THROMBOEMBOLIC PULMONARY-HYPERTENSION DUE TO DISSEMINATED FIBROMUSCULAR DYSPLASIA

We present two patients with thromboembolic pulmonary hypertension ass ociated with unusual complications probably caused by disseminated fib romuscular dysplasia (FMD) or FMD-like vascular lesions. Intimal fibro plasia, which is typical of the vascular lesions associated with FMD, was observed in both patients. The presence of such intimal lesions su ggests that there was a systemic factor that caused the formation of r ecurrent thrombi in the systemic vessels in these patients. These case s are the first ones reported in which an association between FMD and pulmonary hypertension has been observed, The pathogenesis of the thro mbi in our patients was thought to be recurrent pulmonary thromboembol isms resulting from FMD.