Pulmonary hypertension (PH) is characterized by high blood pressure, which affects the pulmonary arteries, the vessels that transport blood from the heart to the lungs. Due to the thickened and narrowed pulmonary arteries, the heart needs to work under stress to properly pump the blood, which can result in enlarged and weakened heart, as well as right heart failure and even death.

The disease can be classified as idiopathic when the reason for its development is unknown, while it can also result from an underlying disease. There are numerous conditions that can cause pulmonary hypertension, including cirrhosis, a liver condition that causes the progressive transformation of healthy tissue into scarred tissue. The scarring blocks the normal blood flow, slowing the organ’s capacity to process nutrients, hormones, drugs and toxins.

Development of Pulmonary Hypertension in Cirrhosis Patients

The development of pulmonary hypertension by patients with cirrhosis is usually related to portal hypertension, which is typical in patients with cirrhosis and can result in PH, as explained in the study “Pulmonary Complications of Cirrhosis,” authored by Rowen K. Zetterman, MD. Portal hypertension, an increase in the blood pressure within a system of veins called the portal venous system, is consistent with a mean pulmonary artery pressure higher than 25 mm Hg at rest or higher than 30 mm Hg exercising, and it can cause pulmonary vascular resistance and pulmonary capillary wedge pressure.

Volume overload from sodium retention can i be one of the causes for high pulmonary artery pressures. In addition, patients with cirrhosis can also develop pulmonary hypertension due to other reasons. Other associated conditions can result in pulmonary hypertension, being the most common multiple pulmonary emboli and autoimmune hepatitis. The disease can also occur as consequence of a liver transplant.

Diagnosis and Prevalence of Pulmonary Hypertension in Cirrhosis Sufferers

Pulmonary hypertension and cirrhosis are more common among women and patients who also suffer from autoimmune hepatitis, and about five percent of the with end-stage liver disease will develop pulmonary hypertension. Being aware of the signs of the disease is one the first steps to detect it early. Progressive symptoms of pulmonary hypertension in patients with cirrhosis include dyspnea on exertion, orthopnea, fatigue, and syncope, being the last one a late sign.

Physicians may request an echocardiography to screen the pulmonary arterial pressure, while values higher than 40 mm Hg should prompt a closer evaluation to confirm the diagnostic. Right heart catheterization is also a common diagnostic method, since patients with portopulmonary hypertension develop pericardial effusions. Routine chest x-rays and ECG can also be conducted to analyze the central pulmonary arteries as well as right atrial and right ventricular enlargement.

Pulmonary Hypertension and Cirrhosis Treatment and Prognosis

There is no cure for pulmonary hypertension, but there are treatments that can help ease the symptoms. Since patients with PH and cirrhosis develop the same intrapulmonary arterial alterations as patients without any liver disease, there are common treatments that can be used. Diuretic therapy can decrease intrapulmonary vascular volume, intravenous pulmonary vasodilators to decrease vascular resistance and pulmonary arterial pressure, as well as prostanoids, endothelion-receptor antagonists, and sildenafil are therapeutic options, while the use of beta-blockers is generally discouraged.

“The prognosis is poor for patients with portopulmonary hypertension. Early studies suggested a median survival of 6 months and a 5-year survival of < 10%, although more recent studies have found a 5-year survival of 50%. The prognosis of portopulmonary hypertension is worse than that for primary pulmonary hypertension because of associated liver disease,” is explained in the study about pulmonary hypertension and cirrhosis survival.