Hydatid disease remains a problem in endemic areas. The combination of both intracardiac and pulmonary hydatid disease in association with chronic pulmonary thromboembolism is a rare presentation. We report a case of a 14-year-old girl with hydatid disease of the lungs, right ventricle, and pulmonary arteries, presenting as chronic pulmonary thromboembolism. She underwent surgery for wedge resection of the pulmonary hydatid in the right lower lobe, removal of the right ventricular hydatid (under cardiopulmonary bypass), and pulmonary endarterectomy (under total circulatory arrest). Her postoperative recovery was uneventful.
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