title: Repair of congenital heart lesions combined with lung transplantation for the treatment of severe pulmonary hypertension: a 13-year experience.
abstract: OBJECTIVE
  In patients with severe pulmonary hypertension associated with congenital heart disease, we prefer to perform repair of the congenital heart disease and lung transplantation whenever feasible so as to augment the donor pool and avoid the cardiac complications associated with heart transplantation. We report our experience with repair of congenital heart disease and lung transplantation and compare the results with those of patients who underwent heart-lung transplantation during the same period.
  
  
  METHODS
  The records of patients who had repair of congenital heart disease and lung transplantation (n = 35) and heart-lung transplantation (n = 16) between 1990 and 2003 were reviewed.
  
  
  RESULTS
  The underlying congenital heart disease in the repair of congenital heart disease and lung transplantation group included transposition of great vessels (n = 2), atrioventricular canal defect (n = 2), ventricular septal defect (n = 9), pulmonary venous obstruction (n = 7), scimitar syndrome (n = 2), pulmonary arterial atresia or stenosis (n = 5), and others (n = 8). Thirteen of the patients undergoing repair of congenital heart disease and lung transplantation (37.1%) had the congenital heart disease repaired before lung transplantation; the remaining congenital heart disease repairs were performed concurrently with transplantation. Sixteen patients underwent heart-lung transplantation because of poor left ventricular function or single-ventricle anatomy. Freedoms from bronchiolitis obliterans at 1, 3, and 5 years were 72.9%, 54.7%, and 54.7% for the repair of congenital heart disease and lung transplantation group and 77.8%, 51.9%, and 38.9% for the heart-lung transplantation group, respectively. Survivals at 1, 3, and 5 years were 62.9%, 51.4%, and 51.4% for the repair of congenital heart disease and lung transplantation group and 66.5%, 66.5%, and 60% for the heart-lung transplantation group, respectively.
  
  
  CONCLUSIONS
  Repair of congenital heart disease and lung transplantation is a feasible treatment option. Long-term outcome is determined by associated complications related to lung transplantation. Despite the complexity of combined congenital heart disease repair with lung transplantation and the resulting perioperative morbidity, the patients had similar outcomes to those of patients who underwent heart-lung transplantation.
authors.affiliation: Division of Cardiothoracic Surgery, Department of Surgery, Washington University School of Medicine, St. Louis Children's Hospital, MO, USA. choongc@msnotes.wustl.edu
authors.forename: Cliff K
authors.initials: CK
authors.lastname: Choong
authors.forename: Stuart C
authors.initials: SC
authors.lastname: Sweet
authors.forename: Tracey J
authors.initials: TJ
authors.lastname: Guthrie
authors.forename: Eric N
authors.initials: EN
authors.lastname: Mendeloff
authors.forename: Fabio J
authors.initials: FJ
authors.lastname: Haddad
authors.forename: Pam
authors.initials: P
authors.lastname: Schuler
authors.forename: Maite
authors.initials: M
authors.lastname: De La Morena
authors.forename: Charles B
authors.initials: CB
authors.lastname: Huddleston
mesh_terms: D002648:Child; D002675:Child, Preschool; D015897:Comorbidity; D005260:Female; D006330:Heart Defects, Congenital / Q000453:epidemiology / Q000601:surgery*; D016041:Heart-Lung Transplantation; D006801:Humans; D006976:Hypertension, Pulmonary / Q000453:epidemiology / Q000601:surgery*; D007223:Infant; D016040:Lung Transplantation; D008297:Male; D012189:Retrospective Studies; D016896:Treatment Outcome