Journal Research Assistant
Journal Research Assistant
Journal Research Assistant

Ivana Jurin, Josip Vincelj, Diana Rudan, Mario Udovičić, Sandra Jakšić Jurinjak, Jasmina Ćatić, Petra Vitlov, Tomislava Bodrožić Džakić Poljak
**Introduction**: Cor triatriatum is one of the rarest congenital cardiac anomalies accounting for 0.1-0-4% of all congenital heart disease. It is frequently associated with other congenital heart defects and is normally diagnosed in childhood mostly because it is symptomatic with symptoms of left heart obstruction or arrhythmia. (1-3) We present three cases of cor triatriatum sinister diagnosed in adult age with successful conservative treatment and follow up. **Case report**: First patient was a 30-year-old gravida with an asymptomatic, recently diagnosed cor tratriatum sinister. She has had a history of heart murmur since childhood, but has never been properly evaluated because she had good effort tolerance. Transthoracic (TTE) and transesophageal (TEE) 2D and 3D echocardiogram documented a fibromuscular membrane across the left atrium, dividing it into two compartments, with the proximal one receiving the pulmonary venous flow. Together with the gynecologist we decided to continue with the conservative approach. A healthy baby girl was successfully delivered at 38 weeks’ gestation, without any complications. Second patient was a 70-year-old woman that was referred to our emergency unit because of dyspnea and palpitations in the last two weeks, with more frequent episodes in the previous days and nonspecific anterior chest discomfort. TTE showed an abnormal membrane that bisects the left atrium into two chambers, an enlargement of right atrium, a mitral and tricuspid regurgitation and a reduced ejection fraction with pulmonary hypertension. On color Doppler was visualized a left-right (L-D) shunt with two jets. L-D shunt with two jets was confirmed on TEE, one jet on interatrial and membrane conjunction and second in proximal part of interatrial septum. The patient refused operative treatment and was successfully treated with pharmacological therapy. The third case was a 33-year-old men that was referred to TTE because of palpitations and a cor triatriatum without shunt was recognized. **Conclusion**: Cor triatriatum is a rare congenital heart disease with varying presentations. The golden standard in three dimensional TTE and TEE.
Rina Dalmatin, Teodora Zaninović Jurjević, Luka Zaputović, Alen Ružić
**Introduction**: Aneurysm of membranous interventricular septum is a rare cardiac abnormality and the prevalence is not known. It can be isolated but is usually associated with congenital heart disease, most often with ventricular septal defect. Most patients are asymptomatic but special caution should be taken in considering potential risk. (1, 2) **Case report:** 58-year-old female is coming because she had chest pressure with discomfort in back, fatigue and weakness. The blood pressure was 140/80 mmHg, heart rate of 80 beats/min and no heart murmur were discovered by clinical examination. Electrocardiography showed sinus rhythm, normal axis and right bundle branch block. The transthoracic echocardiogram discovered and the transesophageal confirmed the aneurysm of the membranous intraventricular septum without septal defects, pathological flows or intracavital masses. **Conclusion:** Aneurysm of membranous interventricular septum is occasional cardiac abnormality and the patients are often asymptomatic. Transthoracic echocardiography, transesophageal echocardiography, CT and MR imaging are methods of choice for diagnosis of VSA aneurysm. Patients with VSA have risk of thrombus formation and stroke, ventricular tachycardia and AV block, aortic insufficiency, intracardiac shunts, ruptures and endocarditis.
Ivana Grgić, Davorka Lulić, Sandro Brusich, Aleksandra Ljubačev, Slavica Kovačić, Luka Zaputović, Teodora Zaninović Jurjević
**Introduction**: Myxomas are the most common benign primary cardiac tumors with the majority (85%) located in the left atrium. Clinical manifestations are diverse and are consequence of embolic phenomena, intracardiac obstruction or constitutional symptoms. Early diagnosis, most perplexing in a patient with constitutional symptoms, remains an important challenge for the clinician, as they have an excellent prognosis following surgical excision, preventing complications and improving quality of life. (1-3) **Case report**: We report a case of a 75-year-old female presented with fatigue and weight loss. Symptoms were first attributed to her history of hyperthyroidism. After series of normal hormonal and thyroid ultrasound findings she was referred to gastroenterologist for the evaluation of microcytic anemia (Hb 101g/L). Even though results of fecal occult blood testing were negative, esophagogastroduodenoscopy and colonoscopy were performed subsequently having sent our patient to hematologist for further examinations regarding anemia. After a year of anemia assessment she underwent transthoracic echocardiography which demonstrated an intraatrial mass. Immediately hospitalized, transesophageal echocardiography (**Figure 1**, **Figure 2**) and magnetic resonance imaging (**Figure 3**) were performed. Features of the mass were consistent with notably large myxoma, occupying nearly the entire left atrium and prolapsing across the mitral valve during diastole. Coronary angiography showed no obstructive coronary artery disease. Our patient underwent urgent cardiothoracic surgery with successful excision of atrial mass attached to the interatrial septum, pathologically confirmed myxoma (**Figure 4**). Cardiac rehabilitation followed immediately after discharge, with normalization of hemoglobin levels. Figure 1. Long axis transesophageal echocardiography showing prolapsing atrial myxoma in A) systole and B) diastole. Figure 2. Parasternal long axis view with left atrial myxoma in A) systole and B) diastole. Figure 3. Magnetic resonance imaging of left atrial myxoma prolapsing across the mitral valve. Figure 4. Surgically excised myxoma, 9x2cm large with thrombus on the surface. **Conclusion**: Diagnosis of atrial myxomas is often challenging due to their asymptomatic nature or nonspecific symptoms presentation. Constitutional symptoms, like weight loss and fatigue and associated laboratory findings (elevated sedimentation rate and anemia) are explained as a result of interleukin-6 production by cardiac myxoma cells. Although histopathologically benign, complications due to their position and embolization can be fatal. Therefore, prompt complete surgical excision after diagnosis, regardless of whether the patient is symptomatic, is strongly recommended. The present case summons the significance of considering atrial myxoma while investigating a patient presenting with constitutional symptoms and persistent unexplained anemia. Early suspicion supported by echocardiogram may facilitate the diagnosis.
Daniel Lovrić, Marijan Pašalić, Vlatka Rešković Lukšić, Dejan Došen, Kristina Gašparović, Jana Ljubas Maček, Zvonimir Ostojić, Marija Brestovac, Jadranka Šeparović Hanževački
**Background:** It has been shown that 2D strain (2DS) is a sensitive indicator for subclinical myocardial injury due to various agents. However, for detecting coronary artery disease (CAD) global longitudinal 2DS has shown only modest diagnostic accuracy, but the sensitivity of regional 2DS assessment has not been adequately evaluated. (1-6) The purpose of this study was to assess the value of regional 2DS performed early in patients with non-ST elevation acute coronary syndrome (NSTE-ACS) for predicting presence and localization of ischemia-inducing stenosis, which could then lead the team in further decision-making. **Methods:** We performed a retrospective analysis of patients admitted through our Emergency Department to the Department for Cardiovascular Diseases from January 2013 till December 2015 with the diagnosis of NSTE-ACS. Patients who did not undergo coronary angiography, patients with known prior coronary artery disease and patients who did not receive an echo in the 24 hours prior to angiography were excluded, as were the patients with images not adequate for 2D strain analysis. A total of 123 patients fulfilled the criteria and were included in the analysis. 4 different clinicians blinded to laboratory and ECG results performed 2DS analysis of global and regional 2D longitudinal peak systolic strain (LPSS) according to the 18-segment model prior to coronary angiography. **Results:** We found significant correlation of a flow limiting stenosis, defined as a stenosis of >70% narrowing on coronary angiography, with segmental decrease in LPSS for all three major coronary vessels. Patients with significant left anterior descending artery (LAD) lesion had significantly lower regional LPSS in anterior, septal and anterolateral mid segments and all apical segments (**Table 1**), as well as global LPSS (-14.43 vs. -17.22, p<0.0001) when compared to patients without significant stenosis in that vessel. Patients with a significant stenosis in LCx had lower global LPSS in basal and mid anterolateral and inferolateral segments, while patients with a significant RCA stenosis has lower global LPSS in basal and mid inferior and basal inferolateral segments (**Figure 1**). Statistically significant difference was present in all myocardial layers and was present even after accounting for potentially confounding factors like arterial hypertension, smoking, alcohol, atrial fibrillation, valvular disease, age or prior medical therapy. ### Table 1: Regional 2D longitudinal peak systolic strain values according to segments and location of coronary stenosis. | Segment | Lession location | LPSS values (normal vs. pathologic) | *P* | | --- | --- | --- | --- | | Mid anterior | LAD | -15.54 vs. -10.93 | <0.0001 | | Apical anterior | LAD | -20.68 vs. -14.08 | <0.0001 | | Mid anterolateral | LAD | -14.20 vs. -11.38 | 0.003 | | Apical anterolateral | LAD | -20.35 vs. -14.26 | <0.0001 | | Mid anteroseptal | LAD | -19.55 vs. -15.33 | <0.0001 | | Apical anteroseptal | LAD | -22.89 vs. -16.15 | <0.0001 | | Mid inferoseptal | LAD | -16.28 vs. -13.88 | 0.008 | | Apical inferoseptal | LAD | -24.00 vs. -17.15 | <0.0001 | | Apical inferior | LAD | -22.67 vs. -18.31 | 0.01 | | Apical inferolateral | LAD | -19.36 vs. -14.27 | 0.001 | | Basal anterolateral | LCx | -14.76 vs. -10.92 | 0.001 | | Mid anterolateral | LCx | -14.12 vs. -10.96 | 0.003 | | Basal inferolateral | LCx | -16.28 vs. -12.09 | <0.0001 | | Mid inferolateral | LCx | -15.19 vs. -12.28 | 0.008 | | Basal inferior | RCA | -16.39 vs. -11.97 | <0.0001 | | Mid inferior | RCA | -16.78 vs. -14.27 | 0.02 | | Basal inferolateral | RCA | -15.58 vs. -12.56 | 0.01 | [†] LAD = left anterior descending coronary artery; LCx = left circumflex coronary artery; RCA = right coronary artery; LPSS – longitudinal peak systolic strain. Figure 1. Example of significantly reduced regional 2D longitudinal peak systolic strain (**A**) in a non-ST elevation acute coronary syndrome patient with ostial 95% significant left anterior descending artery stenosis and preserved anterior wall myocardial thickness (**B**). **Conclusion:** We have shown that there is significant correlation between a decrease in LPSS and localization of significant stenosis in patients with NSTE-ACS. The segments with significantly decreased LPSS closely match the areas of perfusion of each coronary vessel. It seems regional 2DS can help define the territory involved in NSTE-ACS and lead further clinical and interventional decision making, but further prospective studies are needed to confirm this assumption.
