EPIDEMIOLOGY OF CONGENITAL HEART DISEASE IN CROATIA – MULTICENTRIC NATIONAL STUDY, 1995–2000

Autori:

Nataša Rojnić Putarek, Ivan Malčić

Sažetak

Sažetak. Prirođene srčane greške (PSG) raznolika su skupina bolesti koje se pojavljuju u 0,55 do 1% živorođene djece. Tendencija razvrstavanja PSG-a prema Clarkovoj patogenetskoj klasifikaciji zasniva se na pretpostavci da relativno malo razvojnih mehanizama uzrokuje širok spektar fenotipskih oblika grešaka. Ciljevi ovog rada su razvrstati PSG prema patogenetskoj klasifikaciji bolesti te procijeniti stope prevalencije PSG-a u populaciji Republike Hrvatske (RH). U retrospektivnu epidemiološku studiju uključena su djeca s PSG-om rođena od 1995. do 2000. godine i kontrolirana u centrima pedijatrijske kardiologije. U studiju je uključeno 276.565 živorođene djece, od kojih je 2.204 imalo srčanu grešku, 1.126 dječaka i 1.078 djevojčica. Ukupna prevalencija iznosila je 8,0%o. Zabilježene su statistički značajne razlike u prevalenciji PSG-a među pojedinim županijama. Prevalencija glavnih patogenetskih skupina PSG-a nije se mijenjala tijekom vremena, osim anomalija apoptoze, što se očitovalo dvostrukim povećanjem prevalencije muskulamoga ventrikulamog septalnog defekta W SD; 0,6%o do 1,3%o). Od pojedinačnih dijagnoza PSG-a najzastupljeniji je bio perimembranozni VSD (19,0%), a zatim atrijalni septalni defekt tipa ostium secundum (ASD II; 14,4%), muskulami VSD (11,1%) te pulmonalna stenoza (PS; 8,5%). Ove su četiri dijagnoze činile više od 50% svih dijagnoza PSG-a. Prevalencija PSG-a i dijagnostičke mogućnosti ne razlikuju se od onih objavljenih u literaturi. Ipak, razlike u prevalenciji ovih bolesti među županijama upućuju na potrebu daljnjeg istraživanja razvoja i etiologije ovih bolesti.

Summary

Summary. Congenital heart diseases (CHD) comprise a group of different conditions seen in 0.55–1% of live births. Tendency to classify CHD according to Clark’s pathogenic classification is based on the assumption that relatively few pathogenic mechanisms cause a wide spectrum of phenotypic forms of CHD. The aim of this study was to classify CHD according to Clark’s classification and to calculate the prevalence rates of CHD in the Croatian population. We formed the Registry including all children with CHD born between 1995 and 2000 treated in paediatric cardiology departments in Croatia. In the study were enrolled 276,565 live births, 2,204 of them with CHD, 1.126 males and 1.078 females. Total birth prevalence was 8.0‰. A statistically significant difference was found between CHD prevalence rates in several counties. The prevalence of all main pathogenic groups of CHD was constant in time, except for cell death defects where a twofold increase in the prevalence of muscular ventricular septal defect (VSD) was noted (0.6‰–1.3‰). The most common diagnosis was perimembranous VSD (19.0%), followed by atrial septal defect (ASD) type II (14.4%), muscular VSD (11.1%) and pulmonary stenosis (8.5%). This four diagnoses were composing more than 50% of all CHD diagnoses. Prevalence and diagnostic possibilities weren’t different from those presented in the literature. However, differences in prevalence between the counties warrant further investigation into the development and aetiology of CHD.

Volumen: 9-10, 2003

Liječ Vjesn 2003;125:232–241