Long-term effect of a new oral prostacyclin analogue, beraprost sodium, in isolated peripheral pulmonary artery stenosis in the adult.

Abstract

A 20-year-old woman was admitted with increasing breathlessness on exertion. A diagnosis of isolated peripheral pulmonary artery stenosis (PPS) was confirmed by right-sided cardiac catheterization and pulmonary angiography. We tried treatment with a newly developed oral prostacyclin analogue, beraprost sodium. Her symptoms gradually improved 1 month after treatment. After 1 year, we observed a decrease in pulmonary arterial pressure (from 118/16 [mean 59] to 87/13 [mean 40] mmHg) and systolic right ventricular pressure (from 125 to 90 mmHg). This is the first report to describe the chronic effect of oral prostacyclin analogue on isolated peripheral pulmonary artery stenosis.