An autopsied case of primary pulmonary hypertension complicated by hepatopulmonary syndrome

A 57-year-old man, who had received a transfusion five years before, was admitted to our hospital complaining of worsening dyspnea on exertion. Cardiac catheterization was performed, and pulmonary hypertension was diagnosed. Liver dysfunction was also documented. We administered diuretics and observed his clinical course. Gradually worsening hypoxemia and radioisotope accumulation in the kidney following a lung perfusion scintigram suggested the existence of an intrapulmonary shunt. The patient died seven years later due to exacerbation of heart failure secondary to pulmonary infection. Autopsy revealed remarkable hypertensive pulmonary arteriopathy as well as abnormal dilation of precapillary pulmonary arterioles. Esophageal varices suggested portal hypertension. Marked hypoxemia and intrapulmonary vascular dilation suggest the contribution of an hepatopulmonary syndrome.