Heterotaxy syndrome at adult age: A case report

Abstract

Heterotaxy syndrome, also known as Ivemark syndrome, is an uncommon disease characterized by cardiac vascular malformations, congenital asplenia or polysplenia, and abnormal arrangement of the chest and abdominal organs. We reported here, a young adult scheduled for emergency surgery due to right femoral shaft fracture. Surgery was postponed in suspicion of possible delayed intracranial hemorrhage (DICH). Computer tomorgraphy (CT) and cardiac catheterization revealed total occlusion of the Blalock-Taussig (B-T) shunt. The pathogenesis, clinical features, and management for patients with heterotaxy syndrome are discussed.