Fatmir Ferati, Anida Ferati, Ardian Preshova, Mentor Karemani
**Introduction:** The aim of this study was to analyze the function of left atrium (LA) (1) in patients with left bundle branch block (LBBB). **Patients and Methods**: 20 patients without verified cardiovascular disease and 20 with LBBB were examined for left ventricular (LV) and LA function quantification. **Results**: We obtained lower values of EF of LV, 38.33% in patients with LBBB, against 60.81% in patients without LBBB (**Table 1**). The global systolic strain of LV in patients without LBBB was 18.50% against 11.80% of the group with LBBB. The global circumferential strain of LV in patients without LBBB was 30.86% compared to 26.57% in patients with LBBB. EF of LA was 69.9% in patients without LBBB compared to 71.38% in patients with LBBB. The endsystolic volume of LA (ESVLA) in patients without LBBB was 30.1 ml/m2 compared to 45.5 ml/m2 in patients with LBBB. The enddiastolic volume of LA (EDVLA) in patients without LBBB was 6.5 ml/m2 compared to 12.53 ml/m2 in patients with LBBB. LA function timing intervals, expressed through dV/dT of early diastolic atrial emptying volume (EDAEV) in patients without LBBB was 221.5 ml/m2 compared to 95.46 ml/m2 in patients with LBBB, whereas dV/dT of atrial contraction emptying volume (ACEV) in patients without LBBB was 135.8 ml/m2 compared to 203.46 ml/m2 in patients with LBBB. The dV/dt of EDAEV and ACEV ratio is 1.63 in patient without LBBB against 0.46 in patients with LBBB. EDAEV in patients without LBBB was 20.66 ml compared to 9.23 ml in patients with LBBB, whereas the ACEV for the group without LBBB was 10 ml compared to 23.84 ml for the group with LBBB. The EDAEV/ACEV ratio for patients without LBBB was 2.1 compared to 0,4 for the group with LBBB. The systolic expansion time of LA (SET) was 333.33 msec vs 504.76 msec of subjects with LBBB, whereas the RR interval (msec) in patients without LBBB was 780 msec against 744.53 msec in patients with LBBB. The ratio between the LA (SET) and the RR interval (SET/RR interval index) for the group without LBBB was 0.42 against 0.69 for the group with LBBB. ### Table 1: Echocardiographic values according to the presence of left bundle branch block. | VARIABLE | WITHOUT LBBB | WITH LBBB | p value | | --- | --- | --- | --- | | EF LV (%) | 60.81 | 38.33 | 2) | 30.1 | 45.15 | 2) | 6.5 | 12.53 | 2) | 221.5 | 95.46 | 2) | 135.8 | 203.46 | 2) | 20.66 | 9.23 | 2) | 10 | 23.84 | <0.001 | | EDAEV/ACEV index (%) | 2.1 | 0.4 | <0.001 | | RR interval (msec) | 780 | 744.53 | <0.001 | | SET (msec) | 333.333 | 504.76 | <0.001 | | SET/RR interval index (%) | 0.42 | 0.69 | <0.001 | [†] LBBB = left bundle branch block; LV = left ventricle; LA = left atrium; EF of LV = ejection fraction of LV; GLS of LV = global systolic strain of LV; GCS of LV = global circumferential strain of LV; SDI of LV = systolic dyssynchrony index of LV; EF LA = ejection fraction of LA; ESVLA = end systolic volume of LA; EDVLA = end diastolic volume of LA; EDAEV = early diastolic atrial emptying volume; ACEV = atrial contraction emptying volume; SET = systolic expansion time of LA. **Conclusions**: **A)** LBBB does not have influence on the value of EF of LA but in dynamic of atrial functioning. **B)** LBBB causes reduction of EF of VM. **C)** LBBB causes an increase of ESVLA and EDVLA of the LA. **D)** LBBB reduces EDAEV and increase ACEV. **E)** LBBB increases ACEV. **F)** LBBB causes elongation of the SET of LA.
Andreja Černe Čerček, David Bele, Nika Kojc, Zeljka Večerić-Haler
**Introduction**: Bacterial and viral infections may trigger different kind of autoantibody production, including PR3 and MPO ANCA. We report a case of subacute bacterial endocarditis in patient with neurofibromatosis type I presenting as c-ANCA/PR3-ANCA glomerulonephritis (GN). **Case report**: 57-year-old man with neurofibromatosis type 1 and known mitral valve prolapse, prolonged dry cough, and progressive cachexia was admitted to the hospital due to unexplained renal failure with acute nephritic syndrome, sudden unilateral loss of hearing, vertigo, malaise, new onset hemolytic anemia and thrombocytopenia. Common site of infection, as well as solid malignoma were ruled out. Head MRI showed many microscopic hemorrhages. Immunoserology revealed positive c-ANCA/PR3-ANCA and mixed type cryoglobulinemia with low complement fraction C3. The kidney biopsy findings were typical for acute necrotizing crescentic immune complex GN (**Figure 1**). Due to possible unrecognized infection and coexistence of ANCA-associated vasculitis he was initially treated with intravenous immunoglobulin infusion. Following the kidney biopsy the patient suddenly developed clinical signs of sepsis. Blood cultures revealed Streptococcus cristatus and mitral valve endocarditis was diagnosed (**Figure 2**). Antibiotic as well as glucocorticoid therapy was started and surgical treatment of mitral valve endocarditis was performed immediately. His serum creatinine level gradually decreased, minimal glomerular erythrocyturia persisted, c-ANCA/PR3-ANCA and cryoglobulins disappeared, C3 level was normal. Second kidney biopsy (**Figure 1**) was performed to tailor further therapy, and showed the resolution of previously reported findings and steroid therapy was discontinued. Figure 1. First (left) and second (right) kidney biopsy. A) Diffuse proliferative glomerulonephritis with focal glomerular necrosis and extracapillary crescent formation (left) and almost normal renal parenchyma with scarce segmental mesangial proliferation (right). B) Immune complex glomerulonephritis in the first biopsy disappeared in the second biopsy Figure 2. Transesophageal ultrasonography shows a large vegetation on the P2 scallop of the posterior mitral leaflet which prolapses into the left ventricle during systole **Conclusion**: We present the first report of c-ANCA/PR3-ANCA positive immune complex GN associated with infective endocarditis in neurofibromatosis type I. It is particularly important to be aware of overlapping conditions to make diagnosis in time, as this may have crucial consequences on proper therapy and patient’s survival.
Petra Angebrandt, Dejan Došen, Vojtjeh Brida, Eduard Margetić
**Introduction**: Primary cardiac tumors are extremely rare. The incidence is less than 0.1 percent. Benign myxomas are the most common tumors and they account for 27% of primary cardiac tumors. Other benign tumors include papillary fibroelastomas, hemangiomas and mesotheliomas. Malignant primary cardiac tumors are rare. In adult, angiosarcomas, rhabdomyosarcomas, mesotheliomas and fibrosarcomas are seen. Metastatic involvement of the heart is over 20 times more common. Nonprimary cardiac tumors can include: lung and breast cancer, lymphoma, melanoma. Echocardiography is widely available and provides a simple, non-invasive technique for the initial evaluation. The major treatment is prompt surgical resection because of the risk of embolisation or other cardiovascular complications. (1-3) **Case report 1**: MYXOMA: 62-years-old female patient with cardiac mass in left atrium, echo characteristic of myxoma with three episodes of cerebrovascular event prior to echocardiography diagnostic (**Figure 1**). Figure 1. Transesofageal image of myxoma in left atrium. **Case report 2**: LYMPHOMA: 69-years-old male patient with primary malignant cardiac tumor: diffuse large B cell lymphoma that invaded pericardium, left and right atrium, interatrial septum and left ventricle. The diagnose was made by biopsy and afterwards chemotherapy was performed with successful results (**Figure 2**). Figure 2. Parasternal short axis view in the aortic valve level of lymphoma of the heart: tumor is surrounding the aorta. **Case report 3**: HAEMANGIOMA: 53-years-old female patient with cardiac mass on posterior mitral valve leaflet with wide base on cusps. The mass was vascularized with small branch of the left circumflex artery which was seen during coronarography procedure. Patohistology showed that the mass was haemangioma of the heart (**Figure 3**). Figure 3. Long axis parasternal view of the solid tumor on the posterior mitral valve leaflet. **Case report 4**: RIGHT VENTRICLE CARDIAC MASS WITH RVOT OPSTRUCTION: 66-years-old male patient with cardiac mass in right ventricle (3 × 6 × 8.5 cm in dimension) with RVOT obstruction and with secondary 5 cm big lesion in the liver highly suspected for hepatocellular carcinoma. MRI showed no signs of primary cardiac tumor, rather tumor metastasis (**Figure 4**). Figure 4. Parasternal short axis view in the aortic valve level of the rigtht ventricle mass with obstruction of the right ventricular outflow tract. **Conclusion**: Sometimes pathologically benign cardiac tumors can have „malignant“ hemodynamic consequences if it obstructs the normal pattern of the blood flow. Thus, the echocardiography examination includes definition of both the anatomic extent of a cardiac tumor and its physiologic consequences. Even though, cardiac tumors are rare, sometimes we can find the most „strange“echocardiography and clinical manifestation.
