Clinical features and management of isolated cleft mitral valve in childhood

OBJECTIVES We reviewed an institutional experience of isolated cleft mitral valve (ICMV), its clinical features, and management in a pediatric populat ion. BACKGROUND As ICMV is relatively uncommon, earlier reports highlighted its anatomical and echocardiographic features. Few studies have collated their clinical features with their outcome. METHODS All patients with ICMV were retrospectively reviewed. Patients who were considered to have an atrioventricular septal defect or variant were e xcluded. RESULTS Twenty patients (9 male, 11 female) were diagnosed with ICMV. Seven patients had associated cardiac lesions. The median age of diagnosis was 5 .2 years (range 0.4 to 13.6 years). Echocardiography aided by color Doppler demonstrated the ICMV in all patients. however, an incomplete diagnosis wa s made in 4 of 20 patients before surgery. The severity of the mitral regur gitation (MR) at presentation was mild in 11, moderate in 8, and severe in 1 patient. In the 13 patients without associated cardiac lesions, 5 underwe nt mitral valve (MV) repair at median age of 5.2 years (range 1.2 to 7.7 ye ars) for moderate to severe MR, 4 being symptomatic. The severity of the MR in seven of the eight unoperated patients has remained unchanged over the follow-up period (median 8.3 years, range 0.7 to 14.4 years). In total, 10 patients underwent MV repair (median 6.4, range 0.4 to 13.8 years). No pati ent required MV replacement. None of the 10 patients had more than mild MR over the follow-up period (median 0.6, range 0.2 to 11.0 years). CONCLUSIONS Now readily diagnosable by echocardiography, ICMV is a correcta ble cause of MR with a good outcome. Surgery is indicated in those patients with moderate to severe MR and probably should be done early following dia gnosis. (C) 2000 by the American College of Cardiology.