According to a new paper, “Pulmonary hypertension and hepatic cirrhosis,” published in the Spanish-language journal, Revista Clinica Espanola, pulmonary hypertension (PH) occurs frequently in people with hepatic cirrhosis. There are various ways in which hepatic cirrhosis can be caused by PH, but the most common occurrence is something called portopulmonary syndrome–or PPH for short.
PH is a serious disease involving high blood pressure of the lungs due to capillary narrowing, which worsens over time and can be fatal. PH is not curable but it can be managed. Treatments focus on stopping the progress of the disease and relieving symptoms and can include medications such as phosphodiesterase-5 inhibitors, prostanoids, endothelin receptor antagonists and calcium channel blockers, such as diltiazem.
Hepatic cirrhosis is a chronic disease of the liver. Normal liver cells are damaged and replaced by scar tissue. Alcohol abuse is the most common cause of cirrhosis, although diseases such as hepatitis and cystic fibrosis can also cause cirrhosis. Other causes include fatty build-up on the liver, iron or copper build up, poorly formed bile ducts, and genetic or autoimmune disorders. Pulmonary problems (blood flow to the lungs) are common in people with liver disease.
Unfortunately, there are no treatments that can reverse scarring of the liver once it has occurred. Medical management of cirrhosis usually involves addressing the underlying causes, such as encouraging the patient to stop drinking alcohol, to discontinue prescription medications that may exacerbate liver damage and to reduce salt in the diet.
About 5% of people with liver disease develop PH, with women and those with autoimmune hepatitis at greater risk. Those with hepatitis C have a lower risk for PH.
According to the new paper, determining the specific cause of pulmonary hypertension is important for understanding ways of treating the condition. In their report, the authors state: “A correct assessment of hemodynamic, ultrasound and clinical variables enables the differential diagnosis of each situation that produces pulmonary hypertension in patients with cirrhosis.” Hemodynamic refers to understanding blood flow and where the circulation could be affected.
Unfortunately, people with both liver disease and PH have a worse prognosis than people with PH alone. Older studies have indicated that survival is typically about 6 months and that about 10% of people with both conditions with survive approximately 5-years, although more recent studies have found a 5-year survival of 50%. This may be due to improvements in healthcare and the treatment of both conditions.