title: Heart valve surgery in patients with homozygous sickle cell disease: A management strategy.
abstract: BACKGROUND
  Patients with the homozygous sickle cell disease have increased perioperative mortality. Some indications like heart valve surgery, may justify an exchange blood transfusion to reduce the proportion of hemoglobin S (HbS) and complications.
  
  
  METHODS
  We report two female cases aged 20 and 27, of African origin with homozygous sickle cell anemia who underwent heart valve surgery to treat mitral valve regurgitation. This presentation describes the perioperative considerations including anesthesia and postoperative care.
  
  
  RESULTS
  A partial exchange blood transfusion decreased HbS levels from respectively, 90% and 84%, 9% to 27% and 34%, and simultaneously treated the anemia. Neither sickling crisis nor acidosis occurred in any patient, and no special postoperative complication occurred. Average hospital stay was 10 days. Currently, the two patients remain alive and free of cardiac symptoms.
  
  
  CONCLUSIONS
  Although the presence of sickle cell disorders is associated with increased risk of sickling and thus vaso-occlusive complications, they should not be taken as a contraindication for heart valve surgery. Nevertheless, monitoring of certain parameters such as venous, arterial oxygen content, pH, and body temperature is mandatory for a better outcome. Furthermore, preoperative exchange transfusion has a positive influence on the outcome of surgery and on the survival of patients undergoing heart valves surgery. Avoiding intraoperative hypoxia, hypothermia, and vaso-constrictive agents, minimizing HbS levels with preoperative exchange transfusion, and ensuring a stress-free environment with the judicious use of sedatives made surgery relatively safe in these cases.
authors.affiliation: Department of Cardiac Surgery, Cheikh Zaid University Hospital, Rabat, Morocco.
authors.forename: El Mehdi
authors.initials: el M
authors.lastname: Moutaouekkil
authors.forename: Abdelmalek
authors.initials: A
authors.lastname: Najib
authors.forename: Rida
authors.initials: R
authors.lastname: Ajaja
authors.forename: Moha
authors.initials: M
authors.lastname: Arji
authors.forename: Anas
authors.initials: A
authors.lastname: Slaoui
mesh_terms: D000328:Adult; D000755:Anemia, Sickle Cell / Q000601:surgery*; D000758:Anesthesia; D002315:Cardiopulmonary Bypass; D005078:Exchange Transfusion, Whole Blood; D005260:Female; D006801:Humans; D007902:Length of Stay; D008943:Mitral Valve / Q000601:surgery*; D008944:Mitral Valve Insufficiency / Q000601:surgery*; D011183:Postoperative Complications / Q000517:prevention & control; D055815:Young Adult