of anteroseptal region **(****Figure 1****)** with troponin T elevation up to 0.53 ug/L, she was admitted to Coronary Unit at the Department of Internal Medicine. Echocardiography showed closely suspicions on Takotsubo cardiomyopathy with preserved left ventricular systolic function (**Figure 2**), no valvular disease or intracavitary gradient. Her laboratory test results showed progression of deterioration of renal function and oliguria (urea 18,3 mmol/l, creatinine 264 mmol/l, eGFR EPI 17.4 mL/min), inflammation markers were high (Leucocytes 19 x 109, CRP 270 ug/L) predicting urosepsis and obstruction of the right kidney needed to be solved promptly. She was amicably transferred to tertiary center where urologists first resolved kidney obstruction since her kidney function was compromised, and after resolving urosepsis and full recovery she underwent coronarography that confirmed Takotsubo cardiomyopathy with no coronary artery obstruction lesions. Patient was managed with conservative therapy: ACE inhibitors, beta-blockers, analgesics and low molecular weight heparin. After six weeks she came for control echocardiography examination showing full recovery of apical ballooning and electrocardiographically complete resolution of earlier signs of anteroseptal infarction (**Figure 3**). FIGURE 1. Subacute myocardial infarction with ST-segment elevation of anteroseptal region. FIGURE 2. Takotsubo cardiomyopathy with preserved left ventricular systolic function. FIGURE 3. Control echocardiography with full recovery of earlier signs of anteroseptal infarction. **Conclusion:** Reversible left ventricular dysfunction (in this case we saw typical wall motion abnormality – apical ballooning with preserved global systolic function) seem to be pathognomonic in Takotsubo syndrome, and timelines of recovery is variable from patient to patient.

Janez Toplišek, Vojka Gorjup, Miha Lučovnik, Tatjana Stopar Pintarič, Iva Blajič, Špela Mušič, Barbara Krunić, Katja Prokšelj, Špela Tadel Kocjančič, Anja Čopi Jerman, Barbara Salobir

**Introduction:** Despite advanced therapies for pulmonary arterial hypertension (PAH), maternal mortality in women with PAH and their offspring remains high (30-56% and 11-28%) and is especially high during the post-partum period (1, 2). **Case report**: 39-years-old woman was presented in 24th week of pregnancy with moderate dyspnea and cyanosis without peripheral edema. ECG showed right ventricular strain and NT-proBNP was 1300 ng/L. Echocardiogram showed severe precapillary pulmonary hypertension with systolic pulmonary pressure (PAP) 103 mmHg, reduced stroke volume (SVI 29 ml/m2), normal cardiac output (CI 2.6 L/min/m2) and mildly reduced systolic function of the right ventricle (FAC 30%, PMI TDI 0.8) with normal central venous pressure (CVP) 3 mmHg. Right heart catheterization confirmed echocardiographic hemodynamic measurements (mean pulmonary pressure 61 mmHg, pulmonary vascular resistance (PVR) 9.4 WU) and the vasoreactivity test was negative. Epoprostenol was initiated, titrated up to 22 ng/kg/min and on 30th week planned caesarean section was performed with NO inhalations, noradrenalin and dobutamine. Fortunately, standby supportive therapy - ECMO and high urgency lung transplantation, was not needed. The baby did well. The patient was extubated on the same day and sildenafil was added. The patient was discharged after 4 weeks. After 2 months epoprostenol was switched to treprostinil up to 26 ng/kg/min and macitentan was added. NT-proBNP stabilized at 170 ng/L. Echocardiography was performed weekly to monitor CI, CVP and right systolic function indexes. Favorable echocardiographic dynamics after therapy were observed: mean PAP 60 mmHg to 45-50 mmHg, PVR from 10 WU to 7 WU, stroke volume normalized and CVP remained normal. However, right ventricular systolic function improved but did not normalize (**Table 1**). ### Table 1: Echocardiography variables before and after therapy. | **sPAP mmHg** | 106 | 64 | | --- | --- | --- | | **mPAP mmHg** | 64 | 42 | | **PVR (WU)** | 10 | 7 | | **RAP mmHg** | **3** | **3** | | **SV ml** | 51 | 69 | | **SVI ml/m2** | 29 | 40 | | **CO L/min** | 4.6 | 4.7 | | **CI L/min/m2** | **2.6** | **2.7** | | **RA cm2** | **26** | 26 | | **RV TAPSE cm** | 1.8 | 2.4 | | **RV FAC %** | 24 | **30 (3D EF 36%)** | | **RV MPI TDI** **RV free strain %** | 0.8 -12 | 0.6 -24 | | **LV Eccentricity index** | 2.4 / 3.9 | 1.5 / 2.1 | | **Pericardial effusion** | minimal | minimal | [†] sPAP = systolic pulmonary artery pressure, mPAP = mean pulmonary artery pressure, PVR = pulmonary vascular resistance, RAP = right atrial pressure, SV = stroke volume, SVI = stroke volume index, CO = cardiac output, CI = cardiac index, RA = right atrial area, RV = right ventricle, TAPSE = tricuspid annular plane systolic excursion, FAC = fractional area change, MPI TDI = myocardial performance index, 3D EF = three-dimensional ejection fraction, RV free strain = right ventricular free wall longitudinal strain. In **conclusion,** with complete echocardiographic hemodynamic assessment and thorough clinical assessment high-risk pregnancies in patients with severe PAH can be managed. In addition, FAC, MPI TDI, 3D ejection fraction and RV free strain better assess right ventricular systolic function than TAPSE.

Mario Udovičić, Sandra Jakšić Jurinjak, Mira Stipčević, Boris Starčević, Igor Rudež, Vanja Ivanović Mihajlović, Hrvoje Falak, Danijela Grizelj

**Introduction**: Post heart transplantation (HTx) infective endocarditis (IE) is unique among endocarditis after solid organ transplantation because it is the transplanted organ that becomes infected. Since 2016 two HTx patients have been successfully treated in University Hospital Dubrava for cardiac allograft infective endocarditis. **Case report:** The first one, a 77-year-old male patient, who in 2001 underwent orthotopic HTx due to ischemic cardiomyopathy, and who since 2010 was on hemodialysis due to chronic terminal kidney failure, was admitted in June 2016 due to mitral valve endocarditis (**Figure 1**). The other one was a 29-year-old male patient who in January 2016 had undergone orthotopic heart transplantation due to dilated cardiomyopathy and was diagnosed in June 2017 with tricuspid valve endocarditis (**Figure 2**). Both patients had atypical presentation, and in both patients transthoracic and transesophageal echocardiography were immediately performed revealing valve vegetations and proving crucial for establishing diagnosis. Empirical antibiotic treatment was initiated at once, and the patients were referred to the endocarditis team. FIGURE 1. Transesophageal echocardiography showing a vegetation on the anterior mitral leaflet. FIGURE 2. Transthoracic ultrasonography showing a large vegetation on the septal leaflet of tricuspid valve. **Conclusion**: IE is a known, but rare and very often fatal complication of HTx. Frequent central venous catheter access and multiple endomyocardial biopsies appear to predispose to infection (1). The prognosis of post-HTx IE remains poor. Atypical presentation is common in these patients (2), and therefore a high index of suspicion and low threshold for investigation are appropriate in this group in order to avoid delays in diagnosis (3). There are no specific guidelines to diagnostic practice for this particular group, however, echocardiography is the mainstay and essential in diagnosis of endocarditis (4), and therefore has to be performed at the earliest possible time.

