[A case of the multiple peripheral pulmonary artery branch stenosis]

A 31-year-old woman in whom primary pulmonary hypertension had been diagnosed at age 18, was admitted because of dyspnea on exertion and for evaluation of pregnancy risk. The perfusion scanning was not diagnostic, but chest CT suggested stenosis of pulmonary arteries instead of primary pulmonary hypertension. Echocardiograms and cardiac catheterization revealed pulmonary hypertension of pulmonary arterial pressure 71/13 (mean 39) mmHg with no cardiac defects. Selective pulmonary angiograms showed multiple stenosis of pulmonary arteries with poststenotic dilatations. We diagnosed multiple peripheral pulmonary artery branch stenosis with the pulmonary hypertension because systemic vasculitis was excluded because there were no signs of inflammation or any systemic arterial invlolvement This case was thought to be congenital, considering her history of over 30 years. In reports from western countries, maternal rubella and familial factors were important etiologic factors of this disease. Few cases have been reported in Japan and all cases, including the present case, were unrelated to the specific etiological factors. We recommended her to use birth control and have been observing her very carefully since the progression of the pulmonary hypertension is the major determinant of her prognosis.