title: Heart transplantation in the setting of complex congenital heart disease and physiologic single lung.
abstract: OBJECTIVE
  To highlight the success of heart transplantation in patients with complex congenital heart disease and physiologic single lung by providing an update on the world's largest reported cohort.
  
  
  METHODS
  Demographic, perioperative, postoperative, and outcomes data were collected retrospectively on all patients undergoing heart transplant to single lung at Columbia University Medical Center since 1992, and compared with all other patients undergoing transplants performed for single ventricle or tetralogy of Fallot during that time.
  
  
  RESULTS
  Twenty-two patients (mean age, 20.6 years; range, 5 months-47 years) underwent heart transplant to single lung. Compared with controls (n = 67), the single lung group had more male patients and a greater proportion of tetralogy compared with single ventricle patients, although the single lung group had fewer post-Fontan patients. Age, weight, and body surface area were similar between the groups as were use of mechanical circulatory support and mechanical ventilation before transplant. Median time to extubation, time on inotropes, and length of stay were similar. There were 3 perioperative deaths, including a patient who died during postoperative day 1 from primary graft failure, likely related to a combination of elevated pulmonary vascular resistance and volume load. There were 5 additional mortalities during intermediate- and long-term follow-up, none of which were related to single-lung physiology. There was no significant survival difference between the groups.
  
  
  CONCLUSIONS
  In patients with complex congenital heart disease and single lung physiology, heart transplant alone remains an excellent option, with comparable outcomes to patients undergoing transplant with similar cardiac anatomy and dual lung physiology.
authors.affiliation: Division of Pediatric Cardiology, Columbia University Medical Center, New York, NY. Electronic address: wz2116@columbia.edu.
authors.forename: Warren A
authors.initials: WA
authors.lastname: Zuckerman
authors.affiliation: Division of Pediatric Cardiology, Columbia University Medical Center, New York, NY.
authors.forename: Marc E
authors.initials: ME
authors.lastname: Richmond
authors.affiliation: Division of Pediatric Cardiology, Columbia University Medical Center, New York, NY.
authors.forename: Teresa M
authors.initials: TM
authors.lastname: Lee
authors.affiliation: Department of Pediatric Cardiothoracic Surgery, Columbia University Medical Center, New York, NY.
authors.forename: Emile A
authors.initials: EA
authors.lastname: Bacha
authors.affiliation: Department of Pediatric Cardiothoracic Surgery, Columbia University Medical Center, New York, NY.
authors.forename: Paul J
authors.initials: PJ
authors.lastname: Chai
authors.affiliation: Department of Pediatric Cardiothoracic Surgery, Seattle Children's Hospital, Seattle, Wash.
authors.forename: Jonathan M
authors.initials: JM
authors.lastname: Chen
authors.affiliation: Division of Pediatric Cardiology, Columbia University Medical Center, New York, NY.
authors.forename: Linda J
authors.initials: LJ
authors.lastname: Addonizio
mesh_terms: D000293:Adolescent; D000328:Adult; D002648:Child; D002675:Child, Preschool; D003710:Demography; D005260:Female; D006330:Heart Defects, Congenital / Q000601:surgery*; D016027:Heart Transplantation; D006801:Humans; D007223:Infant; D008168:Lung / Q000002:abnormalities*; D008297:Male; D008875:Middle Aged; D012189:Retrospective Studies; D016896:Treatment Outcome
keywords: congenital heart disease; heart transplantation; single lung