Pulmonary hypertension secondary to neurofibromatosis: intimal fibrosis versus thromboembolism

Neurofibromatosis has been known to involve blood vessels throughout the bo dy. Pulmonary involvement with interstitial fibrosing alveolitis has been d escribed but no case of pulmonary vascular involvement has been reported to date. A 51 year old patient with cutaneous neurofibromatosis is described who presented with severe pulmonary hypertension and radiographic, scintigr aphic, and angiographic evidence of chronic thromboembolic pulmonary hypert ension. Severe intimal fibrosis consistent with vascular involvement with n eurofibromatosis was found on endarterectomy with no evidence of pulmonary thromboembolism. Neurofibromatosis of pulmonary arteries should be consider ed as a possible cause of pulmonary hypertension.