Abstract
Primary pulmonary Hypertension is a rare disease of unknown etiology. Watanabe, S. et al. (Chiba Univ.) stated that 137 cases, including our 1 case, have been reported until 1974 in Japan.
We have experienced one autopsied case of primary pulmonary hypertension, a 37-years-old female, who was admitted to the hospital with a complaint of dyspnea in Dec. 1972. For the past 2 years, she had been suffering from dyspnea on exertion. On admission, she had marked cyanosis and slight edema. Second pulmonic valvular sound was splitted, but no murmur was audible. ASLO was 500×, CRP was (++), liver function test was normal, EKG revealed RVH without rBBB, chest X-P showed a dilated right main pulmonary artery with enlarged right ventricle. The pulmonary artery pressure were 100/45, mean 60mmHg, on the other hand, the cardiac catheterisation and angiography disclosed no shunt at any level. Because of clinical and physical findings, this case can be regarded as primary pulmonary hypertension.
Her condition gradually got worse, and in Aug. 1973, 8 months after admission she died.
Autopsy of this case revealed a right ventricle hypertrophy, and there was no congenital or acquired cardiovascular abnormality.
Histological findings in pulmonary arteries and artrioles revealed marked medial hypertrophy, intimal thickening and plexiform lesion, but there was not found cellular proliferation.
We discussed the etiology, symptoms, differential diagnosis, and clinical course of this disorder on literatures.
We conclude that the etiology of this disease is undetermined, and effective therapy is not found at present.
