EASL Campus

A19-year-old female patient with a history of a large extrahepatic congenital portosystemic shunt and severe portopulmonary hypertension (POPH) undergoes medical management of POPH followed by surgical shunt closure. Mean pulmonary artery pressure (mPAP) and pulmonary vascular resistance (PVR) initially improve after shunt closure. Following an episode of SARS-CoV-2 infection, she develops dyspnoea while on macitentan and tadalafil. Right heart catheterisation reveals a mPAP of 35 mmHg (N<20) and a PVR of 9 WU (N<2) and normal right ventricular function. Cross-sectional imaging shows cavernous transformation of the portal vein and portosystemic collaterals.

What is the optimal treatment for this patient?

A. IV epoprostenol and lung transplantation if the PVR stabilizes <5 WU.

B. Combined liver-lung transplantation.

C. TIPS.

D. IV epoprostenol and liver transplantation if the PVR stabilizes <5 WU.

E. Liver transplantation.