Background: An 11-year-old girl presented to a specialist cardiac facility in Mozambique. She had severe heart failure and massive cardiac enlargement, herniation of the heart into the epigastrium, atrial fibrillation, signs of severe pulmonary hypertension and a low cardiac output.
Investigations: Chest radiography, echocardiography, 24 h Holter monitoring, and cardiac catheterization.
Diagnosis: Left and right endomyocardial fibrosis in conjunction with an aneurysmal left atrium, severe heart failure, and atrial fibrillation.
Management: Left ventricular endocardiectomy with mobilization of the chordae tendineae, mitral valve repair, tricuspid annuloplasty, and left atrial resection.