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Portopulmonary Hypertension

By

Mark T. Gladwin

, MD, University of Pittsburgh School of Medicine;


Andrea R. Levine

, MD, University of Maryland School of Medicine

Last full review/revision Sep 2020| Content last modified Sep 2020
Click here for the Professional Version

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.

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

Diagnosis of Portopulmonary Hypertension

  • 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 Cor Pulmonale Cor pulmonale is enlargement and thickening of the ventricle on the right side of the heart resulting from an underlying lung disorder that causes pulmonary hypertension (high pressures in the... read more ).

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 Cardiac Catheterization and Coronary Angiography Cardiac catheterization and coronary angiography are minimally invasive methods of studying the heart and the blood vessels that supply the heart (coronary arteries) without doing surgery. These... read more Cardiac Catheterization and Coronary Angiography ) to measure the blood pressure in the right ventricle and the main artery of the lungs (pulmonary artery).

Treatment of Portopulmonary Hypertension

  • 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 Treatment Pulmonary hypertension is a condition in which blood pressure in the arteries of the lungs (the pulmonary arteries) is abnormally high. Many disorders can cause pulmonary hypertension. People... read more ), 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 Liver Transplantation Liver transplantation is the surgical removal of a healthy liver or sometimes a part of a liver from a living person and then its transfer into a person whose liver no longer functions. (See... read more because the disorder increases the risk of death or serious complications due to the procedure.

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