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Isolated Severe Right Ventricle Endomyocardial Fibrosis in a Child: Vanishing Tropical Disease Revisited.

A 12-year-old girl of poor socioeconomic status from the northern part of India presented with insidious onset facial puffiness, pedal edema, and increasing abdominal girth of 3-year duration. During the past 6 months, she had 3 episodes of spontaneous onset fast irregular palpitations associated with transient loss of consciousness. Physical examination of the grossly malnourished child revealed severe pallor, bilateral pitting pedal edema up to the ankle, and the absence of cyanosis or clubbing. Cardiovascular system examination revealed elevated jugular venous pulse with prominent A wave, prominent precordial pulsations in the left parasternal area in the third and the fourth intercostal space, and apical impulse in the left fifth intercostal space 11 cm lateral to midsternal line with normal size and contour. Right heart border could be percussed in right parasternal area 9 cm lateral to sternal border; auscultation was unremarkable except for the presence of right ventricular (RV) third heart sound and short systolic murmur in tricuspid area. Abdominal examination revealed massive ascites with fluid thrill and hepatomegaly. To summarize, clinical impression was of a cachexic child with chronic right heart failure, massive ascites, hepatomegaly, cardiac enlargement, and ectopic pulsations in RV outflow tract area (Figure 1A). Electrocardiogram (Figure 1B) showed sinus rhythm, right-axis deviation, and qr in V1 suggesting right atrial enlargement and nonspecific ST/T changes. Chest x-ray (Figure 1C) showed massive cardiomegaly with grossly enlarged right heart border. Blood investigation showed severe anemia (hemoglobin, 6.8 g %) and eosinophilia (absolute eosinophil count of 531 per mm3). Transthoracic echocardiographic examination revealed congested inferior vena cava with partial inspiratory collapse, obliteration of apex, and …