title: Cardiopulmonary Bypass Strategy for a Cyanotic Child With Hemoglobin SC Disease.
abstract: Hemoglobin SC (HbSC) disease is a hemoglobinopathy that may produce sickling under conditions of hypoxemia, dehydration, and acidosis. We present a case of HbSC disease and tricuspid atresia, type IB. We describe management by cardiopulmonary bypass CPB using exchange transfusion at initiation of bypass and fractionation of collected blood, allowing platelet and plasma apheresis, as an option for patients unable to undergo this procedure off pump.
authors.affiliation: Department of Anesthesiology, Division of Pediatric Anesthesia, Duke University Hospital, Durham, North Carolina. Electronic address: kelly.machovec@duke.edu.
authors.forename: Kelly A
authors.initials: KA
authors.lastname: Machovec
authors.affiliation: Department of Surgery, Division of Cardiovascular and Thoracic Surgery, Duke University Hospital, Durham, North Carolina.
authors.forename: Robert D B
authors.initials: RD
authors.lastname: Jaquiss
authors.affiliation: Department of Perfusion Services, Duke University Hospital, Durham, North Carolina.
authors.forename: David D
authors.initials: DD
authors.lastname: Kaemmer
authors.affiliation: Department of Anesthesiology, Division of Pediatric Anesthesia, Duke University Hospital, Durham, North Carolina.
authors.forename: Warwick A
authors.initials: WA
authors.lastname: Ames
authors.affiliation: Department of Anesthesiology, Division of Pediatric Anesthesia, Duke University Hospital, Durham, North Carolina.
authors.forename: Hercilia M
authors.initials: HM
authors.lastname: Homi
authors.affiliation: Department of Perfusion Services, Duke University Hospital, Durham, North Carolina.
authors.forename: Richard J
authors.initials: RJ
authors.lastname: Walczak
authors.affiliation: Department of Surgery, Division of Cardiovascular and Thoracic Surgery, Duke University Hospital, Durham, North Carolina.
authors.forename: Andrew J
authors.initials: AJ
authors.lastname: Lodge
authors.affiliation: Department of Anesthesiology, Division of Pediatric Anesthesia, Duke University Hospital, Durham, North Carolina.
authors.forename: Edmund H
authors.initials: EH
authors.lastname: Jooste
mesh_terms: D000925:Anticoagulants / Q000008:administration & dosage; D001804:Blood Transfusion, Autologous; D002315:Cardiopulmonary Bypass / Q000379:methods*; D002675:Child, Preschool; D003490:Cyanosis; D005078:Exchange Transfusion, Whole Blood; D005260:Female; D018729:Fontan Procedure; D006324:Heart Arrest, Induced; D006450:Hemoglobin SC Disease / Q000150:complications*; D006493:Heparin / Q000008:administration & dosage; D006801:Humans; D007036:Hypothermia, Induced; D010166:Palliative Care; D010956:Plasmapheresis; D010983:Plateletpheresis; D011300:Preoperative Care; D018785:Tricuspid Atresia / Q000150:complications / Q000601:surgery*
chemical_list: D000925:Anticoagulants; D006493:Heparin