Iva Uravić Bursać, Vesna Pehar Pejčinović, Viktor Peršić, Ivana Butković

**Introduction**: Double outlet right ventricle (DORV) is congenital cardiac malformation which occurs 1-3% of individuals with congenital heart defects (1). The echocardiography is a method of choice to detect the regional acceleration of flow and in most cases is sufficient for diagnosis and surgical planning (1). When the findings at the imaging examination are inconclusive, cardiovascular MR imaging may play an important role in anatomy of the ventricular septal defect, functional status of both ventricles, and to identify any residual stenosis or regurgitation or coexistent anomalies (2). In DORV both great arteries come from the same pumping chamber (2). These patients require long-term follow up and may present for surgical or catheter-based interventions (3). Possible complications may include heart failure, high blood pressure in the lungs or death (3). **Case report**: We report 43-year-old men with Blalock-Taussig shunt. He visited hospital for cardiac evaluation after he had respiratory infection with shortness of breath and palpitation. Electrocardiography showed right axis deviation and right ventricular hypertrophy. Laboratory examination revealed polycythemia and hypoxemia. This patient has associated ventricular and atrial septal defect and hypoplastic pulmonary artery. The heart showed dilatation of the bilateral ventricles, right ventricular hypertrophy with reduced systolic function (39%). Above the right ventricle is left atrium with pulmonic veins. **Conclusion**: MR imaging provides accurate additional anatomic information in patients with DORV, which is helpful in presurgical planning as well as during follow up.

Kristina Selthofer-Relatić

Obesity has become a global public health issue. Cardiac adaptation to obesity includes structural and functional changes of the heart. Anatomic and biomolecular characteristics of adipose tissue present it as an endocrine organ with neurohumoral adipokines activity. However, fat tissue differs according to embryologic origin, anatomic localization, gender and biomolecular properties. Qualitative and quantitative echocardiographic evaluation in obese patients can be crucial for detecting cardiometabolic risk factors. Increased adipose mass causes functional and morphological changes of the heart. Increased body fat and body surface area are associated with increased blood volume, ventricle preload, resting output and stroke volume; supranormal systolic function and possible aortic root dilatation; abnormal left and right ventricle geometry, as a result of compensatory wall hypertrophy regarding pressure or volume overload; enlarged left and right ventricular end-diastolic diameter and elevated end-diastolic volume; and increased left atrium area and volume. Using indexed parameters in echo analysis in obese patients are recommended. Early detection of obesity related cardiovascular abnormalities may be useful in the future for patient management. Particularly, strain echo acts as a technique for the early detection of subclinical changes of the left ventricle in obese patients, while epicardial adipose tissue is the newest echo detector of cardiac visceral fat as a cardiometabolic and right ventricle remodeling risk factor. Routine clinical echo practice in obese patients can be limited by technical factors, especially right heart morphologic and functional measurements. However, contrast and transesophageal echocardiography offers additional methods for cardiac structure visualization. (1-4)

Damir Raljević, Karlo Stanić, Vesna Pehar Pejčinović, Viktor Peršić

The persistent left superior vena cava is a rare vein anomaly and is encountered in 0.3-0.5% of the population, however, as these anomalies are usually asymptomatic, the prevalence is often underestimated (1). This anomaly is found in 12% of cases with other congenital heart disease. Several anatomical variants of this anomaly are described. In the largest number of cases (80-90%) there are developed both left and right superior vena cava that may differ in size (2). In the event that both vena cava equally developed, in 65% of cases the development of the left innominate vein is absent and there is no communication between the two superior vena cava. Then the persistent left vena cava drains into the right atrium through the coronary sinus (3). In 10-20% cases, the left superior vena cava drains into the left atrium. Also, in 10-20% cases, the right superior vena cava is not developed, and the single left superior vena cava is drained through the coronary sinus into the right atrium (3). In the vast majority of cases this anomaly is asymptomatic and goes unnoticed. Clinical relevance is found in the case of the central venous access and the placement of central venous devices, in which case it may be difficult or impossible to set up a central venous device or provoke potentially dangerous right-left shunt and potential thromboembolic incidents. During this presentation we intend to report a case of multimodal imaging of persistent left superior vena cava.

Iva Jurić, Dražen Mlinarević, Marko Stupin, Sandra Makarović

Cardiac masses are an important and often impressive finding during echocardiography. Masses can be present in any cardiac cavity and they can cause functional obstruction to blood flow with serious consequences. The most common masses are thrombi, tumors and vegetations. While the definitive diagnosis is usually obtained from pathology report, with echocardiography we can establish the localization, size, mobility, echogenicity and the likely diagnosis. Vegetations are infectious masses, irregularly shaped, with a chaotic movement pattern and most commonly located on the upstream side of valve leaflets. Cardiac tumors can be primary or nonprimary, the latter ones being more common. Metastases to the heart are most frequently associated with melanoma, lung, breast or hematological neoplasms. Primary cardiac tumors are mostly benign but can cause significant obstruction to blood flow. Myxomas are the most common benign tumors, followed by papillary fibroelastomas. Primary malignant cardiac tumors are rare, and they include angiosarcomas, rhabdomyosarcomas, fibrosarcomas and mesotheliomas. Thrombi are masses associated with areas of limited blood flow – most commonly left ventricular aneurysms. Initial transthoracic echocardiography (TTE) in the evaluation of cardiac masses can be supplemented by 3D imaging, transesophageal echocardiography (TEE) and especially by contrast echocardiography. A comprehensive TTE/TEE examination paired with clinical information can reasonably suggest what type of mass is present. (1-4) In our presentation we will discuss three patients with the three most common cardiac masses (tumor, thrombus and vegetation) and with different clinical outcomes. We will focus on the echocardiographic examination and the advantages of contrast echocardiography.

