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The nature and severity of congenital heart disease in 78 patients who presented with clefts of the lip and/or palate is reported. The prevalence of bilateral cleft lip and palate in patients with heart lesions was much higher than in cleft patients with normal hearts. Cardiac defects were predominantly conotruncal. Tetralogy of Fallot was present in 24 percent of patients; the prevalence of transposition, atrioventricular septal defect, and truncus arteriosus was also disproportionately high. Patients with conotruncal defects had a greater prevalence of either unilateral or bilateral cleft lip and palate. Most patients with congenital heart disease and clefting had additional abnormalities. Anomalies of other systems were found to be present in 87 percent of patients.

The surgical management of congenital mitral valve (MV) anomalies remains controversial and complex. Valve repair has been said to be preferable to valve replacement. To assess the validity of this suggestion we have reviewed our experience of these procedures at The Hospital for Sick Children, Great Ormond Street, London from 1969-1983. In that time 48 patients have undergone surgery; in 23 repair was practicable (Group A) and in 25 replacement of the MV was required (Group B). Group A patients were slightly older than group B patients (5.9 +/- 1.0 years (SEM) vs 3.6 +/- 0.5 years). Björk Shiley valves were the predominant prosthesis used. Overall mortality was 33%, with 17% in Group A and 48% in Group B. Hospital mortality was 4.5% in Group A and 28% in Group B. Actuarial survival for Group A was 90% at 1 year, and 75% at 2 and 5 years; for Group B it was 52% at 1 and 2 years and 42% at 5 years. Mortality was significantly higher in those aged less than 5 years. Four patients in Group A have required re-operation, in 3 valve replacement was required; 2 of these died in hospital. One patient in Group B has required reoperation for valve-related thromboembolism. Three patients are awaiting replacement of calcified bioprostheses. These data support the concept that repair of MV should be performed where possible. The severity of the anomalies was greater in Group B and a rethink as to whether valve replacement is the best option for severe mitral stenosis in those aged less than 5 years seems indicated, in view of the very high mortality.