抄録
We have experienced two cases of primary pulmonary hypertension; a 9-year-old girl and a 20-year-old male. Both these cases had had several attacks of syncope. The girl had complained of dyspnea and slight cyanosis, especially on exertion. The pulmonary artery pressures were systolic 111, ditolic 60, mean 84 mmHg. Autopsy of this case revealed a marked pulmonary arterio-and arteriolosclerosis with some thrombosis but no evidence of embolism. The male had dyspnea and marked cyanosis on exertion, the cyanosis was investigated. The pulmonary artery pressures were systolic 89, diastolic 55, mean 68 mmHg and the pulmonary arterial wedge pressure was 11 mmHg. The pulmonary vascular resistance was very high.On 100% oxygen inhalation the arterial blood oxygen saturation went up to 100%. However, the saturation dropped to the level of 80% during exercise test even though 100% oxygen was administered. On the other hand the cardiac catheterization showed no left to right shunt at any level.Autopsy of this male revealed a patent foramen ovale, which permitted only one way shunting of right to left, and no other congenital or acquired cardiovascular abnormalities. A marked pulmonary arterio- and arteriolosclerosis without any evidence of pulmonary arterial embolism or thrombosis was revealed. These two cases can be regarded as so-called primary pulmonary hypertension.We found, however, on observation of the pulmonary vascular casts of the male a great number of arterial anastomosis between the bronchial and pulmonary arteries. We discussed the relationship between these arterial anastomosis and the primary pulmonary hypertension, and concluded that these arterial anastomosis can be considered as the sequelae of the fetal state and will have a causal effects on the development of plumonary hypertension by continuous shunting of left to right.
