Pattern of congenital heart defects in children with Down syndrome at the University of Port Harcourt Teaching Hospital, Port Harcourt

Down syndrome (DS) is the most common chromosomal abnormality in man and congenital heart defects (CHD) the most life threatening of its manifestations. The cardiac anomalies and early pulmonary hypertension are associated with high morbidity and mortality. It is thus important to diagnose and intervene early to improve the quality of life. Few studies have been done on Down syndrome patients in Nigeria. Objective: The objective was to find out the incidence and types of congenital heart defects in Down syndrome patients seen in the Paediatric cardiology unit of this tertiary institution. Method: A prospective study of Down Syndrome patients seen at the Paediatric Cardiology clinic of the University of Port Harcourt Teaching Hospital over a three year period was carried out. Data of age, sex, birth order, mother’s age, indication for surgery, clinical and echocardiographic findings and outcome was analysed. Result: Of the 31 patients who had echocardiography done, all had cardiac defects (100%). Patent ductus arteriosus, occurring solitarily or in combinations was the commonest cardiac defect seen followed by ventricular septal defects. Interestingly, over 60 % of the patients had multiple cardiac defects with VSD/PDA, VSD/ASD accounting for 50%. Conclusion: The incidence of multiple cardiac defects in more than 60% of DS patients seen underscores the need for early diagnosis and intervention. Routine extensive cardiac evaluation in the first few weeks of life is advocated. Key word: Down syndrome, congenital heart defects, Port Harcourt, Nigeria.