AASLD: Hepatopulmonary Syndrome And Portopulmonary Hypertension
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AASLD: Hepatopulmonary Syndrome And Portopulmonary Hypertension

By Lauri Gray Eaton
Special to DG News

DALLAS, TX -- November 12, 2001 -- Pulmonary symptoms and abnormalities -- particularly dyspnea, or shortness of breath -- are common in patients with chronic liver disease.

As many as 70 percent of cirrhotic patients undergoing evaluation for liver transplantation complain of dyspneas if questioned, according to Michael B. Fallon, MD, an associate professor of medicine in the division of gastroenterology and hepatology at the University of Alabama-Birmingham, and chief of gastroenterology and hepatology at the Birmingham Veterans Administration Medical Center, in Birmingham, Alabama,.

Dr. Fallon spoke Saturday (Nov. 10) at the annual meeting of the American Association for the Study of Liver Disease (AASLD), being held Nov. 9-13 at the Wyndham Anatole Hotel in Dallas, Texas.

Other pulmonary symptoms of intrinsic liver disease include chronic obstructive pulmonary disease and asthma, Dr. Fallon said. However, "most patients with hepatopulmonary syndrome report the insidious onset of progressive dyspnea."

"The recognition that a subset of patients with hepatic disease develops significant pulmonary vascular alterations -- either microvascular dilation leading to the hepatopulmonary or arteriolar vasoconstriciton leading to portopulmonary hypertension -- indicates that unique changes in the pulmonary vasculature may occur," Dr. Fallon noted. "These pulmonary vascular syndromes significantly impact morbidity and mortality in affected patients and may influence candidacy for liver transplantation."

Hepatopulmonary syndrome occurs in approximately 10 to 15 percent of patients with cirrhosis and is defined by intrapulmonary vasodilation and abnormal alveolar gas exchange.

No clearly effective medical therapies for hepatopulmonary syndrome have been found, Dr. Fallon said.

Four case reports have suggested that use of a transjugular intrahepatic portosystemic shunt (TIPS) may improve gas exchange in hepatopulmonary syndrome, but they remains experimental.

Liver transplantation is the only proven therapy for hepatopulmonary syndrome based on the total resolution of significant improvement in gas exhcange post-operatively in more than 85 percent of reported patients, he said, citing a 1996 conference report at Clinics in Chest Medicine.

Portopulmonary hypertension occurs in 2.5 to 12 percent of patients with cirrhosis and is defined by a mean pulmonary arterial pressure greater than 25 mm Hg with a normal pulmonary capillary wedge pressure (<15 mm Hg).

The effectiveness of liver transplantation as a treatment for portopulmonary hypertension remains controversial. Retrospective data and clinical experience indicate that moderate to severe portopulmonary hypertension (mean artery pressure > 50 mm Hg) is a contraindication to transplantation due to perioperative mortality of approximately 40 percent from cardiopulmonary complications and lack of reversibility of pulmonary hypertension.

Patients with mild pulmonary hypertension appear to have no increase in cardiopulmonary mortality after liver transplantation.

Epoprostenol can decrease pulmonary pressures in some patients, Dr. Fallon said.

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