Jadranka Šeparović, Hanževački, Viktor Peršić
Dear colleagues, **CroEcho2017** is a bi-annual Croatian echocardiography congress with international participation, which has developed from previous national echocardiography meetings and symposiums and which keeps gathering cardiologists focusing not only on echocardiography but also those who are focused on other areas of cardiology. Two years have passed since our previous **CroEcho2015** congress that was held in Opatija. This period has been marked by the improvement and development of new cardiologic percutaneous interventions and cardio support as well as complex imaging and hemodynamic reconstructions. The application of new insights in clinical cardiology in everyday practice, especially echocardiographic knowledge and skills, is an important part of our work and our main driver. Heart MRI and MSCT imaging have significantly contributed to a better understanding of the pathophysiological processes which occur in a wide range of disease syndromes, thus enabling a more precise choice of the treatment modality as well as individual and optimized evidence-based medical treatment. The cardiology of today thus becomes more propulsive and, in cooperation with other complementary specialists, enables a continuous improvement of the quality of care for the heart patient as well as an improvement of the long-term prognosis for the patient. Through clinical review, echocardiography, and imaging presentations in the heart team, we are putting the patient back in the center of our clinical work. Additionally, by putting together a group of specialized cardiology experts focused on solving clinical problems through the exchange of various opinions, we are able to achieve true advances in our profession and aim at achieving the biggest benefits for our patients. These important issues are the cornerstone of modern cardiology and, more than ever, clinical echocardiography is to take this role. This year’s **CroEcho2017** congress will focus on taking an integral view of the patient and on problem-solving in a team. This program is aimed at boosting professional excellence in making a correct and timely cardiac diagnosis and purposeful treatment of the patient. It is our aim to offer you an education which is in accordance with the European and global standards in the areas of basic echocardiography, advanced echocardiography methods, and other imaging modalities in cardiology. The quality of the professional and practical content of our course in echocardiography has been verified by the Education Board of the European Association of Cardiovascular Imaging (EACVI) of the European Society of Cardiology, which has granted our course the status of an EACVI Extramural course. The scientific part of the congress contains original research results presented as extended abstracts, plenary sessions, round tables, and announcements. We highlight the research on strain analysis in acute coronary syndromes and aortic stenosis, specifics in estimating degenerative mitral stenosis and rare cardiomyopathies, as well as interesting case reports where echocardiography influenced the treatment course and outcome of the disease. Finally, **CroEcho** is an opportunity to meet renowned international and national experts and to share scientific ideas and clinical experience in a warm and collegial atmosphere. Congress directors Jadranka Šeparović Hanževački Viktor Peršić
Darja Milovanovič Jarh, Mateja Grošelj, Breda Barbič-Žagar
Cardiovascular disease is the leading cause of death worldwide. Among the risk factors, hyperlipidemia has been associated with the highest population attributable risk for cardiovascular disease. Despite many proven therapies and decades of their use, cardiovascular event rates remain high. International guidelines have identified the problem of statins being prescribed at the lowest dose and often not up-titrated to attain the goal of treatment. The fact that a majority of patients are treated with the initial low doses of statins and that doctors rarely reach for the higher doses indicates a need for intermediate doses. In order to bridge this gap, Krka produces a wide range of rosuvastatin strengths, including the intermediate strengths of 15 and 30 mg. This additional strengths enables treatment to be adjusted to the requirements of individual patients and increase the percentage of patients reaching target lipid levels.
Nina Jakuš, Ivo Planinc, Hrvoje Jurin, Marijan Pašalić, Dora Fabijanović, Daniel Lovrić, Boško Skorić, Jure Samardžić, Jana Ljubas Maček, Hrvoje Gašparović, Bojan Biočina, Maja Čikeš, Davor Miličić
**Background:** Hypereosinophilic syndrome is a hematologic disorder characterized by an elevated eosinophil count in the peripheral blood and eosinophillic infiltration of other organs, resulting in end organ damage (1, 2). Such eosinophilic infiltration of the heart may result in the development of a restrictive cardiomyopathy, leading to symptomatic heart failure. **Case report:** The patient was 23 years old at the time of diagnosis, previously a healthy young man. His initial complaint was exertional dyspnea, paroxysmal nocturnal dyspnea and peripheral edema. Laboratory findings revealed a significantly elevated eosinophil count (41% of the total number of leukocytes), as well as elevated levels of NT-proBNP (3784 pg/mL). He was then referred to our center from a local hospital. Initial echocardiographic examination revealed a slightly dilated left ventricle, with signs of a restrictive cardiomyopathy. Apical 4 chamber view revealed that the apex of the left ventricle is obliterated by a substance, suspected a thrombus formation (**Figure 1**). Magnetic resonance performed with gadolinium contrast revealed diffuse fibrosis of the endocardium and a severely hypokinetic apex of the left ventricle, filled with a thrombus mass (dimensions: 2.7x2.5cm), concluding that the finding is characteristic for endocardial fibroelastosis. In coordination with a hematologist, the patient was started on imatinib, for the eosinophilia, and low molecular weight heparin, as well optimal medical treatment for heart failure. During one of the scheduled checkups, a follow up echocardiographic examination revealed a significant reduction in the overall size of the thrombus mass, but now, with a peduncular formation protruding into the ventricle, moving freely in the blood current (**Figure 2**). At that time, the patient had exhibited a significant clinical improvement, and was regularly undertaking physical activity. Due to a significant embolization risk, he was urgently referred to cardiac surgery, where a successful surgical excision of the thrombus formation was performed, through a medial sternotomy. Figure 1. Initial echocardiogram indicating the existence of a thrombus formation in the apex of the left ventricle. Figure 2. Follow up echocardiogram performed after 7 months of treatment, showing a significant reduction in the overall size of the thrombus mass, but also a peduncular formation protruding into the ventricle. **Conclusion:** Thrombus formation in patients with restrictive cardiomyopathy due to hypereosinophilic syndrome has been previously described in literature (3). On the other hand, complications of treating such thrombus mass are rarely described. This case report stresses the need for close echocardiographic monitoring during the dissolution of such large thrombus masses, anticipating potential embolic complications.
Jana Ljubas Maček, Vlatka Rešković Lukšić, Marijan Pašalić, Marija Brestovac, Borka Pezo Nikolić, Martina Lovrić Benčić, Jadranka Šeparović Hanževački
**Background:** After cardiac resynchronization therapy (CRT), AV delay is usually set by “out of box” value while optimal VV interval is set using intracardiac electrocardiogram method (IEGM) according to the narrowest QRS width (**Figure 1**), regardless of the actual mechanical response of the left ventricle (LV). (1, 2) Our aim was to assess the presence of residual mechanical dyssynchrony after IEGM device optimization using echocardiographic parameters and to define echocardiographic and functional response in patients with early echo-guided optimization (“zero” OPT). Figure 1. Global longitudinal strain in a patient after IEGM-based device optimization is severely reduced to -6.8%. **Patients and Methods**: 98 CRT patients (Pts), with native LBBB, were included in the study. Early post implantation IEGM device optimization was performed in all Pts followed by „zero“-OPT prior to discharge in only 44 Pts – group 1. Other 54 Pts formed the control group – group 2. In Group 1, LV preexcitation (in sequential 10 ms intervals) was modified according to the presence of early presystolic septal motion („septal flash“), LV ejection fraction (LVEF) and global longitudinal strain (GLS). Pts were followed for one year after CRT implantation and their functional capacity was evaluated using six-minute walk test/stress test/cardiopulmonary stress test. They were classified into NYHA class according to the results of the testing. The same functional evaluation was performed prior to CRT implantation in all Pts. **Results:** There was no difference in mean biventricular paced QRS duration between the groups before CRT-optimization: group 1 vs. group 2 - 169,7 vs 177,9 ms, as well as after one year follow-up - 134,3 vs. 137,6 ms, with no difference in biventricular pacing rate - 98,4 vs 97,7%. Mean IEGM VV delay was 16 ms, after “zero” OPT mean VV delay was significantly prolonged to 36 ms). Interventricular mechanical delay (IV MD) also has not been changed significantly (mean 35,3 vs 30,0 ms). Mean IEGM AV delay (112 ms) was very slightly modified to mean SAV of 115 ms. Early after implantation diastolic dysfunction was mostly grade 1 (77% Pts), with grade 2 present only in 11.7% and grade 3 in 8.8%. Mean E/e’ ratio was 14 with diastolic filling time corrected for RR interval 43.1±1.6%. In the group 1, after IEGM optimization 55.2% of Pts still had the presence of septal flash, 62% had prolonged septal-to-posterior wall mechanical delay (≥130 ms) and 59% prolonged tissue-doppler septal-to-lateral delay (SL delay ≥60 ms). Statistically significant improvement after “zero” OPT in many echocardiographic parameters was proven - for LV EF (before vs after 28,0 vs 31,1%), LVESV (173,2 vs 162,5 ml), GLS (-7,55 vs -8,78), LVOT VTI (13,5 vs 15,7 cm), LV ET (250 vs 272 ms) and other. In all Pts functional improvement in NYHA class was revealed during follow up. However, in spite of very good hemodynamic response, significant difference in functional improvement between group 1 and group 2 was not reached. **Conclusion:** Significant number of our patients after the first IEGM-based device optimization still has the presence of septal flash with its unfavourable consequences of intraventricular mechanics. In a great percentage of our patients residual septal-to-posterior mechanical delay was also found. LV intraventricular dyssynchrony represented the most important type of residual dysynchrony in our study that required significant change in initial VV delay (pacing interval between left and right lead) in most Pts. In our study we found that in spite of optimal paced QRS width, the majority of the patients still have unsatisfactory mechanical response that could be improved by echocardiography-based CRT optimization immediately after implantation, in order to attain the precise mechanical synchrony of LV wall motion followed by reverse remodelling of the LV.
