ultiple Peripheral Pulmonary Artery Stenoses in Adults: A Rare ause of Severe Pulmonary Hypertension Necessitating ung Transplantation

tenoses of multiple peripheral pulmonary artery branches represent a rare cause of pulmonary hypertension in hildren, but the prognosis is very poor for such patients. Herein we describe 2 patients with multiple peripheral ulmonary artery stenoses (MPPAS) presenting with severe pulmonary arterial hypertension in adulthood, which as only once been described previously. Both patients lived without significant health problems for decades; owever, after onset of symptoms, their medical condition declined rapidly, necessitating lung transplantation everal months after the diagnosis despite vasodilator therapy. Because MPPAS mimics chronic thromboembolic ulmonary hypertension, this entity may being underdiagnosed, decreasing the possibility of adequate therapy. Heart Lung Transplant 2005;24:1984–7. Copyright © 2005 by the International Society for Heart and Lung ransplantation.