title: Early liver transplantation is crucial in children with liver disease and pulmonary artery hypertension.
abstract: OBJECTIVE
  Early liver transplantation is crucial in children with liver disease and pulmonary artery hypertension. Some severe pulmonary vascular anomalies associated with portal hypertension disappear after isolated liver transplantation. Evolution of pulmonary artery hypertension due to plexogenic arteriopathy is controversial, as this association is still considered a contraindication to isolated liver transplantation. Outcome of pulmonary hypertension after isolated liver transplantation is reported in three patients with portal hypertension.
  
  
  METHODS
  After echocardiographic diagnosis, the patients had a complete hemodynamic exploration, and two had a lung biopsy. After liver transplantation, the survivors had echocardiographic follow up and a second hemodynamic exploration.
  
  
  RESULTS
  In two children, pulmonary pressures and resistances returned to near-normal values 1 and 6 years after successful isolated liver transplantation. The third patient, with the most severe arteriopathy, had to wait 1 year for a donor, and the attempted transplantation was complicated by ventricular tachycardia; death occurred 2 days after surgery.
  
  
  CONCLUSIONS
  Liver transplantation can reverse pulmonary artery hypertension due to high pulmonary resistances complicating liver disease with portal hypertension, provided it is carried out at an early stage. Early detection of pulmonary hypertension by systematic echocardiography may thus be crucial in these children with portal hypertension.
authors.affiliation: Unité de Cardiologie Pédiatrique, Hôpital Marie Lannelongue, Le Plessis-Robinson, France.
authors.forename: J
authors.initials: J
authors.lastname: Losay
authors.forename: D
authors.initials: D
authors.lastname: Piot
authors.forename: J
authors.initials: J
authors.lastname: Bougaran
authors.forename: Y
authors.initials: Y
authors.lastname: Ozier
authors.forename: D
authors.initials: D
authors.lastname: Devictor
authors.forename: D
authors.initials: D
authors.lastname: Houssin
authors.forename: O
authors.initials: O
authors.lastname: Bernard
mesh_terms: D000293:Adolescent; D001656:Biliary Atresia / Q000601:surgery; D002648:Child; D004452:Echocardiography; D005260:Female; D006439:Hemodynamics / Q000502:physiology; D006801:Humans; D006975:Hypertension, Portal / Q000150:complications*; D006976:Hypertension, Pulmonary / Q000150:complications*; D008107:Liver Diseases / Q000150:complications / Q000601:surgery*; D016031:Liver Transplantation; D013997:Time Factors