title: Lung transplantation in children with idiopathic pulmonary arterial hypertension: an 18-year experience.
abstract: BACKGROUND
  The natural history of idiopathic pulmonary arterial hypertension (IPAH) in patients of all ages is one of relentless progression. For those who fail medical therapy, lung transplantation remains the ultimate palliation. In the USA, IPAH is the second leading indication for lung transplantation in children and first for children 1 to 5 years of age. In this study, we report our 18-year experience with lung transplantation in children with IPAH.
  
  
  METHODS
  We performed a retrospective chart review of children with IPAH listed for lung transplant at our center between 1991 and 2009. Our data reflect a total of 26 children ranging in age from 1.6 to 18.9 years. Nineteen were transplanted and 7 died while waiting (27%). The impact of a number of pre-transplant variables on survival was evaluated.
  
  
  RESULTS
  Median survival for those transplanted was 5.8 years, with 1- and 5-year survival rates of 95% and 61%, respectively. Survival was independent of pre-transplant considerations such as age, weight, need for intravenous (IV) inotropes, use of IV pulmonary vasodilators, year of transplant and severity of right-sided cardiac pressures. There was 1 hospital death. Compared with the transplanted group, children who died waiting had a significantly higher incidence of supra-systemic right heart pressures (p = 0.02) and hemoptysis (p = 0.01).
  
  
  CONCLUSIONS
  Our study is the largest to date to look at outcomes for lung transplantation in children with IPAH. Their median survival compares favorably with that of all pediatric lung transplant recipients, 5.8 years vs 4.5 years, respectively. We did not identify any pre-transplant variables that presaged a poorer outcome. Thus, survival seemed more related to factors that influence long-term outcomes in all transplant recipients such as rejection and infection. Lung transplantation remains a viable option for children with IPAH, especially for those with supra-systemic right heart pressures despite maximal medical therapy.
authors.affiliation: Department of Pediatrics, Washington University School of Medicine, St. Louis, Missouri 63110, USA.
authors.forename: Brian S
authors.initials: BS
authors.lastname: Goldstein
authors.forename: Stuart C
authors.initials: SC
authors.lastname: Sweet
authors.forename: Jingnan
authors.initials: J
authors.lastname: Mao
authors.forename: Charles B
authors.initials: CB
authors.lastname: Huddleston
authors.forename: R Mark
authors.initials: RM
authors.lastname: Grady
mesh_terms: D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D065627:Familial Primary Pulmonary Hypertension; D005260:Female; D006801:Humans; D006976:Hypertension, Pulmonary / Q000175:diagnosis / Q000601:surgery*; D007223:Infant; D016040:Lung Transplantation / Q000401:mortality; D008297:Male; D016016:Proportional Hazards Models; D012189:Retrospective Studies; D016896:Treatment Outcome; D055815:Young Adult