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 hyperinsulinism--novel insights into etiology, diagnosis and treatment]. / Kongenitalni hiperinzulinizam- -novosti o nastanku, dijagnosticiranju i lijecenju bolesti.

Congenital hyperinsulinism (CHI) is a major cause of persistent hypoglycemia in the neonatal and early infancy periods. Althought the disease is relatively rare with incidence of about 1:25 000-50 000 live births, the importance of the disease should not be underestimated. Namely, prompt recognition and management of patients with CHI is essential, if permanent neurological impairment is to be avoided. CHI is caused by mutations in one of the 7 genes involved in the regulation of insulin secretion in pancreatic beta-cells. It is important to introduce specific medical therapy as soon as diagnosis is established. Severe, neonatal forms of CHI are often resistant to medications, thus they require surgical procedure. The preoperative genetic testing and scintigraphy are indicated to distinguish histological subtypes of the disease (focal vs. diffuse CHI). Patients with focal disease are usually cured after pancreatic resection, while diffuse disease has much worse prognosis. This manuscript offers novel insights into CHI and emphasizes the role of early diagnosis as crucial for succesful treatment that was recently enriched with novel options.

[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.

Magnetic resonance findings in a neonate with nonketotic hyperglycinemia: case report.

We present brain imaging and spectroscopy data in a neonate with a confirmed classic form of nonketotic hyperglycinemia (NKH), an autosomal-recessive metabolic disorder characterized by accumulation of glycine. To our knowledge, this is the first report of such complete analysis of the changes seen on conventional magnetic resonance imaging, diffusion-weighted imaging, and magnetic resonance spectroscopy at such an early age. The findings in a neonate are consistent with reports in older children with NKH, confirming that pathological changes typical for NKH can be seen in the first postnatal week.

[Transluminal balloon valvuloplasty in neonates and infants with critical aortic stenosis]. / Transluminalna balonska valvuloplastika u novorodencadi i dojencadi s kriticnom aortnom stenozom.

The purpose of this study was to evaluate children who underwent balloon valvuloplasty due to critical aortic stenosis following clinical (low cardiac output, cardiogenic shock, congestive heart failure) and echocardiographic criteria (morphological evidence of left ventricular hypertrophy, with depression of left ventricular function, irrespective of transvalvular gradient). We assessed the effectiveness of balloon valvuloplasty in 5 children (all male) who were submitted to aortic valve balloon dilatation over 3.5 years (10.1998-05.2003). The age at dilatation was 29+/-24 days, BW 3.92+/-0.82 kg and BSA 0.24+/-0.03 m2. In all children the balloon valvuloplasty was performed with manual inflation of balloon at 4-6 bars through the femoral artery. The mean systolic pressure gradient across the aortic valve decreased from 68+/-20.5 mmHg to 9+/-10.95 mmHg, i.e. by 85%, (p<0.01). Aortic valve ring diameter was 9.2+0.84 mm, and balloon/aortic ring ratio 0.8-0.04. The degree of aortic insufficiency immediately after the dilatation did not significantly increase. Dilatation was performed without complications. Long term results were evaluated in all patients 3.2 - 54 months after valvuloplasty and revealed the continuously increasing residual aortic valve gradient (Doppler measurement) 33+/-10.95 mmHg to be significantly lower (p<0.01) than before valvuloplasty. None of the children was showing clinical symptoms of the disease. According to echocardiographic analysis two of them developed aortic valvar insufficiency grade II, two had trivial insufficiency, one was without insufficiency. One child is an candidate for the Ross procedure in future (gradient 50 mmHg, insufficiency grade II, age 4.5 years.). Balloon valvuloplasty provides effective interventional method in the treatment of the neonates and infants with critical aortic stenosis.