Sandra Makarović

The patent foramen ovale (PFO) is a common anatomical variant in humans (prevalence 25%). Most common asymptomatic (incidental), it can be a cause of a paradoxical embolic stroke. A dedicated PFO occluder (Amplatzer PFO Occluder) was first used in 1997. Since, it has been used often as common and safe procedure. Three randomized studies (Randomized Evaluation of Recurrent Stroke Comparing PFO Closure to Established Current Standard of Care Treatment-RESPECT, Closure or medical therapy for cryptogenic stroke with patent foramen ovale-CLOSURE, Patent Foramen Ovale Closure or Antiplatelet Therapy for Cryptogenic Stroke-REDUCE), have proved its benefit for prevention of recurrent ischemic events (most commonly stroke), in patients without conventional stroke pathology. (1-3) We present a series of cases of young people, with cryptogenic stoke, PFO demonstrated on transesophageal echocardiography, using “bubbles” study, and successful transesophageal guided closure of PFO. Prior to PFO closure, a structured and systematic approach allows for a rapid and comprehensive echocardiographic assessment of the atrial septum. Good echocardiographic assessment of the atrial septal anatomy before closure is of paramount importance to allow the procedure to remain safe and effective.

Ivana Lukić, Iva Jurić, Vjekoslav Amidžić, Kristina Selthofer-Relatić

Left ventricular hypertrophy is an adopted response to physiological and pathological stress, while hypertrophic cardiomyopathy (HCM) is defined by the presence of increased left ventricular wall thickness that is not solely explained by abnormal loading conditions. Determination of the etiology, pathophysiology and disease severity is important for the management of patients with HCM. (1-4) According to latest European Cardiology Society Guidelines for HCM (2), etiology in adults are 60% an autosomal dominant trait caused by mutations in cardiac sarcomere protein genes, 5-10% caused by other genetic disorders including inherited metabolic and neuromuscular diseases, chromosome abnormalities and genetic syndromes, malformation syndromes, non-genetic disorders that mimic genetic forms of the disease like amyloidosis and infiltrative diseases. In about 25-30% of HCM etiology is still unknown. In adults, HCM is defined by a wall thickness ≥15 mm in one or more LV myocardial segments as measured by any imaging technique. Transthoracic 2D and tissue doppler echocardiography presents tool for morphologic and hemodynamic evaluation (with additional strain analysis, contrast and transesophageal echocardiography), and cardiac MRI for assessment of cardiac morphology and myocardial tissue characteristics. Routine laboratory tests and specific testing aids the detection of HCM etiology. The majority of HCM cases are inherited autosomal dominant genetic trait with a 50% risk of transmission to offspring. Some cases are explained by de novo mutations, sporadic cases can arise because of incomplete penetrance in a parent and by autosomal recessive inheritance. Regular genetic analysis should include the most commonly implicated sarcomere protein genes, and pedigree analysis should be provided. Annual incidence of cardiovascular death is 1–2% caused by sudden cardiac death, heart failure or thromboembolism. HCM etiology detection is necessary because of treatment possibilities (like Anderson-Fabry disease or ATTR amyloidosis), detection of secondary causes of disease, the need for family screening and differentiating pathogenic from non-pathogenic mutations. Routine clinical practice is challenging, left ventricular hypertrophy and HCM can be presented in different forms and different stages.

Lujza Grueva, Elena G. Nastevska

**Introduction:** Hypothyroidism is one of the most common diseases in endocrinology, which can be diagnosed easily with the current diagnostic procedures and a typical clinical expression. One of the most frequent cardiological complication of hypothyroidism is the pericardial effusion, with incidence of 30-75%, while the heart tamponade is a very rare complication that requires urgent treatment. (1) **Case report:** 60-year-old woman was admitted to the clinic of Cardiology in Skopje, dyspneic with a general malaise. During the physical exam the patient was with slower speech, dry skin and hair, brittle nails, bilateral periorbital edema. Vital signs: heart rate 85 beats per minute, blood pressure 180/100mmHg. 12-lead ECG findings showed low voltage and flattened T wave in all leads (**Figure 1**). During the echocardiography exam (**Figure 2**) was confirmed a large pericardial effusion with a compression on the right-sided cavities and a “swinging heart”, and pericardiocentesis was indicated. More than 1100ml of fluid was evacuated and sent for further investigations. Laboratory findings showed advanced and untreated hypothyroidism (TSH >75.0 ulU/ml, free T4 <3.86 pmol/L; total T4 <12.9 nmol/L). FIGURE 1. 12-lead ECG with low voltage and flattened T wave in all leads. FIGURE 2. Large pericardial effusion. **Conclusion:** The amount of pericardial effusion (PE) in patients with hypothyroidism is correlated with the progress of the disease, and is very common in myxedema, but very rare in mild forms of hypothyroidism. In Macedonia, especially in the rural environments, patients with hypothyroidism are mostly undiagnosed on time, so the complications are more frequent. Effective and on time diagnose of PE is crucial, because of the high risk of cardiac tamponade. Positive outcome and hemodynamic stability in most of the patients with hypothyroidism is achieved after the application of appropriate thyroxin treatment.

Lea Skorup, Ivana Grgić Romić, Jelena Šimić, Božidar Vujičić, Sanjin Rački, Alen Ružić, Luka Zaputović, Ante Matana, Teodora Zaninović Jurjević

**Introduction**: Fabry disease is rare X-linked, recessive lysosomal storage disorder expressed as deficiency in enzyme α-galactosidase A that leads to progressive accumulation of globotriaosylceramide and related glycosphingolipids in various tissues. In cardiac tissues, progressive globotriaosylceramide accumulation leads to irreversible cardiac damage. (1, 2) Males are primarily affected by Fabry disease, but female heterozygotes may also have symptoms. (3) **Case report**: Our patient is 47-years-old who has been suffering from chronic kidney disease since he was 23 years old. Only twelve years later he developed end-stage renal disease and has been undergoing regular haemodialysis in Dialysis Centre Prijedor (Bosnia and Herzegovina). He started his journey towards kidney transplantation in 2014 in University Hospital Centre Rijeka, which was performed in May 2018. In 2014, during pretransplantation workup, echocardiography was performed revealing concentric cardiac hypertrophy without left ventricle outflow tract obstruction (**Figure 1** and **Figure 2**) associated with contractility and diastolic filling impairment. Following suspicion on Fabry disease, diagnosis was made by measuring α-galactosidase enzyme activity in leukocytes and molecular genetic testing of GLA gene mutation. Enzyme replacement therapy was started with intravenous infusion of recombinant α-galactosidase A (agalsidase beta). FIGURE 1. Echocardiography image, apical four-chamber view showing thickened intraventricular septum and lateral wall of left ventricle. FIGURE 2. Echocardiography image, parasternal long axis view showing thickened intraventricular septum and inferolateral wall of left ventricle with prominent papillary muscle. **Conclusion**: Renal disease and echocardiographic features of hypertrophic cardiomyopathy combined with electrocardiographic and clinical criteria should be considered as “red flags” for Fabry disease.

