title: Staged single-ventricle palliation in an infant with hemoglobin SC disease.
abstract: Staged single-ventricle palliation is used to treat many cyanotic congenital heart diseases. Hemoglobin sickle cell disease is associated with anemia and significant vascular sickling sequelae, which increase the risk associated with single-ventricle palliation. To our knowledge, there are no reports in the English-language medical literature of single-ventricle palliation having been performed on a patient who had either sickle cell anemia or sickle cell-hemoglobin C disease. Herein, we discuss our clinical and surgical management of an infant with tricuspid atresia type IA and hemoglobin sickle cell disease who survived single-ventricle palliative procedures through the 2nd stage of a bidirectional Glenn anastomosis.
authors.affiliation: Department of Pediatrics, Section of Cardiology, Baylor College of Medicine and Texas Children's Hospital, Houston, Texas 77030, USA. malaw@TexasChildrensHospital.org
authors.forename: Mark A
authors.initials: MA
authors.lastname: Law
authors.forename: Zoann
authors.initials: Z
authors.lastname: Dreyer
authors.forename: Jeffrey S
authors.initials: JS
authors.lastname: Heinle
authors.forename: Heather A
authors.initials: HA
authors.lastname: Dickerson
mesh_terms: D000714:Anastomosis, Surgical / Q000379:methods; D006348:Cardiac Surgical Procedures / Q000379:methods*; D005260:Female; D005500:Follow-Up Studies; D006352:Heart Ventricles / Q000601:surgery*; D006450:Hemoglobin SC Disease / Q000150:complications*; D006801:Humans; D007231:Infant, Newborn; D010166:Palliative Care / Q000379:methods*; D018785:Tricuspid Atresia / Q000150:complications / Q000601:surgery*