Tropical endomyocardial fibrosis in India: a vanishing disease!

Abstract

Tropical endomyocardial fibrosis in India was a common medical problem in the coastal districts of south India, especially the Kerala State. The clinical and autopsy studies have shown left and right ventricular apical fibrosis, with varying degree of atrioventricular valve regurgitation. Left ventricular endomyocardial fibrosis presents with severe pulmonary hypertension and right ventricular endomyocardial fibrosis presents very high systemic venous pressure and congestive cardiac failure. Surgical management improved the natural history of the disease to some extent. Various infectious and toxic factors were postulated regarding its aetiology. During the last few years, incidence of the disease has decreased considerably. The only explanation identified is the significant improvement in the living standards of the people with the corresponding decline in the childhood malnutrition, infections, worm infestation and associated eosinophilia.

Fig. 1

Geographic distribution of endemic regions of endomyocardial fibrosis. Reprinted with permission from BMJ Publishing Group Ltd.[Heart2009; 95: 9-14].

Fig. 2

The radiologic spectrum in endomyocardial fibrosis. (A) Incidental detection of endocardial calcium during coronary angiogram, (B) Massive cardiomegaly in a patient with congestive cardiac failure. Lower panel shows the fluoroscopic images of endocardial calcium in frontal (C) and lateral view (D), short arrows show, right ventricular and long arrows show left ventricular endocardial calcium. Reprinted with permission from BMJ Publishing Group Ltd. [Heart2009; 95: 9-14].

Fig. 3

Two dimensional echocardiogram of left ventricular endomyocardial fibrosis showing dense calcified fibrous tissue obliterating the cavity of the ventricular apex. LV- left ventricle, LA - left atrium and AO - aorta.

Fig. 4

Angiocardiographic features of typical endomyocardial fibrosis showing apical ventricular cavity obliteration. (A) left ventricular angiogram in right anterior oblique view and the arrow points to an apical diverticuation, and (B) shows right ventricular angiogram in shallow right anterior oblique view. Reprinted with permission from BMJ Publishing Group Ltd. [Heart2009; 95: 9-14].

Fig. 5

The clinical and echocardiographic features of right ventricular endomyocardial fibrosis. Clinical picture (E) shows the massive ascites with no pedal oedema. Echo pictures A to D show: A. apical 4 chamber view showing the fibrotic obliteration of the right ventricle (RV), with apical dimpling, grossly dilated right atrium (RA). B. M mode colour Doppler, of the pulmonary artery showing atrial systolic forward flow into pulmonary artery. C. M Mode echo showing atrial systolic opening of the pulmonary valve. D. Colour Doppler M mode echocardiogram showing the diastolic forward flow into pulmonary artery. Reprinted with permission from BMJ Publishing Group Ltd. [Heart2009; 95: 9-14].

Fig. 6

Summarizes the clinical features of the subjects seen with endomyocardial fibrosis in the tropics and the United Kingdom as reported in 1983. Reprinted with permission from bmj.com [Postgrad Med J1983; 59: 179-85].