[Right pulmonary artery obstruction and pulmonary hypertension secondary to aortitis syndrome]

A 56-year-old woman with aortic arch syndrome and finally right pulmonary artery obstruction secondary to Takayasu's aortitis was presented. She had had a history of visual disturbance and dizziness when she looked upward since 1983. On admission in July, 1984, aortography showed obstruction of the right innominate artery and of the left subclavian artery. Pulmonary arterial pressure, pulmonary perfusion and ventilation images seemed to be normal at that time. After discharge from our hospital, she began in 1987, to be aware of dyspnea on effort. Because of this symptom, she was admitted again in March, 1988. The pulmonary perfusion images showed complete lack of perfusion in the right lung, and arterial blood gas showed hypoxia with 62 mmHg in PaO2, 39 mmHg in PaCO2. Cardiac catheterization confirmed pulmonary hypertension with pulmonary artery pressure of 56/18 mmHg. In conclusion, pulmonary perfusion and ventilation scintigraphy proved to be the best way to clarify the nature of a lesion of the pulmonary artery in aortitis syndrome.