1606 MITRAL VALVE PROLAPSE (MVP) SYNDROME IN DUCHENNE'S MUSCULAR DYSTROPHY (DMD): PRE-VALENCE, SPECTRUM AND ULTRASTRUCTURAL BASIS

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Abstract

The prevalence of MVP syndrome was determined in 20 boys with clinical, biochemical and electromyographic, and muscle biopsy evidence of DMD. Auscultatory evidence of a non-ejection systolic click, documented by phono, suggested MVP in 7 patients. Echocardiography confirmed MVP in these 7 and in 4 others. Pansystolic, anteriorly concave, posterior motion (> 3 mm beyond CD line) was seen in all patients, abrupt mid-systolic posterior motion in 5, and multiple sequence echoes in 6.

Hearts from 3 DMD eatients with MVP were perfused for 4 hours with 2.5% glutaraldehyde at 4°C and ultrastructure, histology and gross anatomic features of mitral valve annulus, anterior and posterior leaflets, chordae tendinae, right and left ventricular myocardium and papillary muscles were studied and compared with hearts from age and sex-matched normal controls.

Gross, histologic and ultrastructural features of mitral valve leaflets, annulus and chordae tendinae in DMD patients were entirely normal. Myocardium, by contrast, showed multifocal areas of myofiber degeneration with fibrosis and loss of contractile elements – thick and thin myofilaments – producing a “moth-eaten” appearance of myofiber. These abnormal findings predominantly involved posterior papillary muscle and posterobasal segment of left ventricle.

Our observations establish: (i) a high prevalence of MVP syndrome in children with DMD and (ii) that MVP syndrome in DMD is an expression of underlying cardiomyopathy and is not due to dystrophic changes of the mitral valve leaflets, annulus or chordae tendinae.

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Sanyal, S., Johnson, W. 1606 MITRAL VALVE PROLAPSE (MVP) SYNDROME IN DUCHENNE'S MUSCULAR DYSTROPHY (DMD): PRE-VALENCE, SPECTRUM AND ULTRASTRUCTURAL BASIS. Pediatr Res 15, 711 (1981) doi:10.1203/00006450-198104001-01623

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