Raising awareness for rheumatic mitral valve disease

Abstract

Rheumatic heart disease (RHD) is a major burden in low- to mid-income countries, where each year it accounts for over a million premature deaths associated with severe valve disease. Life-saving valve replacement procedures are not available to the majority of affected RHD patients, contributing to an increased risk of death in young adults and creating a devastating impact. In December 2017, a group of representatives of major cardiothoracic societies and industry, discussed the plight of the millions of patients who suffer from RHD. A comprehensive solution based on this global partnership was outlined in "The Cape Town Declaration on Access to Cardiac Surgery in the Developing World". The key challenge in controlling RHD is related to identification and removal of barriers to the translation of existing knowledge into policy, programs, and practice to provide high-quality care for patients with RHD. This review provides an overview on RHD by emphasizing the disease medical and economic burdens worldwide, risk factors, recent advance for early disease detection, and overall preventive strategies.

Figure 1. Transthoracic echocardiography from a patient with severe mitral stenosis in parasternal long-axis (A) and short-axis (B) views.

Both leaflets are thickened with a pliable anterior leaflet on the long-axis view and fusion of both commissures on the short-axis view. The anterior leaflet has been described as opening in a “hockey stick” appearance in parasternal long axis view (arrow). Transthoracic echocardiography from a patient with severe mitral regurgitation, apical four-chamber view. A coaptation defect of mitral valve can be detected (arrow) with severely dilated left atrium (C). Color flow mapping showing severe mitral regurgitation (D). LA = left atrium; LV = left ventricle; Ao = aorta; RV = right ventricle MV = mitral valve area, RA = right atrium.

Figure 2. Three-dimensional transesophageal echocardiography images of rheumatic mitral stenosis.

Mitral valve seen from the left atrium (A). En face view of the mitral valve from the left ventricle (B). Rheumatic mitral stenosis with symmetric commissural thickening. Severe rheumatic mitral stenosis with significant thickening of anterolateral commissure, leading to an eccentric valve orifice (D).

Figure 3. Gross and histological pathomorphological aspects of a rheumatic mitral valve in an end state of the disease.

(A) Mitral valves are thick and stiff and show scar retraction. (B) Representative image of Picrosirius red staining showing intense collagen deposition e high degree of fibrosis. (C) Representative immunohistochemistry image for CD68+ cells evidencing a high frequency of macrophages in the inflammatory infiltrate. (D) Representative image of Hematoxylin and Eosin staining of anterior mitral valve leaflets showing presence of nodular calcification (deep purple) and intense inflammatory infiltrate.