Endomyocardial fibrosis in a 12-year-old boy

Endomyocardial fibrosis (EMF) is characterized by the abnormal scarring of the endocardium with deposition of fibrous tissues in the apices and inflow tracts of the right ventricle, left ventricle, or both, leading to symptoms such as heart failure, arrhythmias, and intracardiac thrombosis. EMF is a rare cause of cardiac morbidity and mortality in children of the tropics including Nigeria, hence the presentation of this case report. A 12-year-old male presented to Children emergency ward with abdominal swelling from infancy, which worsened 3 months before presentation, leg swelling and weight loss of 3 months, cough of 1 month, and fast breathing of 1 week. Physical examination revealed dyspnea, pedal and leg edema, absent breath sounds in the right middle and lower lung zone, tachycardia, irregular pulse, elevated jugular venous pressure, and a grossly distended abdomen. Echocardiography demonstrated dilated atria, thickened AV valves, small, thickened ventricular cavities, intra-atrial thrombus, fibrous tissue in the apices and inlets of left and right ventricles, and pericardial effusion. Electrocardiography revealed first-degree AV block, left anterior fascicular block, and left atrial enlargement. Chest radiograph revealed right lobar pneumonia, pleural effusion, and cardiomegaly. The abdominopelvic scan revealed congestive hepatomegaly and ascites. He received diuretics, beta-blockers, aspirin, and antibiotics. Repeated abdominal paracentesis, thoracocentesis, and pericardiocentesis drained 14.2 L, 1.5 L, and 2.1 L of serosanguineous fluid, respectively. EMF is an enigmatic disease which requires further research to unravel its underlying mechanisms and develop more targeted therapeutic strategies.