Sacubitril/Valsartan in Pediatric Heart Failure (PANORAMA-HF): A Randomized, Multicenter, Double-Blind Trial.

BACKGROUND: Sacubitril/valsartan, an angiotensin receptor-neprilysin inhibitor (ARNI), is an established treatment for heart failure (HF) with reduced left ventricular ejection fraction. It has not been rigorously compared with angiotensin-converting enzyme inhibitors in children. PANORAMA-HF (Prospective Trial to Assess the Angiotensin Receptor Blocker Neprilysin Inhibitor LCZ696 Versus Angiotensin-Converting Enzyme Inhibitor for the Medical Treatment of Pediatric HF) is a randomized, double-blind trial that evaluated the pharmacokinetics and pharmacodynamics (PK/PD), safety, and efficacy of sacubitril/valsartan versus enalapril in children 1 month to <18 years of age with HF attributable to systemic left ventricular systolic dysfunction (LVSD). METHODS: Children with HF attributable to LVSD were randomized to sacubitril/valsartan versus enalapril to assess the efficacy and safety of sacubitril/valsartan at 52 weeks of follow-up. The primary end point of the study was to determine whether sacubitril/valsartan was superior to enalapril for the treatment of pediatric patients with HF attributable to systemic LVSD, assessed using a primary global rank end point consisting of ranking patients from worst to best on the basis of clinical events such as death, listing for urgent heart transplant, mechanical life support requirement, worsening HF, New York Heart Association (NYHA)/Ross class, Patient Global Impression of Severity (PGIS), and Pediatric Quality of Life Inventory physical functioning domain. The change from baseline to 52 weeks in NT-proBNP (N-terminal pro-B-type natriuretic peptide) was an exploratory end point. RESULTS: A total of 375 children (mean age, 8.1±5.6 years; 52% female) were randomized to sacubitril/valsartan (n=187) or enalapril (n=188). At week 52, no significant difference was observed between the 2 treatment arms in the global rank end point (Mann-Whitney probability, 0.52 [95% CI, 0.47-0.58]; Mann-Whitney odds, 0.91 [95% CI, 0.72-1.14]; P=0.42). At week 52, clinically meaningful reductions were observed in both treatment arms in NYHA/Ross, PGIS, Patient Global Impression of Change, and NT-proBNP, without significant differences between groups. Adverse events were similar between treatment arms (incidence: sacubitril/valsartan, 88.8%; enalapril, 87.8%), and the safety profile of sacubitril/valsartan was acceptable in children. CONCLUSIONS: In this study, sacubitril/valsartan did not show superiority over enalapril in the treatment of children with HF attributable to systemic LVSD using the prespecified global rank end point. However, both treatment arms showed clinically meaningful improvements over 52 weeks. REGISTRATION: URL: https://www.clinicaltrials.gov; Unique identifier: NCT02678312.

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.

Extracorporeal membrane oxygenation in children: An update of a single tertiary center 11-Year experience from Croatia.

INTRODUCTION: Extracorporeal membrane oxygenation (ECMO) is an important treatment option for organ support in respiratory insufficiency, cardiac failure, or as an advanced tool for cardiopulmonary resuscitation. Reports on pediatric ECMO use in our region are lacking. METHODS: This study is a retrospective review of all pediatric cases that underwent a veno-arterial (VA) or veno-venous (VV) ECMO protocol between November 2009 and August 2020 at the Department of Pediatrics, University Hospital Center Zagreb, Croatia. RESULTS: Fifty-two ECMO runs identified over the period; data were complete for 45 cases, of which 23 (51%) were female, and median age was 8 months. Thirty-eight (84%) patients were treated using the VA-and 7 (16%) using VV-ECMO. The overall survival rate was 51%. Circulatory failure was the most common indication for ECMO (N = 38, 84%), and in 17 patients ECMO was started after cardiopulmonary resuscitation (E-CPR). Among survivors, 74% had no or minor neurological sequelae. Variables associated with poor outcome were renal failure with renal replacement therapy (p < .001) and intracranial injury (p < .001). CONCLUSION: Overall survival rate in our cohort is comparable to the data published in the literature. The use of hemodialysis was shown to be associated with higher mortality. High rates of full neurological recovery among survivors are a strong case for further ECMO program development in our institution.

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.

Management of failing bidirectional cavopulmonary shunt: Influence of additional systemic-to-pulmonary-artery shunt with classic Glenn physiology.

