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CLEFT OF THE MITRAL VALVE IN PATIENTS WITH DOWN'S SYNDROME

Abstract
Differentiation between a cleft of the mitral valve and the cleft of the left side of an atrioventricular septal defect – a lesion commonly found in patients with Down's syndrome - is surgically important since the distribution of the conduction tissue varies between the 2 lesions. In 5 patients with Down's syndrome and cleft of the mitral valve followed in our institution, Echocardiography showed a cleft dividing the anterior (aortic) leaflet of mitral valve with normal papillary muscle position, mural leaflet size, and ratio of the inlet/outlet dimension of the left ventricle. Associated cardiac lesions were present in all 5 patients: perimembranous ventricular septal defect in 3, Ostium secundum atrial septal defect in 2 and patent ductus arteriosus in 2 patients. During the 5.6 years (0.2 to 11) of the follow-up period, surgical repair of the cleft was not indicated since the mitral regurgitation through the cleft remained mild or absent in all the patients. In conclusion, a cleft of the mitral valve has important morphologic differences with atrioventricular septal defect and may occur in patients with Down's syndrome. Functional consequences of the cleft are often minor, although surgical repair may be indicated electively in older patients or if the mitral regurgitation worsen.

Key words
Mitral valve disease, cleft, Down´s syndrome

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