Supracardiac Total Anomalous Pulmonary Venous Return Repair in a 7-Month-Old Infant.

Total anomalous pulmonary venous return is a congenital heart malformation characterized by anomalous pulmonary venous inflow to the right atrium. Surgical repair typically occurs during the first month of life, and survival beyond that age in untreated patients is unlikely. We report an extreme case of supracardiac total anomalous pulmonary venous return in an infant who survived 7 months despite atypical anomalous inflow without atrial-level communication and with right-to-left shunting only through a patent ductus arteriosus.

Pediatric Cardiac Service Development in Croatia.

This article describes the establishment of a pediatric cardiac service program in a limited resource country. According to national epidemiological studies about 330 children with congenital heart disease (CHD) are born every year in Croatia. As a part of the former Yugoslavia, there was not an organized strategy for the pediatric cardiac service.

The aorto-left ventricular tunnel from a fetal perspective: Original case series and literature review.

Introduction: Aorto-left ventricular tunnel (ALVT) accounts for <0.1% of congenital heart defects. Evidence on the prognosis from a fetal perspective is limited.

Rituximab in Treatment of Children with Refractory Vasculitis and Systemic Lupus Erythematosus - Single Center Experience in Croatia.

The aim of this study was to present our experience in rituximab therapy in patients with childhood-onset systemic lupus erythematosus, lupus nephritis, and ANCA-associated vasculitis. We conducted a retrospective clinical chart review of all patients treated with rituximab in the time period from January 2009 to December 2015. Eight patients (3 boys and 5 girls) aged 8 to 15 at the onset of disease were treated with rituximab.

Extracorporeal Membrane Oxygenation After Glenn Procedure in a Child With Influenza B Pneumonia.

A five-month-old male with a hypoplastic left heart syndrome developed severe respiratory insufficiency due to influenza type B viral pneumonia following bidirectional Glenn. He was treated with extracorporeal membrane oxygenation and successfully weaned without neurological consequences.

Pulmonary artery banding in dilative cardiomyopathy of young children: review and protocol based on the current knowledge.

Dilated cardiomyopathy (DCM) is a leading cause of cardiac death in children. Current therapeutic strategies are focused on improving symptoms of congestive heart failure (CHF); the potentials of cardiac regeneration especially in infants and young children are neglected in particular when DCM is classified as "end-stage". Heart transplantation (HTx) serves as the only life-saving option, despite is palliative character with limited survival time.

Overlapping Phenotypes and Degree of Ventricular Dilatation Are Associated with Severity of Systolic Impairment and Late Gadolinium Enhancement in Non-Ischemic Cardiomyopathies.

BACKGROUND Dilatation and other infrastructural rearrangements of the left ventricle are connected with poor prognosis. The aim of our study was to analyze the overlapping phenotypes and dilatation of the ventricle on impairment of systolic function and existence of late gadolinium enhancement (LGE). MATERIAL AND METHODS Consecutive sample of cases with dilated left ventricle due to non-ischemic cardiomyopathy and healthy controls were included from our cardiac magnetic resonance imaging (CMR) database for a period of 3 years (n=1551 exams).

[RIGHT VENTRICULAR ARRHYTMOGENIC CARDIOMYOPATHY – HAVE WE AVOIDED A FAMILY TRAGEDY BY APPLYING CONTEMPORARY DIAGNOSTIC AND TREATMENT APPROACH?].

Arrhytmogenic right ventricular cardiomyopathy (ARVC) is an inherited cardiomyopathy characterized by fibrofatty replacement, dominantly in the right, but often also in the left ventricle. It is a significant cause of sudden death in children and adolescents. A thorough family history and contemporary diagnostic and treatment approach are prerequisites for the prevention of the sudden death syndrome.

A novel de novo TBX5 mutation in a patient with Holt-Oram syndrome leading to a dramatically reduced biological function.

Background: The Holt-Oram syndrome (HOS) is an autosomal dominant disorder affecting 1/100.000 live births. It is defined by upper limb anomalies and congenital heart defects with variable severity.

[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].

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.

[TAKAYASU ARTERITIS AND POSSIBLE CARDIOLOGY REPERCUSSIONS IN THE CHILDHOOD].

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.

[VASCULITIDES IN CHILDHOOD: A RETROSPECTIVE STUDY IN A PERIOD FROM 2002 TO 2012 AT THE DEPARTMENT OF PAEDIATRICS, UNIVERSITY HOSPITAL CENTRE ZAGREB].

