Extracardiac defects in children with congenital heart disease.

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Abstract
Extracardiac defects were found in 66 (13%) of 513 Nigerian children with congenital heart disease. As a groop these 66 children were significantly younger than the remaining 447 children who had congenital heart disease without extracardiac defects. Of the extracardiac defects, 27% occurred in the musculoskeletal system, 23% in the gastrointestinal tract and 7.5% in the CNS. In addition, 15 (23%) and 8 (12%) of the 66 children had congenital rubella and Down''s syndrome, respectively. With the exception of the well-recognized association between Down''s syndrome and endocardial cushion defect, and between congenital rubella syndrome and persistent ductus arteriosus no extracardiac defects appeared to be predictably associated with any specific form of congenital heart disease. The extracardiac defects caused the death of 12% of the patients in the newborn period; and 17 (52%) of 33 surviving patients have mental retardation. Extracardiac defects should be carefully looked for in children with congenital heart disease because early detection and treatment of the non-cardiac defects could improve the overall prognosis. Where facilities for cardiac surgery are limited priority for corrective surgery should be given to patients who have no mental handicaps since they are more likely to derive maximum benefits from the expensive surgical procedures.