Orthotopic Heart Transplantation in a Child With Hereditary Spherocytosis

Hereditary spherocytosis (HS) is a genetic, frequently familial hemolytic blood disease that presents with varying degrees of hemolytic anemia, splenomegaly, and jaundice. The disease arises as a result of defects in any of a number of proteins responsible for maintaining the shape and flexibility of the red blood cell, resulting in an osmotically fragile and characteristically spherical red blood cell. Theoretically, cardiopulmonary bypass can exacerbate hemolysis and subsequent renal dysfunction. There are few reports of open heart surgery for patients with HS and none for orthotopic heart transplantation. We report a 6-year-old boy with HS who underwent orthotopic heart transplantation.