Tanja Mikulandra, Mihaela Roguljić, Andrijana Erak
Anderson-Fabry disease is a hereditary defect of the enzyme alpha-galactosidase, characterized by the accumulation of glycosphingolipids in lysosomes. Patients with Fabry disease are not able to catabolize the membrane neutral glycosphingolipids, especially globotriaosylceramide, which therefore accumulates mainly in the heart, skin, kidneys, blood vessels and central nervous system. The disease is recessive and sex-linked, it is being transmitted with the X chromosome. Characteristic symptoms and signs of Fabry disease are the presence of angiokeratomas (the occurrence of vascular skin lesions), acroparesthesias (periodic painful crises in the extremities), hypohidrosis (the inability to sweat) and the characteristic blurring of the cornea. The disease starts at birth, during childhood it is usually without clinical significance, organ damages become manifest during the fourth decade of life in males and during fifth decade in females. The heart manifestations of the disease are a result of accumulated globotriazilceramides in cardiomyocytes, conduction system cells and valves, with the main consequence being hypertrophic cardiomyopathy of the left or both ventricles. In an advanced stage of the disease, the cardiac muscle changes can lead to heart attack, cardiomyopathy and conduction disturbances. Echocardiography is an excellent noninvasive diagnostic method for the detection of Fabry disease (left ventricular hypertrophy, state of the heart valves and dimension of cardiac chambers). Doctors’ and nurse technicians’ coordination is obligatory for optimal echocardiographic examination, detection and further follow up of the disease. In patients with Fabry disease suspicion, fingerstick or venous blood samples were taken. After the drying process, samples were sent for enzymatic activity and genetic testing. The follow-up of the disease is carried out by the echo controls, determination of biomarkers (GL3, Lyso GL3) and antibodies during the application of enzymatic replacement therapy. (1-3)
Helena Antić, Kauzlarić
Cardiac magnetic resonance (CMR) imaging is a well-established noninvasive imaging modality in clinical cardiology. Its unsurpassed accuracy in defining cardiac morphology and function and its ability to provide tissue characterization make it well suited for the study of patients with cardiac diseases. With or without administration of an exogenous contrast agent, cardiac MRI affords detailed myocardial tissue characterization via various segmented as well as single heart beat approaches. Late gadolinium enhancement was a major advancement in the development of tissue characterization techniques, allowing the unique ability of CMR to differentiate ischemic heart disease from nonischemic cardiomyopathies. Using T2-weighted techniques, areas of edema and inflammation can be identified in the myocardium. To characterize other myocardial features, imaging techniques that capture intrinsic contrast in T1, T2 and other MR-based relaxation parameters are often incorporated into the cardiac MRI examination. Accruing evidence suggests that quantitative approaches, also known as tissue mapping techniques, are helping to further advance MR-based myocardial characterization. A new generation of myocardial mapping techniques are emerging, enabling direct quantitative assessment of myocardial tissue properties in absolute term. (1-3) This review will summarize recent developments involving T1-mapping and T2-mapping techniques and focus on the clinical applications and future potential of these evolving CMR methodologies.
Daniel Lovrić, Kristina Gašparović, Vlatka Rešković Lukšić, Marijan Pašalić, Dejan Došen, Jana Ljubas Maček, Zvonimir Ostojić, Marija Brestovac, Jadranka Šeparović Hanževački
**Background:** Visual assessment of regional wall motion abnormalities (RWMA) on echocardiography represents the current standard in assessing the impact of coronary artery disease (CAD) induced changes in myocardial contractility. Although it has been proven to predict long-term outcomes it has been hard to rely on in acute situations due to the patient dependent variance in image acquisition quality and interoperator variability. (1-4) We hypothesized that regional 2DS assessment due to the evaluation of longitudinal shortening that is barely visible to the naked eye could potentially be more sensitive than visual RWMA assessment in detecting ischemia induced loss of contractility in non-ST elevation acute coronary syndrome (NSTE-ACS). **Methods:** We performed a retrospective analysis of patients admitted through our Emergency Room to the Cardiology Department from January 2013 till December 2015 with the diagnosis of NSTE-ACS. Patients who did not undergo coronary angiography, patients with known prior coronary artery disease and patients who did not receive an echo in the 24 hours prior to angiography were excluded, as were the patients with images not adequate for 2D strain analysis. A total of 123 patients fulfilled the criteria and were included in the analysis and 4 different clinicians blinded to laboratory and ECG results performed 2DS analysis of global and regional 2D longitudinal peak systolic strain (LPSS) according to the 18-segment model prior to coronary angiography. Regional wall motion abnormalities (RWMA) as interpreted by the clinician performing the original echo exam were categorized according to the wall motion score guidelines. **Results:** RWMA assessment shows good predictive power of coronary artery stenosis location in LAD and LCx, but not in RCA (**Table 1**). However, LPSS was significantly more precise overall (mean sensitivity 75.6% vs 39.5%, P<0.001). Statistically significant difference was present even after accounting for potentially confounding factors like arterial hypertension, smoking, alcohol, atrial fibrillation, valvular disease, age or prior medical therapy. ### Table 1: Regional visual wall motion assessment 2D longitudinal peak systolic strain values according to segments and location of coronary stenosis. | Segment | Lession location | *P* | | --- | --- | --- | | APLAX Basal anteroseptum | LAD | 0.098 | | APLAX Mid anteroseptum | LAD | <0.0001 | | APLAX Apical anteroseptum | LAD | <0.0001 | | APLAX Apical inferolateral | LCx | <0.0001 | | APLAX Mid inferolateral | LCx | 0.04 | | APLAX Basal inferolateral | LCx | 0.01 | | A4C Basal inferoseptal | RCA | 0.087 | | A4C Mid inferoseptal | LAD | 0.1 | | A4C Apical inferoseptal | LAD | <0.0001 | | A4C Apical anterolateral | LAD | 0.003 | | A4C Mid anterolateral | LCx | 0.366 | | A4C Basal anterolateral | LCx | 0.015 | | A2C Basal inferior | RCA | 0.133 | | A2C Mid inferior | RCA | 0.722 | | A2C Apical inferior | LAD | 0.15 | | A2C Apical anterior | LAD | <0.0001 | | A2C Mid anterior | LAD | 0.027 | | A2C Basal anterior | LAD | 0.032 | [†] LAD = left anterior descending coronary artery; LCx = left circumflex coronary artery; RCA = right coronary artery; APLAX = Apical long axis view; A4C = Apical four chamber view; A2C = Apical two chamber view. **Conclusion:** We have shown that a decrease in LPSS is significantly more accurate in detecting ischemia-induced loss of myocardial contractility than the visual assessment of RWMA in patients with NSTE-ACS. Our findings imply that 2DS should be employed as a supplementary tool during the echo assessment of patients with NSTE-ACS.
Vesna Pehar Pejčinović, Viktor Peršić, Marko Boban, Marijana Rakić, Helena Antić Kauzlarić, Vladimir Peša
Hypertrophic cardiomyopathy (HCM) and left ventricular noncompaction (LVNC) are both genetically determined and familial diseases. Hypertrophic cardiomyopathy (HCM) is defined as hypertrophy of the myocardium more than 1.5cm, without another identifiable cause, such as long-standing hypertension, amyloidosis, aortic stenosis, glycogen storage disease. Many of the mutations associated with HCM involve the cardiac sarcomeric proteins and include actin, myosin, or troponin component of the sarcomere and it is most frequently transmitted as an autosomal dominant trait. Left ventricular noncompaction is a rare congenital cardiomyopathy which is characterized by the presence of a thin, compacted epicardial layer and a non-compacted thicker endocardial layer of myocardium, with prominent trabeculation and deep recesses communicating with the cavity of the left ventricle. The cause of the disorder has been identified as mutations in genes associated with the mitochondrial function, like G4.5 which encodes the protein tafazzin, genes related with the cytoskeleton, like those of alpha-dystrobrevin or dystrophin, genes that code proteins of the Z line of the sarcomere, like LDB3, which codes the protein Cypher/ZASP, genes of the internal nuclear membrane proteins (LMNA, which encodes lamin A/C) and even genes that code sarcomeric proteins like cardiac alpha-actin and the beta-myosin heavy chain and cardiac troponin T. The clinical picture of both diseases, HCM and LVNC, varies from mild forms until severe forms with heart failure and complex ventricular arrhythmias. LVNC and HCM may appear as overlapping entities. Cases of patients sharing both the LVNC and HCM phenotypes have been already published, and it is speculated that mutations in sarcomere protein genes known to cause hypertrophic cardiomyopathy and dilated cardiomyopathy may be associated with left ventricular noncompaction. (1-5) In our case report, we are presenting patient with clear overlapping pheenotyp for LVNC and HCM, using the imaging method cardiac MRI.
Diana Rudan, Ivana Jurin, Sandra Jakšić Jurinjak, Josip Vincelj
Non-invasive assessment of regional myocardial function is important for diagnosing of myocardial disease, assessing therapeutic interventions, and predicting clinical outcomes. Although there are other non-invasive techniques for assessment of myocardial function, such as magnetic resonance imaging and computed tomography, echocardiography remains the tool of choice in widespread clinical use because of the ease of its usage. Echocardiographic strain imaging, also known as deformation imaging, is a technological advancement that has been developed to objectively quantify regional myocardial function (1, 2). Currently, most echo-laboratories use speckle tracking computer processing to assess myocardial function in many diseases. In this paper, we will show the role that speckle tracking plays in our daily clinical routine.
Marina Prpić, Danijel Planinc, Maja Franceschi
**Introduction**: Amyloidosis is an infiltrative disease characterized by extracellular deposition of insoluble fibrillary protein. There are two major types: light-chain (AL) amyloidosis and transthyretin- related cardiac amyloidosis (ATTR, mutant and wild type). Cardiac involvement can lead to restrictive cardiomyopathy (CMP) and heart failure. 99mTc-Pyrophosphate (99mTc-PPY) has high affinity for TTR amyloid, allowing differential diagnosis with AL and other nonamyloidotic CMP with hypertrophic phenotype, in which 99mTc-Pyrophosphate (PYP) is low or absent. (1-4) **Patiens and Methods**: We are presenting four patients with confirmed diagnosis of cardiac amyloidosis, as follows: 54-year-old lady hospitalized for evaluation of progressive dyspnea and chest pain; 69-year-old lady, with history of hypertension, hospitalized for congestive heart failure; 50 and 64 old gentlemen with progressive dyspnea and intolerance of exertion. Whole body, anterior and lateral static images and SPECT/CT with heart centered in the field of view, were performed 1 and 3 hours after intravenous injections of 740MBq (20mCi) 99mTc-PYP. **Results**: In the first patient scintigraphic planar and WB images (**Figure 1**) showed intense accumulation of tracer in myocardium in comparison to bone uptake (ribs and sternum). Heart-to-contralateral (H/CL) ratio=1.72. Myocardial SPECT revealed tracer uptake in entire myocardium, with highest uptake in the apex, septum and inferior wall (**Figure 2**). The finding was highly suggestive for TTR, also confirmed by genetic testing. In other three patients, only mild accumulation of tracer in myocardium was found, similar or lower to bone uptake, with highest uptake in the apex (SPECT/CT images) and consecutive H/CL ratios 1.13, 1.13 and 1.23 (**Figures 3**, **4**, and **5**Figure 4Figure 5). The finding was suggestive for AL type. Multiple myeloma (IgG lambda type) was confirmed by bone marrow biopsy with phenotypisation. Endomyocardial biopsy was not performed in all pts. Figure 1. Transthyretin-related cardiac amyloidosis. The planar 99mTc-Pyrophosphate scintigraphy (whole body, anterior and left oblique images) showing diffusely intense myocardial activity, greater than in ribs and sternum; regions of interest drawn over the myocardium and the contralateral side show heart/contralateral uptake ratio of 1.72. Figure 2. Reconstruction of single photon emission CT images in short and long axes show uniform and intense myocardial uptake of 99mTc-Pyrophosphate, most prominent in septum and inferior wall. Figure 3. Light chain amyloidosis. 99mTc-Pyrophosphate scintigraphy (whole body and anterior) showing moderate uptake in myocardium, similar to bone; heart/contralateral uptake reveals 1.13; SPECT/CT coronal image show area of increased activity in apex. Figure 4. Light chain amyloidosis. Whole body and anterior bone 99mTc-Pyrophosphate scintigraphy showing mild uptake in myocardium; heart/contralateral uptake reveals 1.13; c) SPECT/CT coronal image show regional area of increased activity in apex. Figure 5. Light chain amyloidosis. Planar 99mTc-Pyrophosphate images show diffuse uptake in myocardium; heart/contralateral uptake reveals 1.20; SPECT/CT coronal image show area of increased activity in apex. **Conclusion**: According to recent recommendations, cardiac 99mTc-PYP scintigraphy using H/CL ratio quantitative score and SPECT/CT is a simple non-invasive imaging technique that can facilitate diagnosis of cardiac amyloidosis and differentiate AL from ATTR, thus avoiding invasive endomyocardial biopsy.
