Congenital Heart Diseases associated with Identified Syndromes and Other Extra-Cardiac Congenital Malformations in Children in Lagos

BACKGROUND: Congenital heart diseases are commonly associated with other extra cardiac congenital malformations. OBJECTIVE: To identify congenital heart diseases associated with identified syndromes and other extra cardiac congenital malformations in children in our hospital. METHODS: A prospective descriptive study done on children with congenital malformations referred to the Lagos University Teaching Hospital, Nigeria (LUTH) for echocardiographic evaluation. A thorough 2D assessment of the chambers, septa, heart vessels and concordance of the atrium and ventricle and the great vessels was made. Echo-cardiographic data obtained included M mode direct measure-ments of dimensions of left atrium, aortic root, right ventricular outflow tract, left ventricle in diastole/systole, wall thicknesses – right ventricular wall, interventricular septum, left ventricular posterior wall. Fractional shortening was derived from M mode data. Final diagnosis of the congenital heart disease was recorded. RESULTS: A total of 101 children with congenital malformations had echocardiography studies done as part of their clinical evaluation, 15(14.9%) were neonates, 53(52.5%) infants 25(24.8%) were aged one to five years and 8(7.9%) were above five years of age. Recognised syndromes were seen in 69(68%) cases. Down syndrome with 54 children contributed 78.3% of those with known syndromes. Other identified syndromes and associations were Marfan’s, Noonan’s, Edwards, Prune Belly, Apert, Ellis-van creveld syndrome and congenital rubella syndrome. Congenital heart diseases were detected in 73(72.3%) patients while 28(27.7%) had no heart defect. The commonest identified congenital heart disease was ventricular septal defect affecting 30 (29.7%) patients.