Multimodality Imaging of Cardiac Myxomas.

Cardiac myxomas are the most common benign cardiac neoplasms. Echocardiography is the first-line imaging modality used to analyze cardiac masses, allowing the detection of tumor location, size, and mobility. However, additional imaging techniques are required to confirm the diagnosis, evaluate tissue characteristics of the mass, and assess potential invasion of surrounding structures. Second-line imaging includes cardiac magnetic resonance imaging (MRI) and/or computed tomography (CT) depending on availability and the patient's characteristics and preferences. The advantages of CT include its wide availability and fast scanning, which allows good image quality even in patients who have difficulty cooperating. MRI has excellent soft-tissue resolution and is the gold standard technique for noninvasive tissue characterization. In some cases, evaluation of the tumor metabolism using 18F-fluorodeoxyglucose positron emission tomography with CT may be useful, mainly if the differential diagnosis includes primary or metastatic cardiac malignancies. A cardiac myxoma can be identified by its characteristic location within the atria, typically in the left atrium attached to the interatrial septum. The main differential diagnoses include physiological structures in the atria like crista terminalis in the right atrium and the coumadin ridge in the left atrium, intracardiac thrombi, as well as other benign and malignant cardiac tumors. In this review paper, we describe the characteristics of cardiac myxomas identified using multimodality imaging and provide tips on how to differentiate myxomas from other cardiac masses.

Urine high-sensitive troponin I in children cannot offer an applicable alternative to serum.

Introduction: In children, congenital heart defects represent the primary cause of increased serum troponin I. The elimination process of cardiac troponin I from the bloodstream and the factors influencing this process remain unknown. The objective of this study was to explore the role of troponin I as an indicator of cardiac damage in children both in serum and urine, a concept previously investigated in adults. Methods: Our prospective study involved 70 children under 24 months of age. The first group underwent ventricular septal defect repair, while the second group involved children who had undergone partial cavopulmonary anastomosis. For these groups, urine and serum troponin I were assessed on four occasions. The third group, consisting of healthy children, underwent a single measurement of urine troponin I. Results: Serum troponin I values exhibited an expected elevation in the early postoperative period, followed by a return to lower levels. Significantly higher concentrations of serum troponin I were observed in the first group of children (p < 0.05). A positive correlation was found between troponin I in the first three measurements and cardiopulmonary bypass and aortic cross-clamping time. There was no discernible increase in urine troponin I directly related to myocardial damage; troponin I couldn't be detected in most urine samples. Discussion: The inability to detect troponin I in urine remains unexplained. Potential explanatory factors may include the isoelectric point of troponin I, elevated urinary concentrations of salts and urea, variations in urine acidity (different pH levels), and a relatively low protein concentration in urine.

Common benign primary pediatric cardiac tumors: a primer for radiologists.

Cardiac tumors are neoplasms arising from or located in the heart or the pericardium. Although rare, primary cardiac tumors in children require an accurate and timely diagnosis. Most pediatric primary cardiac tumors are benign (around 90%). Echocardiography is the first imaging modality used due to its availability, noninvasiveness, inexpensiveness, and absence of ionizing radiation. Computed tomography (CT) and magnetic resonance imaging (MRI) offer better soft tissue visualization as well as better visualization of extracardiac structures. A great advantage of MRI is the possibility of measuring cardiac function and blood flow, which can be important for obstructing cardiac tumors. In this article, we will offer a brief review of clinical, echocardiographic, CT, and MRI features of cardiac rhabdomyomas, fibromas, teratomas, and lipomas providing their differential diagnosis.

Urine High-Sensitive Troponin T-Novel Biomarker of Myocardial Damage in Children.