Sandra Jakšić, Jurinjak, Josip Vincelj, Jasmina Ćatić, Mario Udovičić, Mira Stipčević, Boris Starčević, Igor Rudež
**Background**: Assessment of left ventricular (LV) geometry and function represents the most frequent indication for an echocardiographic study in patient evaluation, management and indication for cardiac surgery. (1, 2) Three-dimensional transthoracic echocardiography (3DTTE) has been shown to be more accurate for quantification of LV volumes, compared to conventional two-dimensional (2D) echocardiography and real time 3DTTE is only imaging technique based on volumetric scanning able to show moving structures in the beating heart. (3) Magnetic resonance imaging (MRI) is currently considered the gold standard (2, 3) but is not widely available and feasible in some patients. Surgical ventricular reconstruction (SVR) is a treatment option in heart failure patients with left ventricular (LV) aneurysm. Endoventricular circular patch plasty (Dor procedure) has been proposed as a treatment for heart failure patients with an extensive myocardial infarction and LV aneurysm. (4) However, accurate patient selection should be performed to set the indication for SVR to determine LV size and shape and to avoid an excessive volume reduction and cavity deformation that can lead to progressive diastolic dysfunction and to a restrictive filling pattern. **Case report**: We present a patient with ischemic cardiomyopathy and LV aneurysm who underwent Dor procedure, and was preoperatively assessed by real time 3DTTE combined with contrast echocardiography to assess LV, mitral valve function and presence of LV thrombus (**Figure 1**). Decision point for aneurysmectomy is often finding of LV thrombus. Contrast echocardiography can be used to determine the presence of thrombus using left ventricle opacification method or detecting myocardial perfusion (**Figure 1**). SVR is often accompanied by mitral valve repair, therefore severity of mitral regurgitation should be carefully evaluated. (3) Significant shortcoming of 3DTTE is the electrocardiographic gating necessary to obtain full volume images and difficulty to acquire images in patients with atrial fibrillation which is often present in these patients. Figure 1. From the same pyramidal three-dimensional data set, the left ventricle can be visualized using different display modalities: multislice (multiple two-dimensional tomographic views extracted automatically from a single 3D data set) (A) with the addition of a contrast agent (B). Surface rendering display of left ventricle volume in the same patient (C). Volume rendering of mitral valve in the same patient (D). **Conclusion**: 3DTTE provides a comprehensive assessment in the management of heart failure patients with LV aneurysm which is crucial for their clinical management.
Ljupka Dimitrovska, Urška Dolores Breskvar Kač, Jana Ambrožič, Mojca Bervar
**Case report**: 66-year-old man with a history of mental disorder, presented with acute pulmonary edema and pneumonia. ECG indicated signs of previous anterior wall myocardial infarction. Based on patient history we concluded that it had occured approximately one month before he was admitted. Echocardiography identified mildly dilated left ventricle with moderately reduced ejection fraction with anterior wall akinesia (**Figure 1**). Apical akinetic segments were filled with thrombus. In accordance with the existing guidelines, we introduced three months of anticoagulation therapy with warfarin. Follow up echocardiography showed large apical pseudo aneurysm (probably in the inferoapical segment) partially filled with thrombus (**Figure 2**). With contrast echocardiography (SonoVue) we tried to identify the site of entry into the pseudoaneurysm. However, we could not confirm clear communication. The patient was offered immediate hospital admission for additional MRI diagnosis and operative treatment, but the patient refused all further diagnostic and surgical procedures. The patient is treated with heart failure drugs. Anticoagulation treatment was stopped due to the size of the pseudoaneurysm. Figure 1. Echocardiography at the time of first admission: thrombus in the left ventricle. Figure 2. Control echocardiography: pseudoaneurysm with thrombus. **Conclusion**: Cardiac pseudoaneurysm are rare but clinically significant lesions. (1-3) Although often challenging to diagnose, advances in non-invasive imaging (echocardiography, MRI, CT) have improved our ability to distinguish cardiac pseudoaneurysms from other pathologies. Most pseudoaneurysms, particularly if acute or associated with symptoms, require surgical repair or percutaneous exclusion to reduce the risk of rupture and potential for thromboembolism. In patients who have a high risk for surgical (or percutaneous) intervention, particularly when a pseudoaneurysm is chronic in nature, conservative management may be prudent. In our patient, a pseudoaneurysm was detected 6 months after myocardial infarction, which is not usual. We assume that the thrombus in the apex temporary closed the initial rupture of the left ventricle. Introduced anticoagulation therapy may have been an additional factor in the aneurysm development, because it probably contributed to thrombus dissolution.
Branimir Bukša, Ante Anić, Zorislav Šušak
The AmplatzerTM AmuletTM (St. Jude Medical) is a second generation AmplatzerTM Cardiac Plug device for left atrial appendage closure for stroke prevention in patients with atrial fibrillation. Second generation device features improved device stability, theoretically may reduce thrombus formation on atrial side and accommodates larger left atrial appendages. Transesophageal echocardiography in follow-up of patients implanted with left atrial appendage occluder AmplatzerTM AmuletTM is usually performed at 6 weeks’ post implantation. Imaging at that time point is performed to verify absence of left atrial or device-related thrombus, to assess the device position and to evaluate completeness of sealing (residual flow). Short-term follow-up are available in literature showing high success rate and excellent sealing (1-3) and in that context we present our uneventful 6 month follow up experience.
Petra Angebrandt, Dejan Došen, Vojtjeh Brida, Vlatka Rešković Lukši, Daniel Lovrić, Jadranka Šeparović Hanževački, Eduard Margetić
**Introduction**: Approximately three-fourths of patients with infective endocarditis have a preexisting structural cardiac abnormality at the time of the endocarditis development. Congenital heart lesions predisposing to infective endocarditis include aortic stenosis, bicuspid aortic valve, pulmonary stenosis, ventricular septal defect, patent ductus arteriosus, coarctation of the aorta, and tetralogy of Fallot. Complications of the infective endocarditis include cardiac, neurologic, renal, and musculoskeletal complications, as well as complications related to systemic infection. Cardiac complications are most common and they include: heart failure, perivalvular abscess, pericarditis, intracardiac fistula with myocardial perforation or aortic dissection. Management of patients with infective endocarditis includes antibiotic therapy as well as surgical treatment whenever it is indicated. (1-3) **Case report**: We present a case report of a case report of a 29-year-old patient with congenital bicuspid aortic valve. Prior to the development of endocarditis the patient underwent a dental procedure without antibiotic prophylaxis. After couple of days he was hospitalized due to clinically signs of sepsis and infective endocarditis was reveled on echocardiography. Patient was treated with antibiotics but due to uncontrolled sepsis and severe aortic stenosis in a bicuspid aortic valve, signs of heart failure developed and patient underwent urgent cardiac surgery where the reconstruction of aortic annulus with bovine pericardium and mechanical aortic valve replacement was done. After surgery despite appropriate antibiotic therapy patient had continuous fever and clinical and laboratory signs of sepsis, so we suspected that abscess was formed. Due to that we performed 3D transesophageal echocardiography which revealed infective endocarditis vegetations on mechanical aortic valve, (**Figure 1**) paravalvular abscess, ventricular septal defect with significant left to right shunt and instability of mechanical valve, by itself, due to big abscess hole. (**Figure 2**, **Figure 3**). After that second cardiac surgery was performed where replacement of mechanical aortic valve with homograft was done, together with surgical repair of ventricular septal defect. After surgery transesophageal echocardiography showed normal function of replaced aortic valve, with mild regurgitation, no signs of infective endocarditis vegetations and without ventricular septal defect. Figure 1. Long parasternal axis view of paravalvular abscess. Figure 2. Transesophageal short axis view of paravalvular abscess, instability of mechanical valve and ventricular septal defect. Figure 3. Color Doppler view of ventricular septal defect. **Conclusion**: Perivalvular abscess should be suspected in the setting of fever despite appropriate antimicrobial therapy. Transesophageal echocardiography is more sensitive for detection of myocardial abscess than transthoracic echocardiography.
Marko Boban
Cardiomyopathies are heterogeneous disorders of the heart muscle or function, which cause arrhythmias, sudden cardiac death or heart failure. Cardiac magnetic resonance imaging (CMR) recently became an important diagnostic tool for both diagnosing and clinical management of cardiomyopathies. By virtue of multiplanarity imaging, advanced temporal and spatial resolution, as well as functional assessment of systolic function, CMR offered important advancement to conventional imaging modalities. By virtue of tissue imagining, further knowledge was gained on pathophysiology of process, prognostic course of disease, and assessment of treatment applicability or efficiency. Controlled studies with prospective follow up reported on imperative position of CMR in management of cardiomyopathies, with reproducible and timely guided therapeutic interventions and well established relevance for improvement in the rates of major adverse outcomes. (1-4) The most important facts on clinical management of cardiomyopathies using CMR will be presented in brief.