Igor Klarić, Damir Raljević, Vesna Pehar Pejčinović, Viktor Peršić, Karlo Stanić, Ivo Kalčić

Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC) is a hereditary cardiomyopathy that is histologically characterized by progressive replacement of the right ventricular myocardial tissue by fibrofatty tissue and usually manifests from the second to fourth decade of life with ventricular arrhythmias origin from the right ventricle, sudden cardiac death and / or abnormal contractility of the right ventricle. Today, it is known that this replacement of normal myocardial of RV with fibrofatty tissues the result of the mutation of five genes encoding heart desmosome proteins responsible for connecting cardiomyocytes. (1-4) Typical morphological features of ARVC are regional contractility disorders, aneurysm, or dyssynchrony of the right ventricle contractions. These abnormalities are typically observed in predilection areas involving the subtricuspidal area, the free wall of basal segment RV and the posterolateral wall of LV. The 2010 Revised Task Force criteria for ARVD clearly highlighted the importance of cardiovascular imaging in diagnosing this clinical entity, primarily keeping in mind 2D echocardiography and cardiac magnetic resonance. They have become widely available, especially 2D echocardiography, are non-invasive and do not expose patients to ionizing radiation. Although not included in the diagnostic criteria, studies confirm that echocardiographic imaging methods using a TDI or speckle tracking are tool which allow us to notice early changes in RV function. Cardiac magnetic resonance has the ability of 3D visualization and high spatial resolution, and also provides both morphological and functional characterization as well as estimation of fibrofatty replacement, and is the gold standard for final diagnosis, while 2D echocardiography serves in clinical follow-up and has shown significant variability in the rate of progression disease.

Lorena Kalčina Uravić, Morana Kasunić Jelić, Andrej Došen, Andrea Grman Fanfani, Ronald Lipovšćak, Kamal Al Rajabi, Davor Horvat

**Aim:** The aim is to present a case of rare malignant tumor, leiomyosarcoma of the inferior vena cava (IVC) with the involvement of the liver and intravascular extension into the right atrium and ventricle. **Case report:** 50-year-old male patient presented to the Emergency Department with upper abdominal pain and meteorism during the past month, without any medical history of chronic illness. After physical exam and blood test, abdominal ultrasonography was preformed which verified an expansive liver formation. Transthoracic echocardiography showed a large echogenic mobile mass extending from the IVC into the right atrium and ventricle, a differential diagnosis tumor or thrombi. Abdominal computer tomography showed a large hyperdense avascular mass with central necrosis in the left liver lobe, thrombosis of portal vein and filling defect of the inferior vena cava from the hepatic veins to the right atrium and ventricle. After taking biopsy, histochemical and immunohistochemical analysis confirmed a diagnosis of leiomyosarcoma. Considering the findings of computer tomography and echocardiographic characteristics of described mass into the right atrium and ventricle we concluded that it is probably a leiomyosarcoma of IVC with intravascular extension. Taking into account the pathohistological diagnosis, the stage of disease at the time of diagnosis, the laboratory and ultrasonography signs of liver disfunction the Expert Consilium concluded that operative or any active treatment is not indicated. **Conclusion:** Leiomyosarcoma of the inferior vena cava is malignant mesenchymal tumor, with low incidence and prevalence. Occurrence of this tumor in the III segment of the inferior vena cava by Kulazlat classification is especially rare. It is important to note that it is a possible and uncommon differential diagnosis of a right atrium tumor (1-3).

Sandra Jakšić Jurinjak, Mario Udovičić, Boris Starčević, Petra Vitlov, Jelena Kursar, Ognjen Čančarević, Miroslav Raguž, Hrvoje Falak, Igor Rudež

**Introduction:** Transthoracic echocardiography is still the diagnostic standard procedure in pre heart transplant and advanced heart failure diagnostics. Despite continued improvements in non-contrast echocardiography, image quality is sometimes suboptimal for assessing regional and segmental wall. (1, 2) **Case report:** 22-year-old patient suffering from dilative cardiomyopathy and diabetes mellitus type I presented with signs of severe dyspnoea and was admitted to intensive coronary unit due to acute heart failure. Dilative cardiomyopathy was verified with severely reduced ejection fraction to 15% of the left ventricle, with apical left ventricular thrombus, clinically NYHA IV stage, initially INTERMACS 3. The hypertrabeculation of left ventricular wall was indicating that the aetiology could be non-compaction cardiomyopathy. He was referred to our transplant centre, where complete pre-transplant work-up was conducted. Despite optimal medical therapy, clinical condition deteriorated, NT-pro-BNP measured up to 13122.9-pg/mL and he was on inotropic support, heart failure medication and anticoagulation therapy due to left ventricular apical thrombus. Right heart catheterization showed a moderate increase of pressures in pulmonary circulation due to congestion and his cardiac index calculated from right heart catheter was 1.48 L/min/m2. Coronary artery disease was excluded by coronary angiogram. Due to deterioration, the patient needed ECMO support, and finally the multidisciplinary heart transplant team decided to implant extracorporeal left ventricular assist device (LVAD). As it is paramount to be aver of left ventricular thrombus in case of LVAD implantation, the contrast echocardiography for opacification of the left ventricle (Optison GE Healthcare) was conducted excluding the thrombus in the left ventricle and showing the extent of left ventricular trabeculation (**Figure 1**). Finally, patient was transplanted after three weeks of extracardiac LVAD support and was discharged from hospital in good condition. As addition, it is interesting that a patient has twin brother without diabetes mellitus, and his echocardiographic report is suggestive of dilative cardiomyopathy –noncompaction but with far better left ventricular contractility and ejection fraction of LV of 35%. FIGURE 1. A, C. Left ventricular opacification showing trabeculated and dilated left ventricle indicating non-compaction cardiomyopathy, no presence of intracardiac thrombus. B. Dilated left ventricle in 4 chamber apical view. D. Bulls eye of left ventricular longitudinal strain showing diffuse poor longitudinal strain**.** **Conclusion:** Use of contrast echocardiography to guide urgent treatment decisions in critically ill patients and making treatment decisions as in device therapy provides a valuable option for improving endocardial border resolution and outcome in these patients.

