title: Portopulmonary Hypertension in Liver Disease Presenting in Childhood.
abstract: OBJECTIVE
  Portopulmonary hypertension (POPH) is a known complication of cirrhosis in adults, but there is little information on its incidence and outcome in children with liver disease. We report 14 patients with POPH and present a synthesis of the medical literature.
  
  
  METHODS
  Diagnosis of POPH in the 14 patients was based on right-sided heart catheterization displaying mean pulmonary artery pressure (mPAP) >25 mmHg, indexed pulmonary vascular resistances >3 Wood units · m, and pulmonary wedge pressure <15 mmHg. A literature review added 84 patients.
  
  
  RESULTS
  In our unit, POPH was found in 0.5% of the children with portal hypertension, 0.9% of the children with end-stage liver disease awaiting transplantation, and 3 children with congenital portosystemic shunts (CPSSs). Analysis of 98 reported patients, including the 14 presented here, showed the cause of liver disease to be chronic liver disease or portal cavernoma in 76 instances (34 with a history of surgical portosystemic shunt) and CPSS in 22 instances. There was a precession with proven hypoxemia caused by hepatopulmonary syndrome in 6 patients. Median survival was 3 months in 56 untreated patients. An 80% 5-year probability of survival in 42 patients was treated by CPSS closure, pulmonary vasodilators, and/or liver transplantation. Mean pretransplant mPAP was 34 and 49 mmHg in transplant survivors and nonsurvivors, respectively.
  
  
  CONCLUSIONS
  POPH is a rare but extremely severe complication of childhood liver disease. Portosystemic shunts, whether congenital or acquired, likely play an important causative role. Early diagnosis is crucial and requires systematic screening by echocardiography in children at risk.
authors.affiliation: *Hépatologie Pédiatrique and Centre de Référence National de l'Atrésie des Voies Biliaires, Hôpital Bicêtre, AP-HP and Université Paris-Sud 11, Le Kremlin-Bicêtre †Centre Marie-Lannelongue, Le Plessis Robinson ‡Cardiologie et Maladies Vasculaires, CHU Pontchaillou, Rennes §Radiologie Pédiatrique, Hôpital Bicêtre, AP-HP, Le Kremlin-Bicêtre, France.
authors.forename: Emmanuelle
authors.initials: E
authors.lastname: Ecochard-Dugelay
authors.forename: Virginie
authors.initials: V
authors.lastname: Lambert
authors.forename: Jean-Marc
authors.initials: JM
authors.lastname: Schleich
authors.forename: Mathieu
authors.initials: M
authors.lastname: Duché
authors.forename: Emmanuel
authors.initials: E
authors.lastname: Jacquemin
authors.forename: Olivier
authors.initials: O
authors.lastname: Bernard
mesh_terms: D000293:Adolescent; D000328:Adult; D006328:Cardiac Catheterization; D002648:Child; D004452:Echocardiography; D005260:Female; D020065:Hepatopulmonary Syndrome / Q000150:complications* / Q000503:physiopathology; D006801:Humans; D006975:Hypertension, Portal / Q000209:etiology / Q000503:physiopathology*; D006976:Hypertension, Pulmonary / Q000209:etiology / Q000503:physiopathology*; D008107:Liver Diseases / Q000150:complications* / Q000503:physiopathology; D008297:Male; D011169:Portal Vein / Q000002:abnormalities / Q000503:physiopathology; D011170:Portasystemic Shunt, Surgical / Q000009:adverse effects; D011652:Pulmonary Circulation / Q000502:physiology; D011669:Pulmonary Wedge Pressure; D055815:Young Adult