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. We stabilized the patient's left-sided heart function before surgically repairing the anomalous venous return 2 months later. Three years postoperatively, the patient was well.

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. After Croatian independence in 1991, even during the war, a need for such service led to the first step in the development of organized care for patients with CHD, a humanitarian mission provided by a non-governmental organization (NGO), from the United States. In the ten-year period (1993-2003), 601 children were operated on during this program. After the end of this program, the Croatian team was not able to cover the whole spectrum of pediatric cardiac care independently. About 60% of the children were sent abroad, and only about 40% of the operations were performed in Croatia. Over the time, the surgical team improved and after a specialized congenital heart surgeon joined, the number and quality of the program in the country raised, and the number of referrals to foreign centers gradually declined. In the meantime, a cardiological interventional program also improved. Today majority of standard congenital heart surgery procedures can be performed in Croatia. Last year our congenital heart team operated on 180 patients with low mortality and the interventional team (pediatric and adult) performed 66 procedures. In the article, we present positive shifts achieved during time as well as weaknesses and reasons for problems in establishing a high-quality CHD center.

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. With this retrospective international case series, we provide information on the outcome of fetuses with ALVT. METHODS: All members of the Association for European Pediatric and Congenital Cardiology's (AEPC) fetal working group and fetal medicine units worldwide were invited for participation. We observed antenatal parameters, neonatal outcome and postnatal follow-up. Additionally, a systematic search of the literature was performed. RESULTS: Twenty fetuses with ALVT were identified in 10 participating centers (2001-2019). Fetal echocardiographic characteristics of ALVT included an increased cardiac-thorax ratio (95%), left ventricular end-diastolic diameter (90%) and a dysplastic aortic valve (90%). Extracardiac malformations were rare (5%). Eight fetuses died at a median gestational age (GA) of 21 + 6 weeks (range, 19-24): all showed signs of hydrops prior to 24 weeks or at autopsy. All others (60%, 12/2) were live-born (median GA 38 + 4, range 37-40), underwent surgery and were alive at last follow up (median 3.2 years, range 0.1-17). The literature reported 22 ALVT fetuses with similar outcome. CONCLUSIONS: In the absence of fetal hydrops, ALVT carries a good prognosis. Fetuses who survive to 24 weeks without hydrops are likely to have a good outcome.

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.

[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. Although rheumatology has thus been involved with early fetal age for some time now, there are also some issues relating to the embryonal stage (the association between methotrexate/folic acid and heart development in embryos), as well as to the late fetal stage (antiprostaglandin anti-infl ammatory agent impact on premature ductus arteriosus closure). We gave special attention to the neonatal lupus syndrome, the most serious complication in the fetal period. Thus the multiple association between cardiology and rheumatology in all age groups, from the embryonal and fetal stage to adulthood, is being realized. The aim of this paper is to present some important links and thus further emphasize the new cooperation between pediatric cardiology and rheumatology from fetal stage to adulthood.

[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. The diagnosis is more often established in adults than in children. Patients: We present a family with four members affected and diagnosed with ARVC already in childhood/adolescence. The average age of symptom presentation was 12 years (10­14 years). The importance of family tree isemphasized and contemporary diagnostic and treatment methods, with the aim of sudden death prevention, are described. Conclusions: Family history is essential for the early ARVC diagnosis. There is a need for revision of current diagnostic criteria in pediatric population. In the future progression of fibrous tissue proportion in relation to age and the stage of the disease should be taken in account. Furthermore, the repolarisation abnormality is inappropriate as a criterion in children less than 14 years old, so in that age group less of present diagnostic criteria can be applied. A combination of modern implantable cardioverter defibrillator and radiofrequency ablation assures an excellent life quality in our patients. However, the longterm prognosis for our patients, because of a progressive course of the disease, remains uncertain.

[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.

