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Portopulmonary Hypertension

By Mark T. Gladwin, MD, Jack D. Myers Professor and Chair, Department of Medicine;Director, University of Pittsburgh School of Medicine;Pittsburgh Heart, Lung, and Blood Vascular Medicine Institute

Portopulmonary hypertension is a condition in which blood pressure is elevated in the arteries of the lungs, called the pulmonary arteries (pulmonary hypertension), and in the portal vein (which drains blood from the liver), and no cause for pulmonary hypertension can be found.

Pulmonary hypertension (see Pulmonary Hypertension) occurs in people with various conditions that involve portal hypertension (see Portal Hypertension), which is most often a complication of cirrhosis of the liver.

People develop shortness of breath and fatigue. Chest pain, coughing up blood (hemoptysis), distended neck veins, and swollen legs can also occur.


  • Echocardiography and right heart catheterization

Doctors suspect portopulmonary hypertension in people with liver disease based on the symptoms and findings during a physical examination. Results of the examination and tests, such as electrocardiography (ECG), suggest that the right heart or the right ventricle is enlarged and strained (cor pulmonale—see Cor Pulmonale: A Type of Heart Failure Caused by Lung Disorders).

To diagnose portopulmonary hypertension, doctors do echocardiography and pass a tube through a vein in an arm or a leg into the right side of the heart (right heart catheterization—see Cardiac Catheterization and Coronary Angiography) to measure the blood pressure in the right ventricle and the main artery of the lungs (pulmonary artery).


  • Drugs to lower blood pressure in the arteries of the lungs

Treatment is needed to lower the blood pressure in the arteries of the lungs. Drugs that dilate the blood vessels (vasodilators—see Treatment), are often effective in lowering the blood pressure. However, the severity of the underlying liver disease is a major determinant of outcome. People with portopulmonary hypertension may not be able to undergo liver transplantation because the disorder increases the risk of death or serious complications due to the procedure.