Long term remission of portopulmonary hypertension with liver transplantation in a patient with cirrhosis associated to autoimmune hepatitis

Arch Argent Pediatr. 2022 Feb;120(1):e17-e20. doi: 10.5546/aap.2022.eng.e17. Epub 2022 Jan 1.
[Article in English, Spanish]

Abstract

Portopulmonary hypertension is an uncommon complication of portal hypertension, running a progressive course with a negative prognosis. Reports in pediatric patients are scarce with short follow up. We describe the case of decompensated cirrhosis who developed PoPH and resolved with liver transplantation, remaining asymptomatic after ten years of follow up.

La hipertensión portopulmonar (HTPP) es una complicación infrecuente de la hipertensión portal, que sigue un curso progresivo con un pronóstico sombrío. Los reportes en pacientes pediátricos son escasos y con períodos de seguimiento cortos. Se describe una paciente con cirrosis descompensada que desarrolló HTPP resuelta mediante trasplante hepático, que permanece asintomática tras diez años de seguimiento.

Keywords: child; liver transplantation; portal hypertension; pulmonary hypertension.

Publication types

  • Case Reports

MeSH terms

  • Child
  • Hepatitis, Autoimmune* / complications
  • Humans
  • Hypertension, Portal* / complications
  • Hypertension, Pulmonary* / etiology
  • Liver Cirrhosis / complications
  • Liver Transplantation*