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Portopulmonary hypertension is pulmonary arterial hypertension associated with portal hypertension without other secondary causes.
Pulmonary hypertension occurs in patients with various conditions that involve portal hypertension with or without cirrhosis. Portopulmonary hypertension occurs less commonly than the hepatopulmonary syndrome in patients with chronic liver disease (3.5 vs 12%).
Presenting symptoms are dyspnea and fatigue. Chest pain and hemoptysis can also occur. Patients have physical findings and ECG abnormalities consistent with pulmonary hypertension and may develop evidence of cor pulmonale (elevated jugular venous pulse, edema). Tricuspid regurgitation is common.
The diagnosis is suspected based on echocardiography findings and confirmed by right heart catheterization.
Treatment is the same as that of pulmonary arterial hypertension except that hepatotoxic drugs and anticoagulants should be avoided (see Pulmonary Hypertension : Treatment). Some patients benefit from vasodilator therapy. The underlying liver disease is a major determinant of outcome. Portopulmonary hypertension is a relative contraindication to liver transplantation because of increased morbidity and mortality from the procedure. However, in some patients who receive a transplant, particularly those with mild pulmonary hypertension, pulmonary hypertension regresses. Some centers consider transplantation in patients who have mean pulmonary arterial pressures < 35 mm Hg after a trial of vasodilator therapy.
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