The aim of our study was to analyze clinical features, laboratory findings, treatment, course and outcome of different types of vasculitis in children. All children aged up to 18 years that have been diagnosed with a vasculitis disorder from 2002. to 2012.

[BICUSPID AORTIC VALVE AND LEFT VENTRICULAR OUTFLOW TRACT DEFECTS IN CIIILDREN - SYNDROME OF BICUSPID AORTOPATHY?].

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.

[RHEUMATOLOGY IN PEDIATRIC CARDIOLOGY FROM FETAL STAGE TO ADULTHOOD. THE ROLE OF FETAL ECHOCARDIOGRAPHY].

Even though in the last ten years pediatric cardiology and rheumatology have formally separated in Croatia as well, strong links still remain between them. They are no longer manifested through rheumatic fever, but through other rheumatic entities: systemic lupus erythematosus, antiphospholipid syndrome, systemic scleroderma, Kawasaki disease, polyarteritis nodosa, and some forms of granulomatous vasculitis. We take special note of the occurrence of complete congenital atrioventricular block (CCAVB) in pregnant women who, due to systemic connective tissue diseases, develop distinctive ANA antibodies (anti-SSA/Ro and/or anti SSB/La), but who are also likely to have the same inflammation within the heart, leading to the development of restricted cardiomyopathy associated with CCAVC.

[MYOCARDIAL INFARCTION AS A CONSEQUENCE OF CHILDHOOD POLYARTERITIS NODOSA – CASE REPORT].

Polyarteritis nodosa (PAN) is a systemic vasculitis histologically characterized by necrotic lesions of small and medium- sized arteries occurring mostly in their bifurcations. PAN is a multi-organ disorder that affects numerous visceral arteries and leads to inflammation and necrosis, which may result in ischemia of vital organs. The etiology of the disease is unknown.

[Coarctation of the aorta in children in the 10-year epidemiological study: diagnostic and therapeutic consideration].

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.

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.

[Congenital coronary artery fistulas: clinical and therapeutic consideration].

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.

Childhood-onset systemic lupus erythematosus in Croatia: demographic, clinical and laboratory features, and factors influencing time to diagnosis.

Objectives: Childhood-onset systemic lupus erythematosus (cSLE) presents with diverse clinical features and often with non-classical symptoms that may delay diagnosis and increase risk of morbidity and mortality. This paper aims to analyse incidence, and clinical and laboratory features of cSLE in Croatia between 1991 and 2010, and to identify factors influencing time to diagnosis.

Results: Medical records at three university-based tertiary care centres were analysed retrospectively for 81 children with cSLE (68 girls).

Treatment of right ventricle to pulmonary artery conduit stenosis in infants with hypoplastic left heart syndrome.

Objectives: To determine the incidence of right ventricle-to-pulmonary artery (RV-PA) conduit stenosis after the Norwood I operation in patients with hypoplastic left heart syndrome (HLHS), and to determine whether the treatment strategy of RV-PA conduit stenosis has an influence on interstage and overall survival.

Methods: Ninety-six patients had a Norwood operation with RV-PA conduit between 2002 and 2011. Details of reoperations/interventions due to conduit obstruction prior to bidirectional superior cavopulmonary anastomosis (BSCPA) were collected.

Polyarteritis nodosa in Croatian children: a retrospective study over the last 20 years.

To analyze the disease characteristics, treatment modalities and outcome of polyarteritis nodosa (PAN) in Croatian children. Cross-sectional study included all children with PAN diagnosed according to EULAR/PRES/PRINTO criteria during the last two decades. PAN was diagnosed in 12 patients (6 girls and 6 boys) mean age (±SD) 11.

Huge coronary fistula from the left anterior descending coronary artery to the right ventricle in a newborn: a rare congenital anomaly.

We present a case of a neonate with a huge congenital fistula from the left anterior descending coronary artery to the right ventricle and additional heart defects. The circumflex coronary artery was supplied by the right coronary artery. The fistula was closed surgically at both ends.

[The development of interventional catheterisation in the referral centre for pediatric cardiology of the Republic of Croatia--a retrospective study 1996-2009].

The goal of our study was to show an increase in the number of interventional heart catheterisations in the last 14 years in the Referral Centre for Pediatric Cardiology of the Republic of Croatia. From January 1996 to December 2009 2379 children (51% male and 49% female) underwent invasive diagnostic procedures (mean age 4.1+3.