Ivana Grgić Romić, Davorka Lulić, Zrinka Matana, Sandro Brusich, Ante Matana, Luka Zaputović, Alen Ružić, Teodora Zaninović Jurjević

**Introduction:** The differential diagnosis of an intracardiac mass include benign and malignant primary heart tumors, metastatic tumors and thrombi. Primary tumors of the heart consist mainly of myxomas, with an incidence of less than 0.5%. Clinical manifestations are consequence of embolic phenomena, intracardiac obstruction or constitutional symptoms. In rare instances, myxomas can cause a mass effect, resulting in mitral valve obstruction (1). **Case report:** 68-year-old male with a history of ulcerative colitis complained to his gastroenterologist about having exercise intolerance. Undergoing regular colitis evaluation, CT thorax and abdomen scan was performed incidentally revealing a large intracardiac mass. Echocardiogram ordered by the consulting cardiologist demonstrated a large ellipsoid left atrial cyst (50×31 mm), occupying nearly the entire left atrium (**Figure 1**). The mass was protruding across the mitral valve orifice in diastole causing functional stenosis with an elevated mean diastolic gradient of 10,9 mmHg. Mitral valve area calculated by pressure half-time was 1.0 cm2. Doppler showed moderate tricuspid regurgitation with a systolic pulmonary artery pressure of 50 mmHg. The systolic function was preserved with an estimated left ventricular ejection fraction of 58%. Transesophageal echocardiography described a cavitating lesion (measuring 15,1 cm (2*,*) attached to interatrial septum with 24 mm base), having characteristics consistent with a hemorrhagic cyst (**Figure 2**). Preoperative coronary angiography displayed coronary artery disease and a rare condition of dual coronary artery supply with left circumflex artery (LCx) providing two (**Figure 3**) and right coronary artery (RCA) one tumor branch (**Figure 4**) producing a characteric "tumor blush". CT showed large intracardiac mass (**Figure 5** and **Figure 6**). Patient underwent cardiothoracic surgery with successful excision of the tumor (4,5x3x2 cm), the pathohistology confirmed myxoma. Postoperative course was uneventful, exercise intolerance symptoms improved, and echocardiographic follow up showed no intracardiac mass. FIGURE 1. Transthoracal echocardiogram apical four chamber view showing a large cyst-like myxoma arising from interatrial septum. FIGURE 2. Transesophageal echocardiogram four chamber view – color Doppler showing the myxoma in the left atrium causing near complete mitral valve obstruction. FIGURE 3. Coronary angiography showing the left circumflex artery supplying the intracardiac tumor with two anomalous branches. FIGURE 4. Coronary angiography showing the right coronary artery supplying the tumor producing characteristic “tumor blush”. FIGURE 5. Contrast enhanced axial CT scan showing minimal enhancenemt of the mass. FIGURE 6. CT cardiac image showing large intracardiac mass. **Conclusion:** We described a rare case of cystic-appearance cardiac myxoma with dual coronary supply mimicking mitral valve stenosis. There are not many patients reported with left atrial myxoma being vascularized from both RCA and LCx as seen in our case (2). Although more than half of atrial myxomas show obstructive symptoms, severe mitral valve obstruction is rare (1). Early echocardiographic examination of patients presenting with exertional dyspnea is advised, as myxomas have an excellent prognosis following surgical excision, preventing complications and improving quality of life.

Karlo Golubić, Ivana Tomašić, Ivo Darko Gabrić

The ejection fraction (EF) is the most commonly used echocardiographic measure of the systolic left ventricular (LV) function. Echocardiography has however several limitations in measuring LV volumes and EF since the formulas for quantifications are based on geometrical assumptions, the EF parameters (end-systolic and end-diastolic volumes) are preload dependent and adequate projections are not always possible to obtain. Previous studies have also shown correlation between different echocardiographic techniques of obtaining EF and even correlation between echocardiography and other methods (MRI, SPECT, CT) but the data on interobserver and intraobserver variability remains scarce. (1) In this study we examined the interobserver limits of agreement and the therapeutic implications of the uncertainty obtained by the result. We examined 22 sequential patients referred to our echocardiography laboratory for LV function assessment. All echocardiography studies were performed by two independent cardiologists blinded to the measurements of the other observer. 2D methods including the biplane Simpson method and “eyeballing” were conducted, while automatic and semiautomatic 2D and 3D methods were not allowed. Bland-Altman analysis was performed to determine the systematic bias and limits of agreement (LOA) of EF between different observers (**Figure 1**). Next the LOA were used to obtain new “confidence intervals” on a previously gathered set of echocardiograms of 171 heart failure patients. We recorded the number of patients that now had different treatment recommendations according to the current ESC guidelines on heart failure and device therapy i.e. the “new” EF was 40%. McNemar’s test was used to determine the statistical significance of the difference with a two-tailed P=0.05 taken as a threshold. The bias was calculated to be -2.3 with LOA (7.8 to -12.5) which is in agreement with similar studies. The number of patients that received “new” treatment recommendations was 99 for the lower LOA and 65 for upper LOA, P<0.001 for both groups. FIGURE 1. The Bland-Altman plot showing the limits of agreement. The limits of agreement are marked as dashed lines in the plot, specified as the average difference (bias) ± 1.96 SD of the difference. We therefore conclude that the interobserver variability of the EF measurement can lead to significantly different treatment strategies in patients with heart failure. Echocardiographic methods introducing less variability (like automatic 3D techniques) should be preferred in determining the EF.

Blanka Glavaš Konja, Vlatka Lukšić Rešković, Irena Ivanac Vranešić, Majda Vrkić Kirhmajer, Zvonimir Ostojić, Marija Mance, Jelena Hucika, Jurica Šalković, Joško Bulum, Martina Lovrić Benčić, Jadranka Šeparović Hanževački