Background: The use of high-sensitive cardiac troponin T (hsTnT) in urine as a marker of cardiac damage in children has not yet been reported. Elimination of cardiac troponins is dependent on renal function; persistently increased serum hsTnT concentrations were observed among individuals with impaired renal function. The aim of this study was to investigate serum and urine hsTnT levels and its correlation in infants and children younger than 24 months of age after cardiac surgery. Methods: This study was conducted on 90 infants and children under 24 months of age who were divided into three groups. The experimental group consisted of patients with intracardiac surgery of ventricular septal defect (VSD), first control group consisted of infants with extracardiac formation of bidirectional cavopulmonary connection (BCPC), and the second control group consisted of healthy children. Troponin T values ​​were determined in serum and urine at five time points: the first sample was taken on the day before cardiac surgery (measure 0) and the other four samples were taken after the surgery; immediately after (measure 1), on the first (measure 2), third (measure 3), and fifth postoperative day (measure 5). The first morning urine was sampled for determining the troponin T in the control group of healthy infants. Results: A positive correlation between troponin T values in serum and urine was found. Urine hsTnT measured preoperatively in children undergoing BCPC surgery was higher (median 7.3 [IQR 6.6-13.3] ng/L) compared to children undergoing VSD surgery (median 6.5 [IQR 4.4-8.9] ng/L) as well as to healthy population (median 5.5 [IQR 5.1-6.7] ng/L). After logarithmic transformation, there was no statistically significant difference in urine hsTnT concentration between the groups at any point of measurement preoperatively or postoperatively. Statistically significant negative correlation was found between serum and urine hsTnT concentrations and glomerular filtration rate estimated by creatinine clearance. Patients who underwent surgical repair of VSD had significantly higher concentrations of troponin T in serum on the first three postoperative measurements compared to those who had BCPC surgery. Conclusions: According to the results of this study, renal function after cardiac surgery appears to have a major effect on the urinary hsTnT concentrations, and we cannot conclude that this is an appropriate marker for the assessment of postoperative myocardial damage in children. Nevertheless, more research is needed to reach a better understanding of the final elimination of cardiac troponins in children.

Influence of Shunt Type on Survival and Right Heart Function after the Norwood Procedure for Aortic Atresia.

The study objective was to compare the results after Norwood procedure between modified Blalock-Taussig shunt (MBTS) and right ventricle-to-pulmonary artery conduit (RVPAC) according to Sano in patients with hypoplastic left heart syndrome (HLHS) and aortic atresia (AA). A total of 146 neonates with HLHS and AA who underwent the Norwood procedure at our institution between 2001 and 2020 were divided into 2 groups according to shunt type (MBTS or RVPAC). Survival after the Norwood procedure was compared between the groups. Longitudinal right ventricular and tricuspid valve function in each group were evaluated using cubic splines method. RVPAC was performed in 103 patients and MBTS in 43 according to surgeon preference. There were no differences in the 30-day mortality rates (16.5% vs 16.3%, P  = 0.973). Survival at 0.5, 1 and 3 years was 79.6%, 74.6%, and 68.9% in RVPAC and 66.8%, 64.3%, and 58.5% in MBTS (P  =  0.293). Among 23 patients undergoing tricuspid valve procedure, different mechanisms of tricuspid regurgitation were observed between the groups. Longitudinal analysis revealed greater prevalence of late right ventricular dysfunction in RVPAC patients. In 77 patients who completed Fontan procedure, the postoperative N-terminal pro B-type natriuretic peptide value was significantly higher in RVPAC vs MBTS (554 vs 276 ng/L, P  =  0.007). No survival advantage of RVPAC over MBTS was observed in neonates with HLHS and AA undergoing the Norwood procedure. Longitudinal analysis demonstrated a greater prevalence of right ventricular dysfunction and higher N-terminal pro B-type natriuretic peptide values during late follow-up in patients with RVPAC.

ATP synthase deficiency due to m.8528T>C mutation - a novel cause of severe neonatal hyperammonemia requiring hemodialysis.

OBJECTIVES: Hyperammonemia in a newborn is a serious condition, which requires prompt intervention as it can lead to severe neurological impairment and death if left untreated. The most common causes of hyperammonemia in a newborn are acute liver failure and inherited metabolic disorders. Several mitochondrial disorders have been described as a cause of severe neonatal hyperammonemia. CASE PRESENTATION: Here we describe a new case of adenosine-triphosphate (ATP) synthase deficiency due to m.8528T>C mutation as a novel cause of severe neonatal hyperammonemia. So far six patients with this mutation have been described but none of them was reported to need hemodialysis in the first days of life. CONCLUSION: This broadens the so far known differential diagnosis of severe neonatal hyperammonemia requiring hemodialysis.

Early postoperative interventional aortic valve closure for severe aortic regurgitation in a neonate after Norwood procedure.

A neonate presented with signs of heart failure early after a Norwood procedure, due to increase of aortic and mitral valve regurgitation. Because repeated surgery was considered risky, we closed the aortic valve by catheter intervention with an Amplatzer Duct Occluder. Aortic regurgitation was abolished almost completely and the child improved. Unfortunately, 2 weeks after discharge, the patient died suddenly.

[Coarctation of the aorta in children in the 10-year epidemiological study: diagnostic and therapeutic consideration]. / Koarktacija aorte kod djece u 10-godisnjoj klinickoj epidemioloskoj studiji: dijagnosticko i terapijsko razmatranje.

