PEDIATRICS Vol. 63 No. 1 January 1979, pp. 116-123
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Mitral Valve Prolapse Syndrome in Children With Duchenne's Progressive Muscular Dystrophy

Shyamal K. Sanyal F.A.A.P.1, Richard K. F. Leung M.D.1, Ralph C. Tierney M.D.1, Richard Gilmartin M.D.1, and Samuel Pitner M.D.1

1 Cardiopulmonary Diseases and Pediatric Neurology Services, St.Jude Childtren's Research Hospital; The University of Tennessee Center for Health Sciences; and the Department of Cardiology, Baptist Memorial Hospital, Memphis

Myocardial function was evaluated prospectively by noninvasive methods in 20 boys with clinical, biochemical, muscle biopsy, and electromyographic evideuce of Duchenne's progressive muscular dystrophy, Auscultatory evidence of a nonejection systolic click suggested mitral valve prolapse (MVP) syndrome in seven patients. Phonocardiography disclosed that the click was mid-systolic ill four patients and early in three. Echocardiographic features consistent with this diagnosis were identifled in all seven patients and in an additional four. One of these had an apical pansystolic murmur, suggestive of mitral regurgitation. whereas in the other three, prolapse of the mitral valve was ‘silent."

Echocardiographic findings included an abrupt midsystolic, posterior motion (greater than 3 mm beyond the CD line) in five patients. multiple sequence echoes in six, and posterior coaptation of the mitral valve near the left atrial wall in six. The feature most characteristic of MVP syndrome was a smooth, pansystolic, anteriorly concave (hammocklike) posterior motion deviating more than 3 mm beyond the CD line. Among the remaining nine patients who did not have echocardiographic evidence of prolapsing mitral valve, none had an early, middle or late nonejection systolic click or a heart murmur, although four patients in this group had moderate to severe scoliosis.

These observations document the occurrence of MVP syndrome in children with Duchenne's muscular dystrophy and indicate that its prevalence is high. We speculate that prolapse of the mitral valve in these patients is an expression of the underlying cardiomyopathy characteristic of Duchenne's muscular dystrophy rather than an isolated, dystrophic involvement of the mitral valve leaflets.

Submitted on February 14, 1978
Accepted on April 24, 1978




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