Gigantic Pulmonary Arteries

Abstract

The new england journal of medicine n engl j med 349;12 www.nejm.org september 18, 2003 1148 Gigantic Pulmonary ArteriesPapworth Hospital Cambridge CB3 8RE, United Kingdom 49-year-old woman presented with a 12-month history of progressive exertional dyspnea and hoarseness. She had received a diagnosis of primary pulmonary hypertension after an uneventful pregnancy and delivery 22 years earlier. At that time, right heart catheterization showed a mean pulmonaryartery pressure of 42 mm Hg. She was treated with oral anticoagulants, and her condition remained stable thereafter. At the time of her current presentation, an x-ray film of the chest (Panel A) showed gigantic pulmonary arteries and cardiomegaly. Magnetic resonance imaging (Panel B) confirmed the presence of marked dilatation of the main pulmonary artery (long arrows), with peripheral vascular "pruning" (short arrows). Repeated right heart catheterization revealed a mean pulmonary-artery pressure of 46 mm Hg and a pulmonary vascular resistance of 980 dyn·sec·cm ¡5 . The reason for the patient's unusually long survival with primary pulmonary hypertension, which is typically a progressive disorder, is unknown.