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CLINICAL RESEARCH: CONGENITAL HEART DISEASE

Characteristics and management of cleft mitral valve

Alain Fraisse, MD^*,*, Tony Abdel Massih, MD, Bernard Kreitmann, MD^*, Dominique Metras, MD^*, Pascal Vouhé, MD, Daniel Sidi, MD and Damien Bonnet, MD

^* Service de Cardiologie Pédiatrique et de Chirurgie Thoracique et Cardiovasculaire, Hôpital de la Timone, Marseille, France
Service de Cardiologie Pédiatrique et de Chirurgie Cardiaque, Hôpital Necker Enfants Malades, Paris, France

Manuscript received March 25, 2003; revised manuscript received June 19, 2003, accepted July 13, 2003.

^* Reprint requests and correspondence: Dr. Alain Fraisse, Service de Cardiologie A, Hôpital de la Timone, 13005 Marseille, France.
afraisse{at}mail.ap-hm.fr

OBJECTIVES: We sought to highlight the clinical, morphologic, and pathogenetic features in patients with a cleft mitral valve (MV).

BACKGROUND: Few studies have addressed the morphologic features of cleft MV and the outcome of these patients. The pathogenetic features, including the developmental relation to an atrioventricular (AV) septal defect, remain unclear.

METHODS: We reviewed the patients with cleft MV that were diagnosed by echocardiography since 1980. Patients with an AV canal, ventriculo-arterial discordance, and hypoplastic ventricles were excluded.

RESULTS: Twenty-two patients were identified at a median age of 0.5 years (range 0 to 10.6). In three patients, no chordal attachments of the cleft to the ventricular septum were seen. Ten patients had significant mitral regurgitation (MR), and three had subaortic obstruction by the cleft. Associated cardiac lesions and extracardiac features were present in 13 and 10 patients, respectively. During the median follow-up period of 1.5 years (range 0 to 11.8), two patients died of extracardiac causes, and one neonate died of severe subaortic obstruction. Surgical repair was performed in 10 patients at a median age of 5.2 years (range 1.3 to 10.6). Multivariate analysis showed no predictors for MV surgery. One patient was re-operated for mitral stenosis associated with aortic valve stenosis. Follow-up echocardiography demonstrated moderate MR in two unoperated patients and moderate MV stenosis in two operated patients.

CONCLUSIONS: A cleft of the MV comprises a wide spectrum. Important morphologic differences exist with an AV septal defect, although the two lesions may be pathogenetically related. Surgical repair always seems possible. Long-term echocardiographic follow-up is warranted.

Abbreviations and Acronyms   AV = atrioventricular   MR = mitral regurgitation   MV = mitral valve

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