Management of Hepatopulmonary Syndrome and Portopulmonary Hypertension

Hepatopulmonary syndrome (HPS) is seen in 20-30% of cirrhotics undergoing liver transplantation (LT) evaluation, and has been associated with worse survival. Pulse oximetry and contrast-enhanced transthoracic echocardiography are the most effective screening tests for HPS in LT candidates. LT is the only available treatment for this condition. Portopulmonary hypertension (POPH) is present in 6% of cirrhotics evaluated for LT. Transthoracic Doppler echocardiography is the best screening test for POPH, requiring right-heart catheterization for confirmation if the estimated right ventricular systolic pressure is higher than 40. LT is indicated for patients with a mean pulmonary arterial pressure (mPAP) <35 mmHg, or baseline pressure between 35-45 mmHg, achieving sustained mPAP <35 while on medical treatment.