**Case report**: 26-year-old patient with a systemic lupus erythematosus diagnosed a year ago was hospitalized because one day temperature without a concomitant increase in inflammatory laboratory parameters. At admission, the transthoracic echocardiography (TTE) and transesophageal echocardiography (TEE) showed pseudoaneurysm of the anterior mitral cusps (**Figure 1**, and **Figure 2**). Blood cultures at admission, as well as those sampled later, were all negative. One year earlier, the patient was hospitalized for febrile pancytopenia and Staphylococcus aureus septicemia. Diagnostics confirmed normal hematopoiesis but revealed systemic lupus erythematosus. Echocardiography performed early during the first hospitalization was normal (**Figure 3**). Antibiotic therapy was initiated. Blood culture test became negative without expected clinical recovery, so corticosteroid therapy was added. After three weeks of treatment, the patient was released home cured. Corticosteroid therapy was terminated after a gradual dose reduction. The patient felt well until the second hospitalization. Endocarditis was not confirmed during the second hospitalization, suggesting the sterile mitral valve pseudoaneurysm. Because of the potential risk for further deformation and mitral valve rupture, surgical valve reparation was suggested and done without complications. FIGURE 1. Transthoracic echocardiography: mitral valve pseudoaneurysm (long axis view). FIGURE 2. Three-dimensional transesophageal echocardiography: mitral valve pseudoaneurysm. FIGURE 3. Transthoracic echocardiography early during *S. aureus* septicemia. **Discussion**: Infectious endocarditis is a challenging disease. Echocardiography is the basic imaging method, especially in the circumstances of the high clinical suspicious based on Duke’s criteria (1). In the case of native valves, the sensitivity of TEE is 90 to 100%, and specificity 90% in the detection of vegetation, perforation or fistula (2). In the detection of paravalvular abscesses, the sensitivity of TEE is 80-90% and of TTE is only 36-50%, or even less for small abscesses (3). In the case of a negative echocardiographic finding and high clinical suspicion of endocarditis, TTE / TEE should be repeated 5-7 days later, in the case of S. aureus infection even earlier (4). A repeated negative study should virtually rule out the diagnosis. This case highlights the importance of two echocardiographic examinations at least seven days apart if there is a doubt about endocarditis, especially in the presence of S. aureus infection as it was the case.

Blanka Glavaš Konja, Vlatka Rešković Lukšić, Joško Bulum, Martina Lovrić Benčić, Marija Mance, Zvonimir Ostojić, Lada Bradić, Davor Radić, Tea Šimonček, Jadranka Šeparović Hanževački

**Case report**: 45-year-old woman was hospitalized for severe chest pain. Her medical problem began four months earlier. ECG and echocardiographic findings were normal. Stress test was stopped because of chest pain at 8 METs. At the time of two-minute chest pain, the ECG recorded intermittent left branch block (**Figure 1**). The 24h Holter ECG showed the frequency dependent left bundle branch block (LBBB) but no chest pain episode. MSCT coronary artery detected normal coronary arteries. Cardiac MR was suggestive to myocarditis. Troponin and inflammatory parameters during the four months of follow-up remained normal. Considering the persistence of symptoms, coronary angiography was performed showing normal epicardial arteries. During angiography, the patient experienced chest pain at the time of right coronary artery (RCA) probing, and coronary artery spasm of the proximal RCA was obvious at the time (**Figure 2**). After the nitroglycerin bolus application, spasm disappears (**Figure 3**) and chest pain released. During the coronary angiography there was no ST-segment elevation, nor intermittent LBBB. Vasospastic angina has been diagnosed. The patient was further treated with calcium antagonists and long-acting nitrates resulting in clinical improvement. FIGURE 1. Stress test: Intermittent left bundle branch block during chest pain episode. FIGURE 2. Coronary angiography: right coronary artery vasospasm during chest pain episode. FIGURE 3. Coronary angiography after nitrate application. **Discussion**: Early diagnosis of variant angina is crucial to avoid major cardiac events (1). In the case of large coronary artery spasm, the ECG can show impermanent ST elevation. LBBB was observed temporarily in our patient during stress test concomitant with chest pain but not in the 24h Holter ECG recording. Coronary vasospasm and myocarditis are both recognized mimics of ST-segment elevation myocardial infarction with normal coronary arteries. The occurrence of both pathologies in the same patient has rarely been described (2). Our patient had no troponin increase recorded, and no inflammatory parameters. It is important to note that the diagnostic sensitivity and specificity of the CMR for pericarditis is under 100% (3). The case confirms the complexity of vasospastic angina diagnosis, and the importance of understanding the diagnostic imaging limitations.

Siniša Roginić, Jelena Zajec Gorički, Marija Čajko, Aleksandar Trbović

**Introduction**: Endocarditis is a devastating infection often leading to significant valvular disfunction. Prolonged antibiotic therapy remains a mainstay of treatment but one third of patients require surgery in course of disease. Classic indications are severe valvular disfunction causing heart failure, septic embolization, difficult to control and local propagation of infection. (1, 2) **Case report**: Our patient has had a mitral valve endocarditis 20 years ago leaving behind presumably moderate mitral regurgitation. Last 3 months she had relapsing fever, anorexia and polyarthralgia which were attributed to her osteoporosis. Transthoracic echocardiography showed deformation and thickening of posterior mitral cusp causing severe mitral regurgitation (MR) described in previous exams, but transesophageal study (TEE) also showed a fresh vegetation on anterior mitral cusp. Streptococcus viridans was repeatedly isolated from blood cultures supporting diagnosis of odontogenic subacute endocarditis. There were no signs of heart failure or peripheral embolization. Patient a priori refused any form of surgical treatment. Vancomycin was administered for 4 weeks (due to penicillin allergy) leading to complete clinical recovery, sterilization of blood cultures and disappearance of anterior mitral cusp vegetation on TEE. After regaining full mobility patient still had no symptoms correlated with a MR. **Discussion and Conclusion**: Severe primary MR after endocarditis in the era of valve reparation seems like straightforward surgical indication. We were surprised to find out that the patient’s reluctance to surgery was justified by her complete recovery from endocarditis and guidelines. Although MR remained severe, left ventricle is mildly dilated (EDD 56mm/ESD 27 mm), hyperdynamic (EF 66%), pulmonary hypertension is mild (sPAP 45 mmHg), and the patient is still in sinus rhythm. Question remains is she truly asymptomatic because recent polyarthritis limited her mobility. Patient was discharged and scheduled for follow up in 3 months.

Mijo Meter, Zora Sušilović Grabovac, Antonia Melada, Josip Katić, Ivona Mustapić, Viktoria Lišnić, Darija Baković Kramarić

**Introduction:** Sinus of Valsalva aneurysm (SVA) is an abnormal dilatation of the aortic root located between the aortic valve annulus and the sinotubular junction. The estimated rate of SVA is approximately 0.09% of the general population and involve up to 3.5% of all congenital heart defects (1). Aneurysm predominantly originates from the right coronary sinus and may rupture up to 35% of the time commonly to the right cardiac chambers (2). We present an unusual case of a patient with SVA originating from the non-coronary sinus. **Case report:** 63-old-year male with no history of prior cardiovascular disease was presented to department with shortness of breath and chest pain. Physical examination showed blood pressure of 140/80mmHg, respiratory rate of 18/min and heart rate of 84 beats/min. Electrocardiogram revealed complete right bundle-branch block. A routine transthoracic echocardiography showed the enlargement of the left ventricle with large aneurysm originating from non-coronary sinus measuring 3.6x4cm. Echocardiography also revealed a trileaflet aortic valve with moderate aortic regurgitation and normal systolic function of the left ventricle. TEE demonstrated a non-coronary SAV protruding into the left atrium cavity with no signs of rupture. Selective angiography showed normal epicardial coronary arteries, and SAV with dilatation of ascending aorta measuring up to 40 mm. Moderate aortic insufficiency was also detected. The patient was referred to the cardiothoracic surgery ward for further operative treatment. **Conclusion:** Although rare, SAV can be a cause of sudden death. Therefore, a combination of transthoracic echocardiography with other imaging techniques, such as TEE, 3D echocardiography, CT angiography and aortic angiography is recommended to obtain comprehensive information and to improve diagnostic accuracy (3).

