Pulmonary hypertension Pulmonary Hypertension 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 can occur in people with various conditions that involve portal hypertension Portal Hypertension Portal hypertension is abnormally high blood pressure in the portal vein (the large vein that brings blood from the intestine to the liver) and its branches. Cirrhosis (scarring that distorts... read more , which is most often a complication of cirrhosis of the liver Cirrhosis of the Liver Cirrhosis is the widespread distortion of the liver's internal structure that occurs when a large amount of normal liver tissue is permanently replaced with nonfunctioning scar tissue. The scar... read more . The combination of pulmonary hypertension and portal hypertension is called portopulmonary hypertension.
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 ) 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 widen (dilate) the blood vessels (vasodilators 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 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.