Our study is a clinical epidemiological retrospective analysis of coarctation of the aorta in a 10-year follow-up (2001-2011). The study includes 201 children, 72 (35.82%) girls and 129 (64.18%) boys (1:1,6), with an average age of 28.57-49.37 mo (0.1-204 mo). They are categorized in 4 age groups: <1 month, 1 month - 1 year, 1 year - 6 years, >6 years. Isolated coarctation of the aorta was found in 125 (62.19%) patients; 33 (16.42%) preductal and 92 (45.77%) postductal. Coarctation of the aorta with an additional heart defect was found in 76 (37.81%) patients; 32 (15.32%) with ventricular septal defect, 28 (13.93%) within a complex heart defect, 11 (5.47%) within the Shone syndrome, and 5 (2.49%) with a dilated cardiomyopathy. Tiredness, intense tachypneic and dyspneic difficulties are dominant features in lower age groups (newborns and infants), while claudications, headaches and epistaxis are typical in older children. In the case of as many as 61 (30.35%) patients diagnosis was missed on the previous cardiological examination. In 20 (9.95%) patients coarctation is found within the known syndromes (Turner, Noonan, Williams Beuren, Ellis van Creveld, Down, partial trisomy 18, fetal valproate syndrome). Echocardiography was performed in all patients, and in 45 (22.38%) it was the only diagnos tic procedure. Altogether, 123 heart catheterizations, 38 multislice computed tomography and 15 magnetic resonance imagings were performed. The gradient on the place of coarctation before surgery or emergency procedures measured by catheter in 132 (65.67%) patients was 57.99 +/- 18.68 mmHg (20-100 mmHg). In 82 (40.80%) patients a bicuspid aortic valve was found. Average age at the time of surgery was 27.92 +/- 47.98 months (0.1-204 mo.). In 169 (84.07%) patients a cardiosurgical intervention was performed; 109 (54.23%) T-T anastomoses, 30 (14.29%) therapeutic catheterisations (balloon dilatation or stent implantation). Fatal outcome occurred in 4 (1.99%) children, all newborns or infants.

Croatian clinical epidemiological study (2008-2011): the use of standardised risk scores in paediatric congenital cardiac surgery for a case complexity selection and gradual progress of cardiosurgical model in developing countries.

OBJECTIVE: By employing the widely used and accepted methodologies of case-mix complexity adjustment in congenital cardiac surgery, we tried to evaluate our performance and use the ABC scores for a case complexity selection that may have different outcomes in various centres. METHODS: We analysed outcomes of cardiac surgical procedures - with or without cardiopulmonary bypass - performed in our institution between January, 2008 and December, 2011. Data were collected from the European Association for Cardio-Thoracic Surgery database. Together with prospective collection of these data, the data of all patients sent abroad to foreign cardiosurgical centres were recorded. RESULTS: During the period of study, 634 operations were performed; among them, 60% were performed in Croatia and 40% in foreign cardiosurgical centres. The number of operations performed in Croatia showed a linear increase: 55, 78, 121, and 126 operations performed in the years 2008, 2009, 2010, and 2011, respectively. Early mortality rates were 1.82%, 5.41%, 3.64%, and 3.48% in 2008, 2009, 2010, and 2011, respectively. The increase in the number of operations was followed by a satisfactory low average mortality rate of 3.85%. The mean ABC score complexity for operations performed in Croatia was 5.77. We determined a linear correlation between ABC score and early mortality, especially for the more complex operations. CONCLUSION: The use of standardised risk scores allows selection of complex cardiac diseases, which may have very different outcomes in various centres. In our case, those with higher ABC scores were correctly identified and referred for treatment abroad. In this way, we allowed gradual progress of the cardiosurgical model in Croatia and maintained an enviably low mortality rate.

[CROATIAN POPULATION AND CLINICAL EPIDEMIOLOGICAL STUDIES OF CONGENITAL HEART DISEASE (1995-2011): THE USE OF ABC SCORE AND RACHS-1 CARDIOSURGICAL MODEL FOR THE ASSESSMENT OF QUALITY OF TREATMENT OF CONGENITAL HEART DISEASE]. / HRVATSKE POPULACIJSKE I KLINICKE EPIDEMIOLOSKE STUDIJE PRIROÐENIH SRCANIH GRJESAKA (1995. - 2011.): PRIMJENA POKAZATELJA ABC I KARDIOKIRURSKOG MODELA RACHS-1 ZA PROCJENU KVALITETE ZBRINJAVANJA PRIROÐENIH SRCANIH GRJESAKA.

