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

Abstract

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.

Children’s heart disease in sub-Saharan Africa: Challenging the burden of disease

“…(53,54) its incidence increasing with age. (37) The economic effects of RHD on communities are well described. (53,55,56) Without access to surgery, the costs of repeated hospitalisation are significant.…”

“…(32,33) The typical age affected is 5 to 18 years. (32,33,34) There is widespread evidence that in developing nations RHD occurs at a younger age than in developed countries, and also progresses more rapidly, though this may reflect more frequent attacks of ARF, (33,35,36,37,38,39,40,41) as without intervention, the sequence of events is predictable: after the initial infection with Lancefield group A β-haemolytic streptococci 3% of patients develop ARF approximately 19 days later. (42) At reinfection, the incidence increases sharply to more than 75% of patients.…”

Epidemiology of valvular heart diseases in Africa

“…(25) Determinants of the outcome of RHD are the age of fi rst attack of RF and the frequency and severity of recurrent attacks. (26) Cardiac involvement in the attack of RF may range from mild to severe and should be suspected on clinical grounds when a murmur (systolic or diastolic) is heard on physical examination or if there is presence of cardiomegaly or unexplained heart failure. (18) Hospital-based studies and cause of death studies across Africa are all consistent and show that RHD is the main cause of cardiac morbidity and mortality in children and young adults, (4) with heart failure being the predominant cause of morbidity and mortality, followed by other complications such as native valve infective endocarditis, systemic embolisation, pulmonary hypertension, atrial arrhythmias, or complications related to valve surgery.…”

“…(18) Hospital-based studies and cause of death studies across Africa are all consistent and show that RHD is the main cause of cardiac morbidity and mortality in children and young adults, (4) with heart failure being the predominant cause of morbidity and mortality, followed by other complications such as native valve infective endocarditis, systemic embolisation, pulmonary hypertension, atrial arrhythmias, or complications related to valve surgery. (26)(27)(28)(29)(30)(31)(32) The leading cause of heart failure in the forms of RHD typically seen in Africa affecting young individuals in their fi rst or second decades is valve regurgitation mainly of the mitral and aortic valves. (26,28) It is common clinical course for RF and RHD to result in death in these young individuals (28) and the reasons for this malignant course are not entirely clear, but may partly be related to recurrent episodes of RF with inadequate secondary prophylaxis and multivalvular involvement.…”

“…(26)(27)(28)(29)(30)(31)(32) The leading cause of heart failure in the forms of RHD typically seen in Africa affecting young individuals in their fi rst or second decades is valve regurgitation mainly of the mitral and aortic valves. (26,28) It is common clinical course for RF and RHD to result in death in these young individuals (28) and the reasons for this malignant course are not entirely clear, but may partly be related to recurrent episodes of RF with inadequate secondary prophylaxis and multivalvular involvement. (28) Delayed clinical presentation also contributes to high morbidity and mortality.…”