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). Mean age at onset was 13.4±2.8 yr (interquartile range 3), and annual incidence varied from 1-15 per million at risk. The most frequent clinical and laboratory features were musculoskeletal symptoms (80%) and increased erythrocyte sedimentation rate (96%). The most frequent immunological laboratory findings were the presence of antibodies against histones (86%), double-stranded DNA (73%), and Sm protein (64%), as well as low levels of C3 complement (69%). Haematuria was present in 58% of children, proteinuria in 56%, and biopsy-confirmed lupus nephritis in 43%. Median time from symptom onset to diagnosis was 2 months (range 0-96). Time to diagnosis was inversely associated with ECLAM score (p<0.001), but it showed no association with age, gender, clinical features or distance from the nearest paediatric centre. CONCLUSIONS: This is the first large-scale, in-depth study of clinical and laboratory features of cSLE in Croatia. Among all demographic, laboratory and clinical features examined, ECLAM score alone was inversely associated with time to diagnosis. This highlights the need to improve detection of children with fewer symptoms early in the course of the disease, therefore serious consequences for prognosis could be avoided.

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. RESULTS: Overall pre-BSCPA mortality was 17%, early mortality after Norwood, 6%. Early angiography was performed in 34 patients due to desaturation at a median of 8 days after the Norwood operation. Fifteen patients (16%) were diagnosed with RV-PA conduit stenosis that required treatment. The location of the conduit stenosis was significantly different in the patients with non-ringed (proximal) and the patients with ring-enforced conduit (distal), P = 0.004. In 6 patients, a surgical revision of the conduit was performed; 3 of them died prior to BSCPA. Another 6 patients had a stent implantation and 3 were treated with balloon dilatation followed by a BSCPA in the subsequent 2 weeks. All patients who were treated interventionally for RV-PA conduit obstruction had a successful BSCPA. Patients who received a surgical RV-PA conduit revision had a significantly higher interstage (P = 0.044) and overall mortality (P = 0.011) than those who received a stent or balloon dilatation of the stenosis followed by an early BSCPA. CONCLUSIONS: RV-PA conduit obstruction after Norwood I procedure in patients with HLHS can be safely and effectively treated by stent implantation, balloon dilatation and early BSCPA. Surgical revision of the RV-PA conduit can be reserved for patients in whom an interventional approach fails, and an early BSCPA is not an option.

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) 11.33 ± 3.08 years. The share of PAN among all vasculitides was 3.8 %. Systemic PAN was diagnosed in 7 children (58 %), microscopic polyangiitis in 3 (25 %), cutaneous PAN in 2 (17 %). The most consistent symptoms were skin involvement (90 %) and arthritis/arthralgia (60 %). The CNS was affected in 33 % of patients. Inflammatory markers (C-reactive protein and erythrocyte sedimentation rate [ESR]) were elevated in all patients, and anti-neutrophil cytoplasmatic antibodies were positive in all patients with microscopic polyangiitis. Therapy mode for all patients was corticosteroids. Immunosuppressive drugs were used as additional therapy for patients with severe symptoms. Two patients (17 %), both suffering from microscopic polyangiitis, died due to renal failure during the follow-up. In comparison with available studies, we found a difference in distribution of childhood polyarteritis nodosa as well as some clinical characteristics (e.g., higher prevalence of neurological and pulmonary symptoms), while other researched features, laboratory and treatment were similar.

