Congenital heart diseases associated with identified syndromes and other extra-cardiac congenital malformations in children in Lagos.

ABSTRACT

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 measurements 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. CONCLUSION: Congenital heart diseases are common in children with congenital malformations. Down syndrome was the most common malformation and the congenital heart disease most associated with the congenital malformations was ventricular septal defect. This study emphasizes the need for cardiac assessment of children with congenital malformations.