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

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.