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CLINICAL STUDIES

Clinical features and management of isolated cleft mitral valve in childhood

Masamichi Tamura, MD^*, Samuel Menahem, MD, FRACP, FACC^* and Christian Brizard, MD

^* Department of Cardiology, Royal Children’s Hospital, Melbourne, Australia
Victorian Cardiac Surgical Unit, Royal Children’s Hospital, Melbourne, Australia

Manuscript received December 31, 1998; revised manuscript received September 23, 1999, accepted November 10, 1999.

Reprint requests and correspondence: Prof. Samuel Menahem, Department of Cardiology, Royal Children’s Hospital, Flemington Road, Parkville, Victoria, 3051, Australia
menahems{at}cryptic.rch.unimelb.edu.au

OBJECTIVES

We reviewed an institutional experience of isolated cleft mitral valve (ICMV), its clinical features, and management in a pediatric population.

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 excluded.

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 was made in 4 of 20 patients before surgery. The severity of the mitral regurgitation (MR) at presentation was mild in 11, moderate in 8, and severe in 1 patient. In the 13 patients without associated cardiac lesions, 5 underwent mitral valve (MV) repair at median age of 5.2 years (range 1.2 to 7.7 years) 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 patient 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 correctable 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 diagnosis.

Abbreviations and Acronyms   AVSD = atrioventricular septal defect   ICMV = isolated cleft mitral valve   LA = left atrium   MR = mitral regurgitation   MV = mitral valve   TOE = transesophageal echocardiography   VSD = ventricular septal defect

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