Rheumatic Heart Disease and Endomyocardial Fibrosis: A Complex Novel Case of Heart Failure

Abstract

Rheumatic heart disease (RHD) and endomyocardial fibrosis (EMF) are major causes of cardiac disease in low-income countries. We present a case of a patient with mitral stenosis and restrictive cardiomyopathy, initially attributed to severe RHD, but with disease progression despite valve replacement, likely secondary to previously undiagnosed EMF.

Keywords: endomyocardial fibrosis; global cardiovascular disease; restrictive cardiomyopathy; rheumatic heart disease.

Graphical abstract

Figure 1

Transthoracic Echocardiogram From 4 Years Earlier Imaging shows rheumatic changes of the mitral valve with a doming appearance of the leaflets during diastole, biatrial enlargement with small ventricles, and a large pericardial effusion. The mean mitral diastolic gradient was 6 mm Hg. BSA = body surface area.

Figure 2

Computed Tomography Angiography 4-Chamber Long Projection Imaging following mitral valve replacement and tricuspid valve repair demonstrates obliteration of the right ventricular cavity with the classic V-shaped configuration described in endomyocardial fibrosis (yellow border). The left ventricular myocardium is relatively smooth, and there is a pericardial effusion (green border). LA = left atrium; LV = left ventricle; RA = right atrium; RV = right ventricle.

Figure 3

Cardiac Magnetic Resonance 4-Chamber Long View Post-Mitral Valve Repair Imaging reveals obliteration of the right ventricular cavity with smooth left ventricular myocardium and moderately to severely dilated atria. Perfusion scan demonstrates thin linear chronic hypoenhancement (arrows), suggestive of chronic linear thrombus or scar in the setting of endomyocardial fibrosis. This was also retroactively noted on previous cardiac magnetic resonance 4 years earlier and was the likely culprit for failure to capture a leadless pacemaker.

Figure 4

Cardiac Magnetic Resonance Right Ventricular Inflow Tract View Imaging shows a dilated right ventricular outflow tract (RVOT) with a severely obliterated and scarred right ventricular cavity (outlined in purple). RA = right atrium; SVC = superior vena cava.

Figure 5

Apical-Predominant Obliteration of the Right Ventricular Cavity Scarring is outlined in blue. Of note, the right ventricular outflow tract (RVOT) and main pulmonary artery (MPA) have increased in dilatation from 4 years earlier, suggesting worsening pulmonary hypertension secondary to endomyocardial fibrosis.