Hepatopulmonary Syndrome in an Adolescent With Insidious Hypoxia and Small Intrahepatic Portal Venous Shunts: Posttransplant Benefit From Sildenafil

Pediatr Dev Pathol. 2020 Nov-Dec;23(6):467-471. doi: 10.1177/1093526620945951. Epub 2020 Aug 19.

Abstract

We report a patient without known preexisting liver disease who presented with hepatopulmonary syndrome (HPS) due to aberrant intrahepatic portal venous development leading to portosystemic shunting. Liver transplantation resulted in resolution of portal hypertension and HPS and sildenafil was safely tolerated in the treatment of persistent fatigue and hypoxemia. Twelve months later, patient has normal allograft function and has returned to normal activity.

Keywords: arteriovenous malformations; congenital portosystemic shunt; liver; pediatrics; sildenafil; transplantation.

Publication types

  • Case Reports

MeSH terms

  • Child
  • Fatigue / drug therapy
  • Fatigue / etiology
  • Hepatopulmonary Syndrome / diagnosis*
  • Hepatopulmonary Syndrome / etiology
  • Hepatopulmonary Syndrome / physiopathology
  • Hepatopulmonary Syndrome / surgery
  • Humans
  • Hypoxia / drug therapy*
  • Hypoxia / etiology
  • Liver Transplantation*
  • Male
  • Portal Vein / abnormalities
  • Postoperative Care / methods
  • Postoperative Complications / drug therapy*
  • Sildenafil Citrate / therapeutic use*
  • Vascular Malformations / diagnosis*
  • Vascular Malformations / physiopathology
  • Vascular Malformations / surgery
  • Vasodilator Agents / therapeutic use*

Substances

  • Vasodilator Agents
  • Sildenafil Citrate