Objectives: Severe hypoxemia in the early postoperative period after bidirectional cavopulmonary shunt (BCPS) is a critical complication. We aimed to evaluate patients who underwent additional systemic to pulmonary shunt and septation of central pulmonary artery (partial takedown) after BCPS. Methods: The medical records of all patients who underwent BCPS between 2007 and 2020 were reviewed. Patients who underwent partial takedown were extracted and their outcomes were analyzed. Results: Of 441 BCPS patients, 27 patients (6%) required partial takedown. Most frequent diagnosis was hypoplastic left heart syndrome (n = 14; 52%). Additional complicating factors included pulmonary artery hypoplasia (n = 12) and pulmonary venous obstruction (n = 3). Thirteen patients (48%) underwent partial takedown on the same day of BCPS, and all of them survived the procedure. The remaining 14 patients (52%) underwent partial takedown between postoperative 1 to 64 days. The reasons for partial takedown were: postoperative high pulmonary vascular resistance (n = 4), early BCPS (<90 days) with PA hypoplasia (n = 3), mediastinitis/pneumonia (n = 3), pulmonary venous obstruction (n = 2), ventricular dysfunction (n = 1), and recurrent pneumothorax (n = 1). Four patients experienced hospital deaths. Six patients died after discharge, 10 achieved Fontan completion, and 6 were alive and waiting for Fontan. Overall survival after partial takedown was 54% at 3 years. The pulmonary venous obstruction (P = .041) and genetic/extracardiac anomalies (P = .085) were identified as risks for mortality after partial takedown. Conclusions: The partial takedown resulted in a 3-year survival rate of more than 50%. Of these patients, a significant number underwent successful Fontan completion who would exhibit potential early death with conservative treatment.

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.

Extracorporeal cardiopulmonary resuscitation with low pump flow for blocked modified Blalock-Taussig shunt followed by spontaneous recanalization.

A 2-week-old male newborn with a double inlet left ventricle developed a cardiac arrest following modified Blalock-Taussig anastomosis in pediatric intensive care unit. Probable causes of the arrest were hemodynamic instability and thrombosed shunt, which was later recanalized on extracorporeal membrane oxygenation therapy, which was successfully used with a pump flow lower than recommended in these patients-without the shunt clip, but without any complications.

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.

Ventricular tachycardia in a 16-year-old boy with primary hyperparathyroidism.

We report a case of primary hyperparathyroidism with hypercalcaemia in a 16-year-old boy who had spontaneous attack of ventricular tachycardia. To our knowledge, presentation of ventricular tachycardia caused by primary hyperparathyroidism in such a young patient has not been reported in medical literature so far.

Cardiopulmonary exercise performance is reduced in congenital diaphragmatic hernia survivors.

BACKGROUND: Congenital diaphragmatic hernia (CDH) is associated with lung hypoplasia. CDH survivors may have pulmonary morbidity that can decrease cardiopulmonary exercise. We aimed to examine whether cardiopulmonary exercise testing (CPET) results differ in CDH survivors versus healthy age-matched controls and whether CPET results among CDH survivors differ according to self-reported daily activity. METHODS: In one medical center in Croatia, CDH survivors-patients with surgically corrected CDH who were alive at age 5 years-were invited to participate in spirometry and CPET. Values were compared with those of controls matched 2:1 by age and sex for each CDH survivor aged 7 years or older. RESULTS: Among 27 CDH survivors aged 5-20 years, 13 (48%) had continued symptoms or spirometric evidence of pulmonary disease. Compared with controls (n = 44), survivors (n = 22) had lower peak oxygen consumption (V˙O2 mean [SD], 35.7 [6.9] vs. 45.3 [8.2] ml/kg per min; P < 0.001). At peak exercise, V˙O2/heart rate (P < 0.001), tidal volume (P = 0.005), and minute ventilation (P < 0.001) were lower in survivors, but the maximal respiratory rate was not different (P = 0.72). Among survivors, mean (SD) V˙O2peak (ml/kg per min) differed by self-reported activity level: athletic, 40.3 (5.0); normal, 35.8 (6.5); and sedentary, 32.1 (6.8) (by ANOVA, P = 0.10 across three groups and P = 0.04 athletic vs. sedentary). CONCLUSION: More than half of CDH survivors continue to have chronic pulmonary disease. CDH survivors had lower aerobic exercise capacity than controls. Self-reporting information on daily activities may identify CDH patients with low V˙O2max who may benefit from physical training. Pediatr Pulmonol. 2016;51:1320-1329. © 2016 Wiley Periodicals, Inc.

[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.