AIM: 1. To present an epidemiological (population and clinical) study of congenital heart defects (CHD) in Croatia in a 16-year period (1995-2011). 2. To analyze outcomes of surgical procedures for all patients in a five-year period (2002-2007) and to compare the results between Croatian and foreign centers. 3. To present the progress in surgical care of CHD in Croatia while acknowledging the requirement of achieving postsurgical mortality rate of below 5%. 4. To evaluate the projection of positive development of CHD management in Croatia in cooperation with major cardiac surgical centers in the neighboring countries. METHODS: Population study includes all children born from 1995 to 2000 and from 2002 to 2007 included in a database modeled by EUROCAT and BWIS. Outcome analysis was made using two models (ABC and RACHS-1) with early mortality rating and prolonged length of stay. Results: Based on two separate studies, mean value of CHD prevalence in Croatia is 7.6 per thousand. Outcome analysis according to the two mentioned evaluation models for the 2002-2007 period shows that children operated on in Croatia had a lower level of complexity compared to the complexity of those sent to foreign centers, but early mortality was below the assigned margin of 5% and there were also no differences in prolonged length of stay. As much as 63% of surgeries in the period were performed abroad, while the remaining 37% were performed in Croatia (351:202). In the following four-year period (2008-2011) there was a significant increase in the number of surgeries performed in Croatia when compared to those performed abroad (59:4% or 380:264). Independent EACTS analysis points to a positive trend of gradual increase in the number of surgeries and acceptance of higher complexity level of surgeries performed in Croatia, while maintaining the assigned margin (early mortality below 5%). Contribution: Proper selection of patients according to the complexity of cardiac surgical procedure is a prerequisite for both low mortality and fewer postsurgical complications. Professional advance relying on close cooperation with foreign centers is much faster and more acceptable than by "learning curves". CONCLUSION: Pediatric cardiac surgery in the developing countries must rely on the experiences of developed cardiac surgical centers in the neighboring countries due to complexity of congenital heart defects. Pediatric cardiology is inherently a public health problem, but the problem exacerbates with the appearance of a large number of adults with congenital heart defects (GUCH patients).

[Congenital coronary artery fistulas: clinical and therapeutic consideration]. / Kongenitalne koronarne arterijske fistule: klinicko i terapijsko razmatranje.

A coronary artery fistula is a link between one or more coronary arteries with another heart cavity or a segment of systemic or pulmonary circulation. Arterial blood from a coronary vessel enters another segment via myocardial capillary bed. These are very rare anomalies which constitute approximately 0.2 - 0.4% of all congenital heart defects. Still, they are clinically significant if they are of medium or large size and are manifested with a series of clinical symptoms such as angina pectoris, arrhythmias, myocardial infarction, endocarditis, progressive dilatation, heart failure and cardiomyopathy, pulmonary hypertension, thrombosis of the fistula and formation of aneurysms with possible ruptures. We present six patients with a coronary arterial fistula, their history, diagnostic procedures and outcomes. Therapeutic closure of coronary artery fistulas is recommended in all symptomatic, but also in asymptomatic patients, if there are significant roentgenographic, electrocardiographic and other abnormalities. In recent times transcatheter closure of coronary fistulas has become a possible alternative to surgery and is becoming increasingly used thanks to improved diagnostic possibilities and technology. If possible, interventional closure of fistulas is precisely the method preferred in pediatric patients. The choice of method depends on the anatomy of the fistula, presence or absence of additional defects, and on the experience of an interventional cardiologist or a heart surgeon. If performed well, the effects of both methods are good. This paper presents two children with a fistula between the right coronary artery and the right ventricle (RV), one child with a fistula between LAD and RV, one child with a fistula between the main tree of the left coronary artery (LCA) and RV, one child with a fistula between LCA and the right ventricular outflow tract (RVOT), and one child with a fistula between LCA and the right atrium (RA). The last one (LCA-RA) is not described in the latest classification of anomalies of coronary blood vessels in children based on MSCT coronarography, so we consider our presentation to be a contribution to the new classification. Along with the descriptions of fistulas and presentations of interventional and cardiosurgical interventions, we are also presenting a rare case of spontaneous closing of the fistula within the first six months and of a reopening of the fistula between the right coronary artery and the right ventricle after six years.

[Distribution of congenital heart disease in Croatia and outcome analysis. A Croatian epidemiological study (2002-2007)]. / Distribucija prirodenih srcanih grjesaka u Hrvatskoj i analiza ishoda lijecenja. Hrvatska epidemioloska studija (2002.-2007).

