[An autopsy case of chronic thromboembolic pulmonary hypertension with long-term progressive dyspnea].

A 71-year-old man who had been suffering from slowly progressive dyspnea on exertion for 16 years was admitted because of hemoptysis and severe dyspnea in 1997. His medical history included respiratory failure in 1986. At that time, chest X-ray films showed cardiomegaly and striking enlargement of hilar pulmonary arteries, while a lung perfusion scan revealed segmental defects. A diagnosis of chronic thromboembolic pulmonary hypertension (CTPH) was made on the basis of multiple filling defects detected by pulmonary angiography and marked pulmonary hypertension (104/29 mmHg) measured by right heart catheterization. The patient died unexpectedly 1 month later. Autopsy revealed a large new thrombotic lesion straddling the organizing thrombotic lesion in the right pulmonary artery. It is important to keep CTPH in mind when examining and treating patients with unexplained dyspnea. In this respect, our case was considered of value to an understanding of the natural course of CTPH.