Zorislav Šušak, Ante Anić, Branimir Bukša
Percutaneous left atrial appendage closure for stroke prevention in patients with atrial fibrillation has distinctively advanced in recent years (1). The current recommendations by the European Society of Cardiology include patients with high stroke risk and contraindications to long-term oral anticoagulation (2). Preprocedural transesophageal echocardiography remains the reference technique to exclude the presence of left atrial appendage thrombi prior to left atrial appendage closure and to assess left atrial appendage anatomy and accurate measurements regarding challenging anatomies and device sizing (3). Procedural transesophageal guidance is generally recommended with any current CE mark device in addition to fluoroscopy. Transseptal puncture is well positioned with transesophageal bicaval and short axis views. Device sizing depends on the widest landing zone on fluoroscopy or transesophageal echocardiography which is by standard recommendation upsized by 2-5 mm. Device is adequately positioned at landing zone and deployment is controlled by transesophageal echocardiography and fluoroscopy. Prior to device release, evaluation of proper deployment is done by transesophageal echocardiography. Finally, after deployment additional assessment of possible residual flow is performed. Procedural transesophageal guidance is widely available imaging method which is contributing to procedural success and safety. We would like with our patients series to depict key procedural steps.
Damir Raljević, Davorka Žagar, Vesna Pehar Pejčinović, Viktor Peršić, Dijana Travica Samsa, Ivo Kalčić, Ingrid Buljanović
Atrial fibrillation is an important factor associated with left atrium (LA) enlargement and thrombus formation in the LA appendage. Transesophageal echocardiography is the procedure of choice for assessment of dimension of the LA, as well as thrombus detection in the LA cavity or LA appendage. It can detect thrombi with a high degree of sensitivity and specificity varying from 93% to 100%. Cardiac CT represents a gold standard for the assessment of anatomy of the LA complex. The mean CT sensitivity for identifying thrombus in the LA was 81% and the mean specificity 90%. Because of the high spatial resolution and the excellent myocardial border detection magnetic resonance imaging (MRI) is considered the most accurate technique for the non-invasive assessment of atrial volumes. Cardiac magnetic resonance imaging helps in tissue characterization of various intra-atrial masses and thus differentiation between cardiac tumors and thrombi. Both CMR and cardiac CT currently represent very important imaging modalities used for the comprehensive evaluation of the LA. (1-3) In our work we show a small series of patients with suspected mass in the LA appendage on the transesophageal ultrasound in which a cardiac MSCT and/or cardiac MRI is performed.
Matias Trbušić
**Background**: Pneumopericardium is a rare condition defined as a collection of air in the pericardial cavity. It is usually result of chest injuries, iatrogenic causes (bone marrow puncture, thoracic surgery, pericardiocentesis, endoscopic procedures), and infective agents causing purulent gas - producing pericarditis. (1, 2) **Case report**: We represent a case of 82-year old female patient with spontaneous pneumopericardium caused by malignant ulcer that created fistula between the pericardium and colon. She was admitted in very severe condition with chest pain, severe respiratory distress, hypotension, distended neck veins and tachyarrhythmia. High blood leukocyte, high C reactive protein levels and combined respiratory and metabolic acidosis were present. Electrocardiogram showed atrial fibrillation and diffuse micro voltage. Chest X ray and CT showed normal sized heart surrounded by air (halo sign) below the aortic arch, and large left pleural effusion. CT also revealed neoplastic process of the transverse colon infiltrating stomach and diaphragm. Due to intrapericardial spontaneous contrast echoes the image quality of 2-D echocardiography was poor making difficult to verify signs typical for tamponade such as early diastolic right ventricular collapse and early diastolic right atrial collapse (**Figure 1**). Nevertheless, significant respiratory variations (>25%) in transmitral and transtricuspid blood flow velocities have been noticed by Pulsed Doppler echocardiography. Also, despite the air within the pericardium, inferior vena cava was well visualized showing dilatation and lack of significant inspiratory collapse. It was concluded that the patient has tension pneumopericardium and resulting cardiac tamponade caused probably by a valve mechanism. (3) Under fluoroscopy control emergent pericardiocentesis was performed with needle extraction of 160 mL of very unpleasantly smelling air. Mechanical ventilation was also started together with antibiotic therapy, volume replacement and pleural drainage. The patient was stabilized and next day transferred to another clinic in overall stable condition were the complex abdominal surgery was performed. In conclusion, spontaneous pneumopericardium is a rare life threatening condition that can be caused by infiltrating malignant process. It can be rapidly recognized by physical examination, X ray, CT and fluoroscopy. Despite the low 2D image quality due to air bubbles, echocardiography, especially Pulsed Doppler and inferior vena cava visualization, an undeniable role in hemodynamic assessment and verification of tamponade. Figure 1. A) dilated neck veins, B) Electrocardiogram: frontal end precordial micro-voltage, C) X ray: halo sign around the heart, D) CT scan: halo sign around the heart (full arrow), infiltrating colon carcinoma (empty arrow), E) CT scan: possible place of communication between the colon and the pericardium, F) Echocardiography - Pulsed Doppler of transmitral flow: > 25% respiratory variations.
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 transient ischemic attack (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 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 the Neurology Department where she was treated for stroke. Her brain magnetic resonance imaging 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**). Patient received percutaneous surgical closure. Figure 1. Transthoracic echocardiography: patent foramen ovale with right-to-left shunt. 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, (**Figure 2**) a septal defect, 14mm in diameter with left-to-right shunt (**Figure 3**). The patient is on the waiting list for surgical treatment. Figure 2. Transthoracic echocardiography: atrial septal aneurysm. Figure 3. Transthoracic echocardiography: 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.
Sandra Jakšić Jurinjak, Josip Vincelj, Mateja Sabol Pušić, Mario Sičaja, Boris Starčević
**Introduction**: Patent foramen ovale (PFO) is a defect of the interatrial septum usually with no clinical repercussions, incidence of about 25%, but can also be associated with various clinical conditions such as cryptogenic stroke, migraine, platypnea-orthodeoxia syndrome, and decompression illness (1). Recent studies have demonstrated that transcatheter closure of such defects is a safe procedure with long term efficacy in preventing paradoxical embolism. Transesophageal echocardiography (TEE) is pivotal in diagnosing PFO with precise characterization of septal abnormalities, selection of patients suited for transcatheter closure, but also for guidance during the intervention, assessment of intervention and as appropriate method for follow up, especially in case of a residual shunt after the intervention (2). **Case report**: We describe the significance and role of two and three dimensional (2D and 3D) transesophageal echocardiography performed in series of five patients diagnosed with cryptogenic stroke and concomitant PFO who underwent transcatheter closure. TEE was performed during and after transcatheter closure (**Figure 1**), both PFO diameter and morphology were assessed by TEE before transcatheter closure. Right-to-left interatrial shunting was assessed by contrast TEE facilitated with Valsalva’s maneuver. During transcatheter closure, TEE was used for guidance and positioning of the Amplatzer occluder. Immediate complete closure was documented by color Doppler TEE. All patients received antibiotic prophylaxis 1 hour prior to the procedure. After post-interventional transthoracic echocardiography with early success of closure tested by a venous injection of right heart echo contrast agent patients were discharged. Figure 1. A) Transesophageal image of simple PFO; B) Transcatheter closure – transesophageal echocardiography was used for guidance and positioning of the Amplatzer occluder (multiplane view); C) 3D transesophageal image of occluder positioned in interatrial septum. **Conclusion:** Some studies showed that the success of the procedure – transcatheter closure, with the goal being complete occlusion without any residual shunt, is more dependent on the anatomy of the atrial septum than the type of the device used for the procedure (3). Therefore, also in our opinion, 2D and 3D echocardiographic assessment, initially in patient selection and afterwards during the intervention, is crucial for the successful treatment.
