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Situs inversus totalis is a rare anatomic condition characterized by the mirror-imaged arrangement of asymmetric thoracic and abdominal organs. Although associated cardiopulmonary disease is uncommon, end-stage lung disease can develop in patients with situs inversus, necessitating transplantation. In this report, we describe a 30-year-old patient with situs inversus totalis and end-stage pulmonary hypertension who underwent successful orthotopic left lung transplantation.
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