Tropical Endomyocardial Fibrosis

Abstract

Tropical endomyocardial fibrosis is a common cause of restrictive cardiomyopathy worldwide, but is relatively rare in developed countries. We present a case of tropical endomyocardial fibrosis with right ventricular involvement initially mistaken as Ebstein's anomaly. We highlight the need for timely and accurate diagnosis to ensure appropriate management. (Level of Difficulty: Intermediate.).

Keywords: CMR, cardiac magnetic resonance; EMF, endomyocardial fibrosis; LGE, late gadolinium enhancement; RV, right ventricular; TTE, transthoracic echocardiogram; cardiac magnetic resonance; cardiomyopathy; fibrosis; restrictive; right ventricle.

Graphical abstract

Figure 1

Transthoracic Echocardiogram Images (A) Transthoracic echocardiogram without echo-enhancing contrast demonstrates right atrial dilation and right ventricular apical hypertrophy. (B) Contrast-enhanced echocardiography demonstrates perfusion of hypertrophied regions, suggesting vascularity.

Figure 2

Cardiac Magnetic Resonance Images (A) Black-blood fat-suppression phase imaging demonstrates signal intensity of the right ventricular apex similar to that of myocardium. (B) Late gadolinium enhancement phase imaging demonstrates diffuse patchy enhancement of the hypertrophied right ventricular apex, suggesting fibrosis.