What question did this study set out to answer?

To highlight the rare occurrence of pulmonary arterial hypertension caused by congenital portosystemic shunt and its acute management in a child.

July 2, 2026Archives of Pediatric Critical Care0 citationsOpen Access

Severe pulmonary arterial hypertension associated with congenital portosystemic shunt presenting as acute liver failure in a child

NLN LeeYonsei University NTNuri TchahSeverance Hospital JSJae Hee SeolYonsei University

Key Points

To highlight the rare occurrence of pulmonary arterial hypertension caused by congenital portosystemic shunt and its acute management in a child.
Case report of a 2-year-old girl presenting with acute liver injury and diagnosed with severe PAH and CPSS.
Utilized echocardiography to assess cardiac function and pressures.
Received inhaled nitric oxide and other PAH-targeted therapies, including sildenafil and bosentan.
Echocardiography confirmed severe pulmonary hypertension and right cardiac enlargement.
Child required venoarterial extracorporeal membrane oxygenation and percutaneous atrial septostomy.
Achieved stabilization allowing for discharge on oral medication after targeted PAH therapy.

Abstract

Pulmonary arterial hypertension (PAH) associated with congenital portosystemic shunt (CPSS) is rare in children, and evidence to guide acute management is limited.We report the case of a 2-year-old girl who presented with marked acute liver injury and coagulopathy, leading initially to suspicion of acute liver failure, and was subsequently diagnosed with severe PAH associated with CPSS.Echocardiography demonstrated severe pulmonary hypertension, right-sided cardiac enlargement, and suprasystemic right ventricular pressure.Despite inhaled nitric oxide therapy, she developed refractory cardiogenic shock with worsening hepatic dysfunction and required venoarterial extracorporeal membrane oxygenation and percutaneous atrial septostomy for right heart decompression.Computed tomography showed congenital absence of the left portal vein and a portosystemic shunt.Definitive shunt closure was deferred until clinical stabilization could be achieved.She received sequential PAH-targeted therapy and was discharged on oral sildenafil, bosentan, and furosemide.This case underscores the importance of early cardiopulmonary assessment in children with unexplained presentations resembling acute liver failure and supports stepwise stabilization as a bridge to definitive CPSS management.

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