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The Spectrum of Severe Rheumatic Mitral Valve Disease in a Developing Country: Correlations among Clinical Presentation, Surgical Pathologic Findings, and Hemodynamic Sequelae

Richard H. Marcus, MB; Pinhas Sareli, MD; Wendy A. Pocock, MB; and John B. Barlow, MD
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From the University of the Witwatersrand, Johannesburg, South Africa. Requests for Reprints: Richard H. Marcus, MB, FCP(SA), Noninvasive Cardiac Imaging Laboratory, The University of Chicago Medical Center, Department of Medicine/MC5084, 5841 South Maryland Avenue, Chicago, IL 60637. Acknowledgments: The authors thank Drs. D. Kitchiner and C. Ostermeyer for assistance in the collection of surgical data, Dr. D. Gretler for technical contributions, and Dr. T. Karrison for guidance on statistical analysis of data. Grant Support: In part by research grant L72 from the Chairman's Fund, Anglo American Corporation of South Africa, and by the Knocker-Bradlow Award of the College of Medicine of South Africa.

Copyright ©2004 by the American College of Physicians

Ann Intern Med. 1994;120(3):177-183. doi:10.7326/0003-4819-120-3-199402010-00001
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Objective: To describe the demographic, pathologic, and hemodynamic profiles of patients with severe rheumatic mitral valve disease in a developing country and to assess their relation to uncontrolled rheumatic disease activity.

Design: Retrospective, cross-sectional, cohort study.

Setting: Tertiary medical center in Soweto, South Africa.

Patients: 714 of 737 consecutive black patients, 4 to 73 years old, with pure mitral regurgitation, pure mitral stenosis, or mixed mitral disease who had mitral valve surgery and in whom preoperative and surgical data were concordant.

Measurements: Valve lesions were evaluated on the basis of clinical, echocardiographic, hemodynamic, and surgical pathologic data. Active rheumatic carditis was diagnosed according to clinical evidence for concurrent acute rheumatic fever (Jones criteria), macroscopic appearances at surgery, and histologic findings.

Results: 219 patients had pure mitral regurgitation, 275 had pure mitral stenosis, and 220 had mixed lesions. Ongoing rheumatic activity was diagnosed in 106 patients with pure regurgitation (47%) and in only 5 patients with pure stenosis (2%). Pure regurgitation was the most common lesion in the first and second decades; the relative prevalence of pure stenosis increased with age. Purely regurgitant valves had pliable, unscarred leaflets (95%), dilated mitral annuli (95%), elongated chordae tendineae (92%), and anterior leaflet prolapse (81%). In contrast, purely stenotic valves had fused leaflet commissures (100%) and rigid leaflets (38%) but no evidence of prolapse.

Conclusions: The spectrum of rheumatic mitral valve disease that is hemodynamically severe in developing countries differs from that currently reported in the United States. Severe, pure rheumatic mitral regurgitation is as prevalent as pure stenosis but has an entirely different time course, surgical anatomy, and relation to disease activity, suggesting a separate pathophysiologic mechanism.


Grahic Jump Location
Figure 1.
Time-course analysis (by decades) of the relative prevalence of pure mitral regurgitation, mixed mitral valve disease, and pure mitral stenosis.

MR = mitral regurgitation; MS = mitral stenosis; MV = mitral valve.

Grahic Jump Location
Grahic Jump Location
Figure 2.
Relations among age, disease activity, and valve lesion.

Histograms show the prevalence of rheumatic activity among patients presenting with severe mitral valve disease during the first three decades of life. MR = mitral regurgitation; MS = mitral stenosis; MV = mitral valve.

Grahic Jump Location




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