[BICUSPID AORTIC VALVE AND LEFT VENTRICULAR OUTFLOW TRACT DEFECTS IN CIIILDREN - SYNDROME OF BICUSPID AORTOPATHY?]. / BIKUSPIDALNA AORTALNA VALVULA I GRJESKE IZLAZNOG TRAKTA LIJEVE KLIJETKE U DJECE - SINDROM BIKUSPIDALNE AORTOPATIJE?

UNLABELLED: Although bicuspid aortic valve (BAV) is considered the most common congenital heart defect (CHD) in adult age, with the 0.5-2% prevalence, BAV is not part of epidemiological studies of congenital heart defects (CHD) in children. Aortic valvulogenesis disorder is part of the left ventricular outflow tract (LVOT) genetic disorders which include: hypoplastic left heart syndrome (HLHS), aortic stenosis (AS) and insufficiency (AI), dilatation of the ascending aorta (DAA), coaretation of the aorta (CoA), Shone's syndrome (SS), and probably some other disorders. Our observations indicate that BAV related significant pathologic and hemodynamic changes occur in children already. In an 11-year long retrospective study (2000-2011) we have found 229 BAV patients, predominantly males (1.7). The most common BAV associated disorder was CoA (75 patients, 32.6%). Of all the children with BAV, 62.4% (143:229) had hemodynamic alterations on the aortic valve which manifested themselves as aortic stenosis and/or insufficiency. AS is mostly progressive and becomes hemodynamically relevant in childhood age already, while AI is mostly mild and rarely hemodynamically relevant. A large proportion of patients had isolated AS with DAA (21 or 14.7%), while most patients had combined AS and AI (29 or 20.3%). Due to morphological changes on the valve itself and on the adjoining defects, numerous interventional and cardiosurgical procedures have been performed. Their number has been growing with age, in accordance with the expected progression of pathological changes on the valve (AS, AI) or on the aorta (DAA). DAA in children with BAV was found in 76 (33.2%) patients, in various combinations with other associated LVOT anomalies. Already in childhood have the following surgical procedures on children with primary BAV diagnosis been performed : resection of CoA with T-T anastomosis was performed in 56 patients (24.5%); balloon aortic valvuloplasty in 28 patients (12.3%); commissurotomy in 19 patients (8.3%); balloon dilatation of CoA in 15 patients (6.5%); subaortic membrane resection in 11 patients (4.8%); Ross procedure in 8 patients (3.5%); resection of CoA with reconstruction in 8 patients (3.5%); valvuloplasty in 6 patients (2.6%); ascending aortoplasty in 5 patients (2.2%); mechanical valve replacement in 3 patients (1.3%); "subclavian flap" in 3 patients (1.3%); biological aortic valve replacement in 2 patients (0.9%); Bentall procedure in 1 patient (0.4%); David procedure in 1 patient (0.4%). CONTRIBUTION OFTHE STUDY: A BAV finding in children is a predictive factor for a progressive development of morphological changes in various LVOT parts, requiring that in some patients hemodynamic repercussions be removed already in childhood. CONCLUSION: The term valvular aortopathy, that is bicuspid aortic valve syndrome, should be in use already for children, and the anomaly should be included in epidemiological CHD research.

[TAKAYASU ARTERITIS AND POSSIBLE CARDIOLOGY REPERCUSSIONS IN THE CHILDHOOD]. / TAKAYASUOV ARTERITIS I MOGUCE KARDIOLOSKE REPERKUSIJE U DJECJOJ DOBI.

Vasculitides are rare rheumatic diseases of unknown etiology whose main characteristic is a necrotizing inflammation of blood vessels. We are presenting two patients with Takayasu arteritis (TA) as entity forms of rare rheumatic diseases. One patient had TA type IIa and the other type IV. In the first patient we found severe symptoms of obstructive lesions of aortic branches, particularly severe coronary artery stenosis and complete occlusion of the left subclavian artery, and thoracic artery stenosis below the isthmus. The disease was diagnosed in the acute phase, treated extensively with medicaments (glucocorticoids, cytostatics, methotrexate) and a complex cardiac surgical procedure, and due to relapse the biological (Rituximab) therapy was used. The second patient was detected following symptomatic arterial hypertension, with absent pulses of lower limbs, whose cause was found in severe narrowing of the aorta from diaphragm to femoral arteries bifurcation (mid-aortic syndrome). The disease was not active when diagnosis was made. The patient was treated with a particular cardiac surgical procedure and with multiple medicaments due to a relapse. Both patients have reached adolescent age and are successfully treated with a satisfying quality of life. Type IIa with an additional occlusion of coronary arteries is not described in the available literature. Forementioned vasculitides emphasize the importance of pediatric cardiologists and rheumatologists teamwork.

[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.