RESUMEN
PURPOSE: The prevalence of congenital heart anomalies is known to be higher in patients with clefts of the lip and/or palate(CL/P). The purpose of this study was to determine the prevalence and type of congenital heart anomalies in patients with CL/P. METHODS: We investigated congenital heart anomalies in 756 patients presented with CL/P from January 1986 to December 1997 by reviewing their clinical records. RESULTS: The prevalence rate of congenital heart anomalies in patients with CL/P was 4.2% (32 of 756). Congenital heart anomalies in those were ventricular septal defect (15 of 32), atrial septal defect (4 of 32), tetralogy of Fallot (3 of 32), patent ductus arteriosus (2 of 32), double outlet right ventricle(2 of 32), pulmonary stenosis (1 of 32), transposition of the great arteries (1 of 32), pulmonary atresia (1 of 32), coarctation of aorta (1 of 32), anomalous systemic venous drainage (1 of 32), and aortic aneurysm with patent ductus arteriosus (1 of 32). It was significant that the prevalence rate of congenital heart anomalies in cleft palate with or without cleft lip (CP+/-L) was 6.8% (30 of 442), because the prevalence rate of congenital heart anomalies in cleft lip alone was not higher than in normal population (0.6%; 2 of 314). Of the 30 patients with congenital heart anomalies, 12 patients (40 %) had conotruncal defects. CONCLUSION: The prevalence of congenital heart anomalies in patients with CP+/-L was much higher than normal population. Cardiac defects were predominantly conotruncal. Predominance of conotruncal defects among congenital heart anomalies in those was associated with abnormalities of neural crest cell proliferation and migration developing into conotruncus and palate.