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The authors report a case of the association of primary biliary cirrhosis and pulmonary hypertension presenting with syncopal complete atrioventricular block. The clinical, aetiopathological and therapeutic aspects of this pathology are reviewed with special emphasis on the value of early diagnosis of the pulmonary hypertension in cases of unusual dyspnoea in patients with primary biliary cirrhosis especially those of an age when they could benefit from liver-heart-lung transplantation.
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