Portopulmonary hypertension and right heart failure in patients with cirrhosis

In 5-6% of patients with portal hypertension a pathological state exists in which changes in the pulmonary vasculature cause an increase in pulmonary vascular resistance. The resultant increased work of the right ventricle may cause right heart failure and liver congestion. Patients with cirrhosis are at increased risk of mortality and transplant graft failure. The present review examines the latest advances in diagnosis, treatment and management of portopulmonary hypertension.

Portopulmonary hypertension may be screened with transthoracic echocardiography and following up with a right heart catheterization in patients in whom the right ventricular systolic pressure is calculated to be 50 mmHg or greater. Therapy with prostanoids, endothelin-1 inhibitors and phosphodiesterase-5 inhibitors, or a combination of therapies, may be very effective in moderating pulmonary artery hypertension and, in selected patients, allowing liver transplantation to proceed safely. The model for end-stage liver disease (MELD) score is being weighted to accelerate responders on the waiting list for a transplant.

Advances in diagnosis and therapy of portopulmonary hypertension allow patients with cirrhosis who respond to vasodilators to undergo liver transplantation safely. Unfortunately liver transplantation does not always result in reversal of pulmonary hypertension. There are now reports of de-novo pulmonary hypertension after liver transplantation.