Urška Dolores Breskvar Kač, Ljupka Dimitrovska, Jana Ambrožič, Mojca Bervar
**Introduction:** Hypereosinophilic syndromes (HES) are a group of multisystemic disorders marked by a sustained overproduction of eosinophils, in which eosinophilic infiltration and mediator release cause damage to multiple organs. (1) Cardiac involvement is present in 40% of cases and is one of the most serious manifestations of HES, accounting for approximately one-half of deaths. (2) We report a case of Loeffler endocarditis detected by echocardiography. **Case report:** 68-year-old male presented with symptoms of behavioural changes. The patient was diagnosed with rheumatic polimyalgia two years ago and treated with low-dose prednisolon. Development of eosinophilic granulomatosis with polyangiitis (EGPA) has been suspected. Magnetic resonance imaging of the brain revealed multiple ischemic lesions. Two-dimensional transthoracic echocardiography (TTE) showed a normally sized left ventricle with normal systolic function without regional wall motion abnormalities and no signs of diastolic disfunction. The valves showed no significant pathology. An echodense structure that obliterated the left ventricular apex was detected, consistent with thrombus (**Figure 1**). The formation was further investigated with administration of contrast (Sonovue) and three-dimensional TTE and transesophageal echocardiography. Loeffler endocarditis with apical thrombus was suspected with distant embolization to the central nervous system. High-dose prednisolone therapy in combination with pulsed cyclophosphamide regimen was started and anticoagulation therapy with warfarin was instituted. The symptoms began to resolve and the patient reports no further manifestations of the disease. Serial monthly follow-up TTE exams demonstrate apical thrombus regression and no further cardiac involvement so far. Figure 1. Transthoracic echocardiography. Apical four chamber view. A homogenous echodense mass in the left ventricular apex **Discussion and Conclusion:** Eosinophil-mediated heart damage evolves through three stages. The early stage of cardiac involvement begins with eosinophilic infiltration, followed by an intermediate thrombotic stage, and, finally, a late fibrotic stage, resulting in endomyocardial fibrosis, leading to restrictive cardiomyopathy. (2) The major complication of intracardiac thrombus formation is the detachment and distal embolization of thrombotic material, leading to embolic events, which are a major cause of morbidity among patients with HES. (3, 4)
Denis Došen, Željko Baričević, Kristina Marić-Bešić, Darko Anić, Maja Strozzi
**Introduction**: Atrioventricular (AV) canal defects are a group of congenital cardiac defects involving the AV septum and AV valves (ie. mitral and tricuspid valves). Combinations of these anatomic abnormalities result in complete (both atrial and ventricular septal defects [ASD/VSD] and partial (only ASD) forms that are manifested in varying clinical presentations. (1-4) **Case 1**: 35-year-old patient with Down syndrome and untreated complete AV canal defect who developed Eisenmenger syndrome and has been treated with bosentan (**Figure 1**). Figure 1. 4 chamber view – arrows pointing towards primum atrial septal defect, inlet ventricular septal defects and a common atrioventricular valve. Complete AV canal defect is a result of complete failure of fusion between the superior and inferior endocardial cushions. It is characterized by a primum ASD that is contiguous with a posterior (or inlet) VSD and a common AV valve. **Case 2**: 61-year-old patient with partial AV canal defect who undergone surgical correction 2 years ago and is now in NYHA I class status (**Figure 2**). Figure 2. 4 chamber view – arrows pointing towards primum atrial septal defect and single atrioventricular valve/two separate valve orifices. Partial AV canal defect is due to incomplete fusion of the superior and inferior endocardial cushions and consists of a primum ASD and a single AV valve annulus with two separate valve orifices. Due to abnormal fusion of the left tubercle of the superior and inferior cushions, the anterior leaflet of the mitral valve typically is cleft. **Case 3**: 26-year-old patient with transitional AV canal defect. Due to 1.5:1 left to right shunt without signs of significant right ventricle volume overload, she will undergo further evaluation in 6 months (**Figure 3**). Figure 3. Cardiac magnetic resonance imaging showing interatrial aneurysm with primum atrial septal defect (big arrow) and large ventricular septal defects partially obstructed with septal tricuspid valve leaflet (narrow arrow). Transitional AV canal defect is anatomically a subtype of complete AV canal defect as it consists of a large primum defect, cleft mitral valve and inlet VSD. However, dense chordal attachments to the ventricular septum lead to small insignificant ventricular shunting and delineation of distinct left and right AV valve orifices, resulting in a defect that is similar to the physiology of a partial AV canal defect. **Case 4**: 48-year-old patient undergone surgical correction 2 months ago due to development of bidirectional shunt (dominantly left to right with Qp/Qs 2.1:1) (**Figure 4**). Figure 4. 4 chamber view shows large primum atrial defect and inlet ventricular septal defects. Intermediate AV canal defect is a rare subtype of complete AV canal defect in which a bridging tongue of tissue divides the common AV valve into two distinct orifices. This defect is similar to a complete AV canal defect and usually has both large primum atrial and inlet ventricular septal defects. Due to the natural division of the common AV valve into left and right AV valve components by the tongue of tissue, surgical division is not required. **Conclusion**: Recent advances in echocardiography make it the first-line modality to diagnose AV canal defects; 3D transesophageal echocardiography is often used to determine additional anatomical and functional factors that impact surgical management.
Ljupka Dimitrovska, Jure Dolenc, Matija Jelenc, Zvezdana Dolenc-Stražar, Tomislav Klokočovnik
**Introduction**: The purpose of this paper is to present the case of patient with a primary cardiac tumor in the left atrium (1), which was suspected to be sarcoma. **Case report**: 51-year-old female was transferred from peripheral hospital for further diagnosis due to a suspected tumor in left atrium. She sought medical attention after experiencing shortness of breath, fatigue, fainting under light exertion. Echocardiography was performed, which showed a left atrial mass measuring 4x3x2cm, growing from the inferolateral wall (**Figure 1**). The mass was occupying more than half the left atrium and was pushing or adherent to the posterior leaflet of mitral valve, therefore causing mild to moderate functional mitral stenosis. Depending solely on the location of the tumor it was suspected that this is probably myxoma. MRI confirmed the echo findings (**Figure 2A**), probabbly a sessile myxoma. However, MRI could not confirm nor execlude sarcoma. PET CT showed a hypermetabolic left atrial mass without any distant metastasis (**Figure 2B**). Biopsy of tumor mass was not performed because of the high risk for embolic complications. In view of the extent of the mass and the limited exposure, we proceeded to perform an auto transplantation – heart explanation with ex vivo resection and reconstruction of the left atrium with CorMatrix patch. The patient returned to the operating room on the second postoperative day due to cardiac tamponade and required the insertion of a permanent pacemaker for complete heart block prior to her discharge 3 weeks following surgery. The histology revealed a spindle cell tumor consistent with a malignant intimal sarcoma. No further adjuvant therapy was given. Ten months following surgery, she remains symptom free with no evidence of any cardiac masses on echocardiography or distant metastases on CT scan. Figure 1. Echocardiography demonstrating a left atrial mass that was occupying left atrium and was pushing or adherent to the posterior leaflet of mitral valve. Figure 2. Cardiac magnetic resonance (A) and PET-CT (B), demonstrating the mass in the left atrium. PET CT did not show any signs of metastasis in the rest of the body.
Kristina Selthofer-Relatić
Stroke is a leading cause of morbidity and mortality. According to pathophysiologic mechanisms, stroke can be divided into two major groups: ischemic (85%) and hemorrhagic (15%). 20% of ischemic strokes are caused by cardiac embolisms. Brain imaging (CT, MR) confirms the diagnosis, some specific characteristics seen during the imaging of the brain may suggest a distinct cardiac cause. Clinical history, examination and electrocardiogram can shed light on a potential cardiac cause of ischemic stroke. Echocardiography is a key method in the evaluation, diagnosis and management of embolic stroke (transthoracic echo, transesophageal echo, 3D echo, contrast echo). The etiological factors of cerebrovascular events are: blood clots, tumor fragments, infected and noninfected vegetations, calcified particles, atherosclerotic debris. Cardiac sources of emboli can be divided according to embolic potential into a) **high risk**: intracardiac thrombi (atrial arrhythmias - atrial fibrillation, ischemic heart disease, non-ischemic cardiomyopathies, prosthetic valves and devices), intracardiac vegetations, intracardiac tumors, aortic atheroma; b) **low risk**: spontaneous echo contrast without clot, left ventricle aneurysm without clot, intracardiac calcifications, valvular anomalies, septal defects and anomalies. The indications for echocardiography in patients with neurological events are: abrupt occlusion of a major peripheral or visceral artery in patients of any age; younger patients with CV events (45 years) with neurologic events without evidence of cerebrovascular disease or other obvious cause, patients for whom a clinical therapeutic decision will depend on the results of echocardiography.
Josip Vincelj, Sandra Jakšić Jurinjak, Mateja Sabol Pušić
Contrast echocardiography is a noninvasive technique for the assessment of myocardial function and perfusion which improves estimation of endocardial border delineation through ventricular cavity opacification. The first report of contrast echocardiography dates from 1968, when Gramiak and Shah reported their observation of „clouds of bubbles” in the aortic root following injections of saline through an intra-aortic catheter (1). The first contrast agents used were indocyanine green, agitated saline solution, sonicated solutions of dextrose and patient’s blood, while today we are using second generation contrast agents: perflutren, perfluoropentane, perfluoropropane, perfluorooctylbromide and sulphur hexafluoride. Contrast echocardiography is used for the quantification of heart cavity dimensions, volumes, ejection fraction (EF), assessment of the regional myocardial contractility, detection of blood shunts between cavities and detection of intracardiac masses (2). It is indicated in the case of suboptimal endocardiac borders display in two or more segments of left ventricle, using the conventional methods. Using the contrast agents with perflutren (OptisonTM) the endocardial border is visible in more than 93% of the patients, opacification of the whole left ventricle is achieved in 87% of the patients. Nowadays, contrast echocardiography has multiple possibilities, from global and regional left ventricle function assessment, to left ventricle shape visualization (**Figure 1**), detection of intracardiac masses, estimation of myocardial perfusion with differentiation of viable myocardium from stunned and hibernating myocardium, when associated with stress echocardiography (3). Figure 1. Apical four-chamber view showing aneurysm of the left ventricle (A). Left ventricular tumor after administration of the contrast agent OptisonTM (B). Many noninvasive diagnostic laboratories are using contrast echocardiography in diagnosing 10-15% of patients. In conventional echocardiography we can sometimes have difficulties deciding whether some intracardiac mass represents a tumor or thrombus (**Figure 1**). During contrast echocardiography, if the mass is without opacification then it represents thrombus because it doesn’t take up contrast. But if the mass does opacify through its vascularization, then it is a tumor. Contrast echocardiography can also have a key role during percutaneous ablation of hypertrophic cardiomyopathy in order to choose the target septal branch for IVS thickness reduction and decrease of LV outflow tract gradient to accomplish an optimal clinical and hemodynamic result after the intervention (4). After many years of investigations, noninvasive assessment of heart cavities and left ventricle myocardial perfusion has become the clinical reality using the contrast echocardiography.
Ivo Planinc, Daniel Lovrić, Dora Fabijanović, Boško Skorić, Hrvoje Jurin, Jure Samardžić, Nina Jakuš, Jana Ljubas Maček, Maja Čikes, Davor Miličić
**Introduction**: Non-thrombotic pulmonary embolism (PE) is a rare cause of respiratory insufficiency (RI), andit may be caused by embolization of different cell types, foreign material or gas. (1) **Case report**: 63-year old female who was surgically treated for left ocular intra-bulbar melanoma, and had silicon-oil instillation in the same eye had an episode of unexplained acute RI in November 2016 for which she was treated in a local hospital. In January 2017 she presented to emergency department of our hospital with eye pain associated with nausea and vomiting. Acute glaucoma was diagnosed, and she was treated with acetazolamide and mannitol. Approximately 2 hours later she became severely dyspnoeic and hypoxemic, and showed left sided hemiparesis. Due to progressive RI she was sedated, intubated, and mechanically ventilated. Bedside echocardiography revealed normal systolic function of normal sized LV, large amount of hyper-echogenic microbubbles in LV, and RV dilatation with signs of increased RV afterload with sPAP estimated to 50mmHg. Thrombotic massive/sub-massive PE, ACS or other possible etiological factors were excluded. Initially, head CT did not show signs of cerebrovascular ischemia. In spite of maximum respiratory support delivered by mechanical ventilation (IPPV FiO2 100%, RR 25/min) ABG analysis revealed further progression of respiratory acidosis (pH 7.1, pCO2 20kPa, pO2 7kPa), and therefore decision was made to implant veno-arterial ECMO support. Before starting ECMO circuit the patient had to be resuscitated due to an episode of pulseless electrical activity. During the first 24h after ECMO circuit initiation, echocardiography could still show persistence of hyper-echogenic LV microbubbles. During the first hospital day, physical exam also revealed abdominal and thoracic petechial rash. 3 days later the patient was successfully weaned off both ECMO support and mechanical ventilation. Follow-up head CT showed ischemic lesion in right frontal lobe. After prolonged hospital stay, the patient fully recovered, regained full motoric function of her left extremities, and was transferred to Rehabilitation Clinic. **Conclusion**: Differential diagnosis of LV microbubbles with a triad of pulmonary distress, petechial rash and neurological symptoms includes inadvertent air insufflation during infusion handling, fat embolism, or fat-like embolism syndrome which could have been induced by silicon-oil derived from the eye.