Andreja Čleković-Kovačić, Renata Ivanac Janković, Ivana Petrović Juren, Vlasta Soukup Podravec

**Introduction:** Coagulase-negative staphylococci (CoNS) are gram-positive cocci which are common inhabitants of the skin. Infections are usually associated with intravascular catheters, artificial heart valves, vascular grafts and prosthetic joints. (1, 2) We herein present the case of 44-year-old female who presented with an aggressive form of infective endocarditis involving the mitral valve following a central venous catheter due to infection with CoNS. **Case report:** 44-year-old female patient presented with end stage renal disease of unknown origin has been hospitalized for the first day of hemodialysis. At the arrival she was febrile (38 degrees Celsius), with chills and extremely messy place of the temporary non-tunneled jugular catheter insertion. Laboratory findings showed highly elevated inflammatory parameters and chest X-ray was normal. Blood cultures were taken, and because of basic suspicion of catheter-related infections, it was extracted and directed to a microbiological analysis which pointed coagulase negative Staphylococci infection vancomycin-sensitive. Meanwhile, transthoracic and transesophageal echocardiography was performed and vegetation on mitral valve, measured 8x12mm was detected. Despite antimicrobial therapy, patient was febrile, and infection was uncontrolled, and vegetation was of impressive size. CT scan of abdomen and brain were negative to abscess or embolization, so tertiary center was contacted and further observation at the clinic was indicated, but patient refused it. Fortunately, in the control interval the vegetation began to decrease (up to 8x9 mm), the patient became afebrile and insisted on leaving the hospital. Therapy continued during regular hemodialysis up to a complete of 6 weeks. After treatment, control transesophageal echocardiography shows residual changes of mitral valve vegetation accompanied smaller mitral regurgitation, and hemodialysis flow properly. **Conclusion**: A vegetation size larger than 10 mm accompanied with uncontrolled infection in the absence of developed complications is cutoff for consideration of operative treatment. We rarely count on patient noncompliance, but these patients are special challenge for treatment both in reference centers and in county hospitals.

Marija Mance, Daniel Lovrić, Ivica Šafradin, Bojan Biočina, Rajko Ostojić, Jadranka Šeparović Hanževački

**Introduction**: Outside of the immunocompromised patient group, infective endocarditis (IE) of all four valves is a rare finding. It is a potentially deadly disease causing multiple complications, as well as a plethora of associated symptoms and clinical signs which can confound early diagnosis and lead to unnecessary testing. (1-4) **Case report**: We present 71-year-old male patient who was admitted to the hospital for diagnostic work-up of microcytic anemia. Medical history revealed permanent atrial fibrillation and arterial hypertension. In the previous two months he was feeling occasionally feverish, with night sweats and orthopnea. His clinical status showed irregular heartbeats with apical systolic heart murmur, bilateral basal crackles in the lungs, hepatosplenomegaly, scrotal edema and swollen legs. He underwent endoscopy without visible signs of hemorrhage and one tubulovillous adenoma with low grade dysplasia was removed. As plasma M protein was present, further hematologic tests were performed including bone marrow biopsy which was normal. Bone scintigraphy and PET CT showed pathologic accumulation in multiple ribs and vertebrae due to compressive fractures, lytic lesions and spondylodiscitis, in bone marrow and spleen without signs of malignancy as well as enlarged mediastinal, tracheal, axillar and inguinal lymph nodes. Patient was treated with diuretics, digoxin, betablockers, proton pump inhibitors, reimbursement of albumin and blood transfusions. During the third day of hospital stay patient became febrile. Transthoracic and transesophageal echocardiographic examination revealed signs of volume overload, normal ejection fraction, vegetations on all four valves with severe mitral regurgitation (MR) due to ruptured chordae and flail of P2 and P3 segments (**Figure 1**, **Figure 2** and **Figure 3**), severe tricuspid regurgitation (TR) and pulmonary hypertension (**Figure 4**, and **Figure 5**). As Streptococcus gallolyticus was isolated in hemocultures, antibiotic therapy was modified according to antibiogram and diagnosis. Coronary artery stenosis was ruled out by MSCT coronary angiography. Patient was admitted to cardiac surgery and a successful mitral valve replacement with a bioprosthetic valve and surgical debulking of vegetations at other valves was performed. Postoperative echocardiography showed good position of mitral bioprosthesis without paravalvular MR, intermediate aortic regurgitation and severe TR without visible vegetations. In a two-year follow up patient is clinically stabile and remains in NYHA II class. FIGURE 1. Aortic and mitral valve infective endocarditis. FIGURE 2. Endocarditis of the mitral valve and ruptured mitral chordae. FIGURE 3. Massive mitral regurgitation. FIGURE 4. Tricuspid valve endocarditis. FIGURE 5. Pulmonic valve endocarditis. **Conclusion**: Severe anemia may be a sign of infective endocarditis and postpone the diagnosis due to initial gastroenterological and hematologic work-up enabling severe destruction of affected valves.

Behija Hukeljić Berberović, Nermir Granov, Zina Lazović, Lejla Divović, Omer Perva

