Linear insertion of atrioventricular valves in children with and without Down's syndrome

Abstract

OBJECTIVE : Study various parameters encountered in the linear insertion of the atrioventricular valves (LIAVV) in patients with and without Down's syndrome (DS).; MATERIAL: A group of patients (n = 6240) aged from 0 to 16 years with and without congenital heart disease (CHD) registered over 6 years (1.07.2005-30.06.2011) in the National Registry of the Pediatric and Congenital Heart Disease of the Society of Cardiology in Lebanon.; METHOD : Children were divided in two groups; Group I : children with DS and group II : children without DS. In the two groups, the frequency of LIAVV whether isolated or associated with other CHD (excluding the atrioventricular canal), the distribution boy-girl and the various associated cardiac anomalies were studied. We used nonparametric tests for comparing the two groups. The p value < 0.05 was considered significant.; CONCLUSION : Encountered predominantly among a male population, the isolated LIAVV is less frequent in children without DS but often with family history of Down's syndrom.; RESULTS : Group I consists of 155 patients including 31 (20%) with LIAVV, isolated in 22 cases (14.2%) and associated with CHD in 9 cases (5.8%). Group II was composed of 5995 patients; the incidence of LIAVVwas 0.2% (14 patients) with a p value < 0.00001. Regarding sex, there was a marked male predominance : respectively 78% and 56% in groups I and II. In patients with isolated LIAVV, 86% of patients were males. Family history of DS is found in 3/7 children without T21 with isolated LIAVV. A patient, in whom an atrial septal defect ostium primum type with LIAVV was diagnosed during the first month of life, has seen his septal defect closed spontaneously five months later.