Rina Dalmatin, Luka Bastiančić, Teodora Zaninović Jurjević, Davorka Žagar, Luka Zaputović, Alen Ružić
**Introduction:** Cardiac tumors are extremely rare with the incidence <0.1%, found in approximately 7 of 12.000 autopsies. Papillary fibroelastomas (PFs) are the second most common benign cardiac tumors, peduncular and mobile, typically speckled and echolucent. The mean patient age at detection is 60 years. According to the literature (1-3), PFs are most commonly seen at the aortic (36%) and mitral (29%) valve, while they can be found on the tricuspid valve in 11% cases or at the pulmonic valve in 7% patients. These benign cardiac tumors usually (70%) have symptoms and they are mostly due to embolization, either of tumor itself or the tumor associated thrombus. **Methods and Results:** From 2002 till today, at the Cardiovascular Department at University Hospital Centre Rijeka, we found 9 cases of PF that have been patohystologically proven. The PFs were most often find at the aortic valve (4 cases), in 2 cases they were situated at the pulmonary valve and the same was the number of PFs found at the mitral valve, while only 1 were found at the tricuspid valve. Only 2 cases were completely asymptomatic, while 7 had symptoms probably related to PF: 4 of them had cerebral embolic symptoms and in 3 cases the coronary embolism was suspected. In only one case the tumor had hemodynamic implications by reducing the effective mitral valve area. In our series, 6 patients with PF were sent to cardiac surgery, and in 3 cases the observation was indicated. **Conclusion:** PFs are rare benign cardiac tumors with a high embolic potential. The surgery is reasonable therapeutic option for all patients at risk of embolization or in those with other indications for heart surgery. Nowadays, the PF of the left heart should be considered for operation, while those at the pulmonary and tricuspid valve are removed only in case of surgical revascularization, aortic aneurysm correction or other heart valve disease that is hemodinamically significant. The global PF registry is needed for better understanding of PFs.
Zvonimir Ostojić, Vlatka Rešković Lukšić, Jadranka Šeparović Hanževački
**Introduction**: Compared to rheumatic mitral stenosis (RMS), degenerative mitral stenosis (DMS) is usually described as less sever. For the severity assessment echocardiographic Doppler parameters are often used, despite the well known possibility of DMS severity underestimation due to the influence myocardial diastolic function and concomitant valve patohlogy. (1, 2) The aim of this study was to compare severity of DMS compared to RMS and to determine whether Doppler parameters underestimate severity of DMS compared to other echocardiographic measurements. **Methods**: We preformed a single center, retrospective, observational study including all patients who were diagnosed with mitral stenosis in past three years. Data were reanalyzed using fully digital echocardiographic database and hospital charts. Mitral stenosis (MS) was defined as turbulent flow with mean diastolic gradient >2 mmHg across mitral valve (MV) in conjunction with morphologic changes. In patients who had 3D examinations made, mitral valve area (MVA) was calculated according to pressure half time (PHT), continuity equation, 3D transthoracic echocardiography, and 3D transoesophageal echocardiography. **Results**: Patients with DMS (N=38, female N=23; 60.5%) compared to patients with RMS were significantly older (80±9.4 vs. 67±7.1; p1** | 1.38±0.35 | 1.14±0.25 | 2** | 4.93±0.68 | 5.06±0.59 | 0.36 | | **LVPWd (cm)3** | 1.22±0.30 | 1.02±0.21 | 4** | 2.45 | 1.46 | 5** | 1.92±0.76 | 1.55±0.38 | 0.02 | | **MVA VTI6** | 1.26 | 1.26 | 0.07 | | **LAA7** | 34.24±7.6 | 42.77±17.23 | 0.004 | | **MI ≥28** | 11 (29%) | 20 (42%) | 0.19 | | **AS ≥29** | 30 (79%) | 15 (32%) | 10** | 5 (13%) | 10 (21%) | 0.32 | | **TI ≥211** | 8 (21%) | 16 (34%) | 0.18 | | **PH12** | 48±18 | 55±22 | 0.2 | | **TAPSE13** | 17.8±4.8 | 17.7±5.3 | 0.9 | [†] 1Intraventricular septal width in diastole; 2Left ventricle internal dimension in diastole; 3Left ventricle posterior wall width in diastole; 4Mitral annulus calcification: 1- annular calcification; 2- calcification extend 1/2 of mitral cusp; 5Mitral valve area calculated using pressure half time; 6Mitral valve area calculated using velocity time integral; 7Left atrium area; 8Mitral insufficiency at least moderate in severity; 9Aortic stenosis at least moderate in severity; 10Aortic insufficiency at least moderate in severity; 11Tricuspid insufficiency at least moderate in severity; 12Systolic pressure in pulmonary artery; 13Tricuspid annular plane systolic excursion. ### Table 2: Comparison of calculated mitral valve area with different echocardiographic methods. | **MVA** (1) **calculation** | PHT (2) 1.83±0.64 — VTI3 1.46±0.49 | PHT 1.83±0.64 — 3D TTE4 1.32±0.45 | PHT 1.83±0.64 — 3D TOE5 1.20±0.42 | VTI 1.46±0.49 — 3D TTE 1.32±0.45 | VTI 1.46±0.49 — 3D TOE 1.20±0.42 | 3D TTE 1.32±0.45 — 3D TOE 1.20±0.42 | | --- | --- | --- | --- | --- | --- | --- | | **p value** | p=0.019 | p=0.013 | p<0.001 | p=0.557 | p=0.074 | p=0.491 | [†] 1Mitral valve area; 2Pressure half time; 3Velocity time integral; 43 dimensional transthoracic echocardiography; 53 dimensional transesophageal echocardiography **Conclusion**: As expected, patients with DMS are older, with higher rate of comorbidities and higher prevalence of significant AS. On the other hand, patients with RMS had larger LAA and more often AF. Our results indicate that Doppler assessment of MS, using PHT, significantly underestimates severity of MS in DMS patients when compared with other methods, especially 3D planimetry. Furthermore, the same observations are described in our group of patients with RMS. In our opinion, cause of misleading PHT measurement might be of different cause between groups, but this requires further studies.
Marija Brestovac, Vlatka Rešković Lukšić, Blanka Glavaš Konja, Joško Bulum, Martina Lovrić Benčić, Marko Jakopović, Jadranka Šeparović Hanževački
**Introduction**: Pulmonary embolism is frequently seen in malignant diseases and requires anticoagulant treatment (1). The decision on fibrinolytic therapy is based mainly on hemodynamic state of the patient. **Case report**: 56-year-old female patient was admitted to our department because of severe dyspnea and chest pain. She was a heavy smoker and few years ago she underwent chemotherapy and surgical treatment for colorectal carcinoma, maintaining complete remission. Five months’ prior admission she started complaining of dyspnea, dry cough, pain in the left hemithorax, inappetence and significant weight loss. Among other diagnostic tests, pulmonary CT angiography was performed and massive pulmonary embolism was diagnosed. At the time of admission, the patient was hemodynamically stable and D-dimer values were only slightly elevated (2 mg/l). According to patient history, it was more likely that she had had chronic pulmonary embolism, so we decided to start therapy with low molecular weight heparin and warfarin, rather than fibrinolytic therapy. Echocardiography findings revealed severe right heart pressure overload with mild cavity dilatation, preserved systolic function, mild to moderate tricuspid regurgitation and a massive thrombus in truncus of the pulmonary artery (PA) with signs of pulmonary valve (PV) obstruction (**Figure 1**). There were no signs of deep vein thrombosis nor relapse of colorectal carcinoma. After adequate anticoagulation, 11 days later, echocardiography revealed progression of the obstructive mass in the PA trunc. However, the patient was still hemodynamically stable and eupneic. Even though a fibrinolytic therapy was considered again, we decided against that treatment option. A CT pulmonary angiography was repeated and showed signs of pulmonary artery angiosarcoma: an endoluminal mass infiltrating pulmonary valve up to the pulmonary artery bifurcation, lobar and segmental branches and with suspected pulmonary infarction without mediastinal lymphadeopathy (**Figure 2**). The patient was transferred to department for lung diseases. PET/CT body scans showed metabolic activity in the pulmonary artery mass and a lymph node above the pulmonary artery while cytological findings taken during secondary carina punction revealed mesenchymal malignant neoplasia. A MR scan of the heart and pulmonary MR angiography described a partially necrotic mass attached to pulmonary valve without myocardial infiltration (**Figure 3**). Considering the complexity of the case, a definite treatment decision is going to be made by a medical council including cardiologists, pulmonologists and cardiac surgeons (2, 3). Figure 1. Echocardiography: proliferative mass of pulmonary artery truncus. Figure 2. Computerized tomography scan: proliferative mass of pulmonary artery truncus. Figure 3. Magnetic resonance angiography: proliferative mass of pulmonary artery truncus. **Conclusion**: Although pulmonary embolism may accompany malignant diseases, other etiologies of the infiltration of the pulmonary artery should also be taken into account, especially when considering fibrinolytic therapy. Regular echo follow-up is helpful in visualizing the thrombus regression under anticoagulant therapy as well as setting indications for further diagnostic and treatment options.