Portopulmonary hypertension is one of the rare pulmonary complications of portal hypertension. Portopulmonary hypertension, is the right ventricular dysfunction caused by increased pulmonary artery pressure which developed due to the pulmonary vascular vasoconstruction. Vasoconstrictors, that cannot be broken-down by the liver and reach to the pulmonary circulation with portosystemic collaterals were blamed for the pathogenesis. First signs were exertional dispnea and fatigue. Patients with symptoms and liver transplantation candidates must be screened for portopulmonary hypertension. Transthoracic echocardiography is used as the screening test. If right ventricular systolic pressure was high at this test, right ventricular catheterization must be done. Specific vasodilatator agents nitric oxide, prostacyslins, endothelin-1 receptor antagonists and phosphodiesterase inhibitors are used for the treatment. Liver transplantation can be done successfully to those who responds the vasodilatator therapy.
Key words: Liver transplantation, portal hypertension, portopulmonary hypertension, cirrhosis
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