Portopulmonary hypertension (POPH)

(Expert Rev Gastro Hepatol 2015;9:983)

• Pulm HTN in Pt w/ portal HTN w/o other cause. ESLD→ ↑ endothelin→ pulm vasoconst.
• Rx w/ same therapies as for idiopathic PAH, incl prostacyclin analogs, endothelin receptor antagonists, sildenafil; liver transplant is often curative