Characteristics and management of cleft mitral valve.
OBJECTIVE
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.
Fraisse
Alain
A
Service de Cardiologie Pédiatrique et de Chirurgie Thoracique et Cardiovasculaire, Hôpital de la Timone, Marseille, France. afraisse@mail.ap-hm.fr
Massih
Tony Abdel
TA
Kreitmann
Bernard
B
Metras
Dominique
D
Vouhé
Pascal
P
Sidi
Daniel
D
Bonnet
Damien
D
D002648:Child; D002675:Child, Preschool; D004452:Echocardiography; D005500:Follow-Up Studies; D006330:Heart Defects, Congenital; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008943:Mitral Valve / Q000002:abnormalities* / Q000000981:diagnostic imaging / Q000473:pathology / Q000601:surgery; D008944:Mitral Valve Insufficiency / Q000209:etiology; D011662:Pulmonary Subvalvular Stenosis / Q000209:etiology; D016896:Treatment Outcome