OBJECTIVE: The aim of our paper is to show results of population and hospital registry of congenital heart disease in Croatia. METHODS: Information on patients born during the five-year study and with diagnosis of congenital heart defects, obtained all across the country, were collected in the population and hospital registry set up according to the EUROCAT and BWIS registries principles. RESULTS: Between October 1, 2002, and October 1 2007, there were 205051 live births in Croatia, of which 1480 patients were diagnosed with congenital heart defects, accounting for 0.72% of the live-born children. The most common diagnosis was ventricular septal defect with percentage of 34.6%. Among 1480 children, 430 needed an operation. Among 553 cardiac surgeries performed, 202 were done in Croatia and others were done in institutions abroad. Mortality rate after surgery was 5%. Only after adjustment for complexity there are marked differences in mortality and occurrence of postoperative complications between Croatia and centers abroad. CONCLUSION: The importance of the registry of congenital heart disease relies in the field of epidemiological research but can be used as a tool for future planning of health services.

Evaluation of paediatric cardiosurgical model in Croatia by using the Aristotle basic complexity score and the risk adjustment for congenital cardiac surgery-1 method.

OBJECTIVE: The Aristotle basic complexity score and the risk adjustment in congenital cardiac surgery-1 method were developed and used to compare outcomes of congenital cardiac surgery. Both methods were used to compare results of procedures performed on our patients in Croatian cardiosurgical centres and results of procedures were taken abroad. METHODS: The study population consisted of all patients with congenital cardiac disease born to Croatian residents between 1 October, 2002 and 1 October, 2007 undergoing a cardiovascular operation during this period. RESULTS: Of the 556 operations, the Aristotle basic complexity score could be assigned to 553 operations and the risk adjustment in congenital cardiac surgery-1 method to 536 operations. Procedures were performed in two institutions in Croatia and seven institutions abroad. The average complexity for cardiac procedures performed in Croatia was significantly lower. With both systems, along with the increase in complexity, there is also an increase in mortality before discharge and postoperative length of stay. Only after the adjustment for complexity there are marked differences in mortality and occurrence of postoperative complications. CONCLUSION: Both, the Aristotle basic complexity score and the risk adjustment in congenital cardiac surgery-1 method were predictive of in-hospital mortality as well as prolonged postoperative length to stay, and can be used as a tool in our country to evaluate a cardiosurgical model and recognise potential problems.

[Diagnostic value of myocardial biopsy in the diagnostic algorithm in cardiomyopathies in children]. / Dijagnosticka vrijednost biopsije miokarda u dijagramu dijagnostickog toka kardiomiopatija u djece.

The purpose of this work was to show the importance of myocardial bioptate analysis using different methods in the diagram of diagnostic flow in primary cardiomyopathies in children. According to the guidelines of the Task Force on Cardiomyopathies of the WHO/ISFC, we identified 121 children (50 f and 71 m) as having cardiomyopathy, giving an average occurrence for all cardiomyopathies of 38.81 for each 10,000 pts examined in our outpatient clinics for paediatric cardiology. The dilated cardiomyopathy (DCM) was identified in 52 pts (42.9%), hypertrophic cardiomyopathy (HCM) in 43 pts (35.5%) and restrictive cardiomyopathy (RCM) in 6 pts (4.8%). We placed 11 pts (9.0%) in the group of specific cardiomyopathies. In nine pts (7.4%), it was impossible to classify the cardiomyopathy. Most of those with DCM had been diagnosed prior to the age of 3 years (RR 1.9, 95% CI 1.4-2.47). There were no statistically significant differences in the incidences of DCM as compared to HCM (Z 0.923, p < 0.1779), but we encountered a significantly lower occurrence of RCM (Z 6.044, p < 0.001). The biopsy of endocardium and myocardium was done to confirm the etiology of primary cardiomyopathy in 22 pts, 12 m and 10 f, age 1 to 17 (average age 9.5y). The bioptates were analysed by light microscope (Dallas criteria) in all pts, 13 bioptates by direct immunofluorescence, 8 by immunohystochemical method (two hystochemically by the method of coloring with Kongo red, one by the microscopy in polarised light), 7 by electron microscope, and 5 by PCR method where DNA and RNA of cardiotrophic viruses was used. Out of 10 pts with DCM, in 4 myonecrosis as a consequence of acute myocarditis and in 6 signs of late inflammatory processes, as a consequence of chronic immunologic myocarditis, were found. In 4 of them rebiopsy proved complete healing. In 5 pts with HCM the diagnosis was confirmed hystologically. One bioptate was analysed by electron microscope to rule out mitochondriopathy. Out of 4 pts with RCM due to inflammation, in 3 pathohistological findings proved diagnosis and in one showed primary amyloidosis. In one patient pathohystological finding showed fibroelastosis. In one patient heart tumor (fibroma) has been found.