Prosthetic heart valve thrombosis is one of the most dreaded complications of mechanical heart valves. Suboptimal anticoagulation is the major risk for developing prosthetic heart valve thrombosis. Prosthetic heart valve thrombosis usually presents with dyspnea or embolic events (1). We present patient with prosthetic heart valve thrombosis without any symptoms, diagnosed via transthoracic echocardiography twenty days after mitral valve replacement during regular echocardiograph follow-up. In the medical history we obtained the information that the patient didn’t take anticoagulation drugs for five days after hospital discharge. Transthoracic echocardiography control revealed reduced leaflet mobility and high transvalvular gradients: MV maxPG 22mmHg, MV meanPG 12mmHg (**Figure 1**). Transesophageal echocardiography showed the presence of thrombus on prosthetic valve measuring 11x7 mm. Patient was readmitted to the hospital and treated with thrombolytic drug alteplase. According to the American College of Cardiology/American Heart Association Guidelines fibrinolysis can be considered in a thrombosed left-sided prosthetic heart valve, which is of recent onset (<14 days) with class I-II symptoms and a small thrombus on transesophageal echocardiography. Control echocardiography after treatment (**Figure 2**) showed MV meanPG 3.9mmHg and normal mobility of mitral valve. Successful thrombolytic therapy was followed by warfarin and intravenous unfractionated heparin until the INR is 3-4. FIGURE 1. Transthoracic echocardiography control revealed reduced leaflet mobility and high transvalvular gradients: MV maxPG 22mmHg, MV meanPG 12.3mmHg. FIGURE 2. Control echocardiography after treatment showed: MV maxPG 10mmHg, MV mean PG 3.9mmHg. Current American College of Cardiology/American Heart Association Guidelines assign Class I recommendation to transthoracic echocardiography or transesophageal echocardiography imaging in patient with prosthetic valve only in the presence of clinical symptoms or sings of valve dysfunction. Pathological studies and observational registries indicate that the risk of valve thrombosis highest in the first 3 month after surgical implantation of prosthetic valve, suggesting that anticoagulant thromboprophylaxis in this time frame may be beneficial. (2) Early follow up after surgical implantation is important because early detection and treatment of thrombus formation may lead to short-term reduction in the risk of TE events and long-term prevention of prosthetic valve degeneration.

Petra Angebrandt, Dejan Došen, Maja Hrabak, Eduard Margetić, Irena Ivanac Vranešić

**Introduction**: Pectus excavatum, a deformity of the sternum and ribs caused by an unbalanced costochondral hypertrophy, is a congenital abnormality with a prevalence of 1/1000 and it is commonly considered to be an incidental finding without clinical significance. In more advanced cases there may be a considerable compression and relocation of thoracic organs which can lead to serious cardiac or respiratory symptoms. (1-3) **Case report**: 19-year-old male patient was admitted for two-month history of palpitations, shortness of breath and continuous chest pain. Physical exam showed significant pectus excavatum and thoracic lordosis with otherwise normal findings. Resting 12-lead ECG and laboratory tests were normal, including cardiac troponin T levels. 24-hour ECG revealed premature ventricular beats with a paroxysm of non-sustained ventricular tachycardia. Echocardiography (**Figure 1**) showed normal biventricular size and function and raised suspicion of a large tumor mass compressing the left atrium (LA). No obvious signs of flow obstruction in LA using color and spectral Doppler were noticed. Mitral valve showed billowing of the anterior leaflet with otherwise normal valve function. The next diagnostic step was thoracic CT scan (**Figure 2**) which revealed extremely thin sagittal thoracic diameter with vertebrae compressing posterior wall of the LA and no signs of a tumor mass. Exercise stress test showed normal functional capacity with no signs of ischemia or arrhythmias in ECG. Patient was started on minimal dose of bisoprolol and follow up 24-hour ECG showed no ventricular arrhythmias. Since CT scan is the gold standard for determining the severity of the pectus excavatum defect, pectus severity index (PSI) was calculated and in our patient the value was 4.8. A normal chest has an average PSI of 2.5 and patients with a PSI of >3.25 are considered candidates for surgery. Patient was scheduled for additional respiratory function tests and will be referred to thoracic surgeon. FIGURE 1. Apical four chamber view of extra cardiac mass compressing left atrium. FIGURE 2. CT scan with calculation of pectus severity index. **Conclusion**: Severe cases of pectus excavatum can have significant impact on cardiorespiratory function and in those patients, surgery should be considered. On echocardiography, LA compression by vertebrae can even mimic a tumor mass but thoracic CT scan is a gold standard for diagnostic and severity assessment of this condition.

Ognjen Čančarević, Sandra Jakšić Jurinjak, Boris Starčević, Martina Menegoni, Daniel Unić

**Background**: Constrictive pericarditis (CP) is uncommon cause of predominantly right heart failure (1). In CP pericardium creates a stiff ventricular-pericardial unit which leads to increased diastolic pressures, limitation of ventricular relaxation and equalization of intracardiac diastolic pressures producing „single diastolic chamber” (2). **Case report**: 48-years-old male was admitted to Department of Gastroenterology for the investigation of upper abdominal pain. Investigations showed deranged liver biochemistry tests and computed tomography showed widespread ascites and small pleural effusion. Abdominal ultrasonography showed increased size of liver with dilated hepatic veins. The patient was referred to a cardiologist. A transthoracic echocardiogram (TEE) showed normal both ventricular dimensions, dyskinetic motion of intraventricular septum, small pericardial effusion without thickened pericardium. Inferior vena cava (IVC) was dilated with minimal respiratory variation. Because of nonconclusive TTE further diagnostic test including cardiac magnetic resonance imaging (MRI) was recommended. The patient was treated with diuretics and was discharged from hospital after clinical improvement. After three months he was admitted to Department of cardiology with signs and symptoms of right heart failure. On this admission, TTE showed paradoxical cardiac septal motion (“septal bounce”). Doppler inflow study showed respiratory variations of E-wave in mitral inflow (decreased >25% during inspiration) and increased E-wave during inspiration in tricuspid inflow. Pericardium was thickened (7mm) now without pericardial effusion. Estimated pulmonary artery pressure was around 40mmHg. The IVC was dilated without respiratory variation. MRI showed intensive T2 signal on pericardium, late gadolinium enhancement: pathologic imbibition in thickened pericardium. Right heart catheterization showed prominent x-descent and y descent, “square root“ sign, drop of left ventricular pressure and increased right ventricular pressure during inspiration, equalization of left ventricular end-diastolic pressure and end-diastolic right ventricular pressure (16mmHg). This finding was consistent with constrictive pericarditis. The patient was referred to a cardiothoracic surgeon and underwent a successful pericardiectomy. **Conclusion**: CP should be considered in all patients with unexplained right heart failure. Because diagnosis is sometimes difficult to establish it may be necessary to use multiple diagnostic tools.

Jadranka Šeparović-Hanževački, Viktor Peršić

CroEcho2019 is a three-day congress aimed at offering content for cardiologists and cardiology fellows dedicated to echocardiography and clinical decision-making. CroEcho2019 will focus on interesting cardiology topics from the area of clinical decision-making where echocardiography plays a central role. We will focus on tackling clinical scenarios, in which the making of a purposeful choice and the clinical interpretation of several imaging methods is of utmost importance. The program is composed of plenary lectures, scientific oral presentations, a teaching course, and interactive educational content (imaging workshops, practical work with a mentor, virtual practice). CroEcho2019 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). We look forward to welcoming you in Poreč!