title: Evidence of pulmonary vascular disease after heart transplantation for Fontan circulation failure.
abstract: OBJECTIVE
  Elevated pulmonary vascular resistance may contribute to late Fontan circulation failure but is difficult to assess in such patients. Our aims were to assess outcomes of patients with failed Fontan circulation after heart transplantation and to determine whether elevated pulmonary vascular resistance might have contributed to the failure.
  
  
  METHODS
  Fifteen patients (14 Fontan circulations, 1 Kawashima circulation) underwent transplantation. The most common indication was ventricular dysfunction (mean ventricular end-diastolic pressure 12.5 mm Hg). Patients with early failures (n = 4) required transplantation less than 1 year after the Fontan operation. Those with late failures (n = 11) underwent transplantation at least 1 year after the Fontan operation. Mean age at transplantation was 11.6 years. Mean Fontan-transplantation interval was 7.4 years. Mean pulmonary arterial pressure, transpulmonary gradient, and pulmonary vascular resistance before and after transplantation were assessed. Paired t tests of variable differences were used to compare variables. Survival was estimated by the Kaplan-Meier method.
  
  
  RESULTS
  In-hospital mortality was 7%. There were 2 late events (1 death, 1 retransplantation) related to compliance or rejection issues. Graft survivals were 93%, 82%, and 82% at 3, 5, and 7 years, respectively. Posttransplantation pulmonary vascular resistance was elevated (>2.0 Wood units . m 2 ) in 11 of 14 survivors past initial hospitalization (mean 3.3 +/- 1.7 Wood units . m 2 ). Only patients with early Fontan failures (3 of 4) had normal posttransplantation pulmonary vascular resistance. In paired comparisons, posttransplantation transpulmonary gradient was increased by a mean of 6.8 mm Hg ( P < .0001) relative to pretransplantation value.
  
  
  CONCLUSIONS
  Outcomes after heart transplantation for failed Fontan circulation were good. Mild-to-moderate pulmonary vascular disease was evident after heart transplantation for late failure. Elevated pulmonary vascular resistance is a likely contributor to Fontan circulation failure.
authors.affiliation: Department of Surgery, University of Colorado Health Sciences Center, Children's Hospital, 1056 E. 19th Avenue, Denver, CO 80218, USA. mitchell.max@tchden.org
authors.forename: Max B
authors.initials: MB
authors.lastname: Mitchell
authors.forename: David N
authors.initials: DN
authors.lastname: Campbell
authors.forename: Dunbar
authors.initials: D
authors.lastname: Ivy
authors.forename: Mark M
authors.initials: MM
authors.lastname: Boucek
authors.forename: Henry M
authors.initials: HM
authors.lastname: Sondheimer
authors.forename: Biagio
authors.initials: B
authors.lastname: Pietra
authors.forename: Bibhuti B
authors.initials: BB
authors.lastname: Das
authors.forename: Joseph R
authors.initials: JR
authors.lastname: Coll
mesh_terms: D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D018729:Fontan Procedure; D016027:Heart Transplantation / Q000379:methods; D006801:Humans; D011183:Postoperative Complications / Q000601:surgery*; D011651:Pulmonary Artery / Q000503:physiopathology; D012086:Reoperation; D012189:Retrospective Studies; D016019:Survival Analysis; D017211:Treatment Failure; D014652:Vascular Diseases / Q000150:complications / Q000503:physiopathology* / Q000601:surgery*; D014655:Vascular Resistance / Q000502:physiology; D018754:Ventricular Dysfunction / Q000150:complications / Q000601:surgery*