1606 mitral valve prolapse (mvp) syndrome in duchenne's muscular dystrophy (dmd): pre-valence, spectrum and ultrastructural basis

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. ARTICLE PDF AUTHOR INFORMATION AUTHORS AND AFFILIATIONS * St. Jude Children's Research Hospital,

Memphis, TN Shyamal K Sanyal & Warren W Johnson Authors * Shyamal K Sanyal View author publications You can also search for this author inPubMed Google Scholar * Warren W Johnson View

author publications You can also search for this author inPubMed Google Scholar RIGHTS AND PERMISSIONS Reprints and permissions ABOUT THIS ARTICLE CITE THIS ARTICLE 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 (Suppl 4), 711 (1981).

https://doi.org/10.1203/00006450-198104001-01623 Download citation * Issue Date: 01 April 1981 * DOI: https://doi.org/10.1203/00006450-198104001-01623 SHARE THIS ARTICLE Anyone you share the

following link with will be able to read this content: Get shareable link Sorry, a shareable link is not currently available for this article. Copy to clipboard Provided by the Springer

Nature SharedIt content-sharing initiative