[The development of interventional catheterisation in the referral centre for pediatric cardiology of the Republic of Croatia--a retrospective study 1996-2009]. / Razvoj interventne dijagnostike u Referentnom centru za pedijatrijsku kardiologiju RH--retrospektivna studija 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+3.8 years, range 1 day-20.5 years). In that period in 22.27% (2379/530) of the children a certain interventional procedure was performed. The number of interventional catheterisations increased from 20/160 (12.5%) in 1996 to 70/224 (31.25%) in 2009. Some interventional procedures were introduced before 1996 (Rashkind BAS, dilatation of pulmonary valve stenosis and myocardial biopsy), while other methods were introduced in the following order: dilatation of critical aortic stenosis in newborns and other ages from 1996, ductus Bottali closure from 1997, interatrial septal defect closure from 2000, native coarctation and recoarctation of the aorta from 2003. Of 530 interventional catheterisations involved in the study during the period of 14 years, there were 140 atrioseptostomias (26.4%), 80 pulmonary valve dilatations (15.1%), 58 aortic valve dilatations in older children (6.8%), 9 dilatations of critical aortic stenosis (1.7%), 58 dilatations of coarctation (10.9%) (of which 32 native and 26 recoarctations), 133 ductus Botalli closures (25.1%) (4 with PFM coils, 68 with Cook coils and 61 with Amplatzer Duct occluder). From 2000 interatrial septal defects were closed in 37 patients, 14 of which were closed with Cardioseal-STARflex system (37.84%) and 23 with Amplatzer device (62.16%). Myocardial biopsy was performed in 37 patients (7%) with 4-5 endomyocardial samples per patient (mainly from the left ventricle) so that we had 120 heart samples in total for further analysis. Hi2-test determined a constant rise in catheterisations in relation to the mean number (p<0.05) except for the years 1997 and 1998 when it was significantly lower than the average. A positive trend in the increase of interventional catheterisations was determined (p<0.005). The share of interventional catheterisations compared to diagnostic catheterisations was found to have statistically significantly increased (p<0.05) from 1996 to 2009. We conclude that in the observed period there is a slight increase in the overall number of catheterisations and a more intense increase of interventional catheterisations, particularly after 1999.

[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.

False aneurysm origination from the proximal anastomosis of a right ventricular to pulmonary artery shunt following staged repair of hypoplastic left heart syndrome.

The Norwood I operation, including placement of a shunt from the right ventricle to the pulmonary arteries, has been adopted by many surgeons for neonates with hypoplastic left heart syndrome. A three-year-old male who had undergone the Norwood I operation, and the Glenn operation, presented with a cervical pulsating tumor prior to the operation for total cavopulmonary connection. At the Glenn operation, the right ventricle to the pulmonary artery shunt was closed with a clip proximally, and the distal part was resected. Following the Glenn operation, the child had had recurrent deep sternal infections caused by Serratia marcescens. Cardiac catheterization showed a false aneurysm from the proximal shunt anastomosis. The bleeding after resternotomy was managed by initiating cardiopulmonary bypass via the groin vessels. Cerebral air embolies were prevented by systemic application of potassium, to achieve cardioplegic arrest during chest opening. The shunt was removed and the defect was closed. After the shunt was confirmed to be free from infection, a total cavopulmonary connection was performed after three days postoperatively. The case illustrates the management of retrosternal aneurysms during resternotomy in children.

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.

Ostial patch plasty of an aberrant right coronary artery in a symptomatic teenager.

An otherwise healthy 15-year-old girl presented with a history of transient myocardial ischemia. At the time, troponin levels were elevated to 20 microg/L. A myocardial perfusion scintigraphy evaluation revealed a minor scarring with an irreversible inferior defect at rest and at exercise. On admission, the patient was free from symptoms. Transthoracic echocardiography and computer tomography examinations revealed an aberrant origin of the right coronary artery (RCA) from the left sinus of Valsalva, just below the commissure. Repair was accomplished by incision into the RCA and patch plasty. An anomalous aortic origin of a coronary artery from the incorrect coronary sinus of Valsalva is a rare congenital cardiac defect that is associated with an increased risk of sudden death and cardiac morbidity; however, ischemia can occur even when the patient remains asymptomatic. Although this anomalous artery often shares a common orifice with the other coronary artery, it did not in the reported case. In conclusion, because of the high risk of sudden cardiac death, aggressive surgical management is indicated in symptomatic patients.

[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.