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

Creator(s)

Voytek Slowik, Children's Mercy HospitalFollow
Amber Hildreth
M Cristina Pacheco
Laura S. Finn
Jeremy King
Giridhar Shivaram
Matthew Files
Evelyn K. Hsu
Simon Horslen

Document Type

Article

Publication Date

11-2020

Identifier

DOI: 10.1177/1093526620945951

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.

Journal Title

Pediatric and developmental pathology : the official journal of the Society for Pediatric Pathology and the Paediatric Pathology Society

Volume

23

Issue

6

First Page

467

Last Page

471

Keywords

rteriovenous malformations; congenital portosystemic shunt; liver; pediatrics; sildenafil; transplantation

Recommended Citation

Slowik V, Hildreth A, Pacheco MC, et al. Hepatopulmonary Syndrome in an Adolescent With Insidious Hypoxia and Small Intrahepatic Portal Venous Shunts: Posttransplant Benefit From Sildenafil. Pediatr Dev Pathol. 2020;23(6):467-471. doi:10.1177/1093526620945951