Objectives: Pulmonary arterial dilatation is considered a consequence of chronic pulmonary hypertension (PH), but despite its relatively common detection, its prevalence and prognostic impact have not yet been systematically investigated. The aim of the study was to investigate these factors in a relatively large cohort of severe PH patients. Methods: One hundred and forty-one consecutive patients diagnosed with PH were monitored for a mean of 957 days. Data including functional class, exercise capacity, invasive hemodynamics and pulmonary artery (PA) echo/CT scan measurement were performed and outcomes prospectively collected. Results: PA dilatation is a common feature, present in the 76.6% of cases in this cohort of severe PH patients. Survival at 1, 2 and 3 years was 83, 71 and 58%, respectively. On univariate analysis, the baseline variables associated with a poor outcome were related to pulmonary arterial hypertension associated with connective tissue disease (CDT-PAH), New York Heart Association (NYHA) functional class, 6-min walk test and right atrial pressure. On multivariate analysis only CDT-PAH and NYHA functional class remained independently associated with poor survival. Conclusions: PA dilatation is commonly detected in severe PH patients and is not associated with an increased risk of death.

1.
Simonneau G, Galiè N, Rubin LJ, Langleben D, Seeger W, Domenighetti G, Gibbs S, Lebrec D, Speich R, Beghetti M, Rich S, Fishman A: Clinical classification of pulmonary hypertension. J Am Coll Cardiol 2004;43(12 suppl S):5S–12S.
2.
Chin KM, Rubin LJ: Pulmonary arterial hypertension. J Am Coll Cardiol 2008;51:1527–1538.
3.
Barst RJ, McGoon M, Torbicki A, Sitbon O, Krowka MJ, Olschewski H, Gaine S: Diagnosis and differential assessment of pulmonary arterial hypertension. J Am Coll Cardiol 2004;43(12 suppl S):40S–47S.
4.
Galiè N, Hoeper MM, Humbert M, Torbicki A, Vachiery JL, Barbera JA, Beghetti M, Corris P, Gaine S, Gibbs JS, Gomez-Sanchez M, Jondeau G, Klepetko W, Opitz C, Peacock A, Rubin L, Zellweger L, Simonneau G, et al: Guidelines for the diagnosis and treatment of pulmonary hypertension: the Task Force for the Diagnosis and Treatment of Pulmonary Hypertension of the European Society of Cardiology (ESC) and the European Respiratory Society (ERS), endorsed by the International Society of Heart and Lung Transplantation (ISHLT). Eur Heart J 2009;30:2493–2537.
5.
Weyman AE: Principles and Practice of Echocardiography, ed 2. Philadelphia, Lippincott Williams & Wilkins, 1994.
6.
Boyd U, McGavack TH: Aneurysm of the pulmonary artery: a review of the literature and report of two new cases. Am Heart J 1939;18:562–578.
7.
Deterling RA, Clagett T: Aneurysm of the pulmonary artery: review of the literature and report of a case. Am Heart J 1947;34:471–499.
8.
Arom KV, Richardson JD, Grover FL, Ferris G, Trinkle JK: Pulmonary artery aneurysm. Am Surg 1978;44:688–692.
9.
Bartter T, Irwin RS, Nash G: Aneurysms of the pulmonary arteries. Chest 1988;94:1065–1075.
10.
Trell E: Pulmonary arterial aneurysm. Thorax 1973;28:644–649.
11.
Butto F, Lucas RV Jr, Edwards JE: Pulmonary arterial aneurysm: a pathologic study of five cases. Chest 1987;91:237–241.
12.
Mesquita SM, Castro CRP, Ikara NM, Oliveira SA, Lopes AA: Likelihood of left main coronary artery compression based on pulmonary trunk diameter in patients with pulmonary hypertension. Am J Med 2004;116:369–374.
13.
Steurer J, Jenni R, Medici TC, Vollrath T, Hess OM, Siegenthaler W: Dissecting aneurysm of the pulmonary artery with pulmonary hypertension. Am Rev Respir Dis 1990;142:1219–1221.
14.
Senbaklavaci O, Kaneko Y, Bartunek A, Brunner C, Kurkciyan E, Wunderbaldinger P, Klepetko W, Wolner E, Mohl W: Rupture and dissection in pulmonary artery aneurysms: incidence, cause, and treatment – review and case report. J Thorac Cardiovasc Surg 2001;121:1006–1008.
15.
Watson AJ: Dissecting aneurysm of arteries other than the aorta. J Pathol Bacteriol 1956;72:439–449.
16.
Walley VM, Virmani R, Silver MD: Pulmonary arterial dissections and ruptures: to be considered in patients with pulmonary arterial hypertension presenting with cardiogenic shock or sudden death. Pathology 1990;22:1–4.
17.
Humbert M, Sitbon O, Chaouat A, Bertocchi M, Habib G, Gressin V, Yaici A, Weitzenblum E, Cordier JF, Chabot F, Dromer C, Pison C, Reynaud-Gaubert M, Haloun A, Laurent M, Hachulla E, Simonneau G: Pulmonary arterial hypertension in France: results from a national registry. Am J Respir Crit Care Med 2006;173:1023–1030.
18.
Thenappan T, Shah SJ, Rich S, Gomberg-Maitland M: A USA-based registry for pulmonary arterial hypertension: 1982–2006. Eur Respir J 2007;30:1103–1110.
19.
Sitbon O, Humbert M, Nunes H, Parent F, Garcia G, Hervé P, Rainisio M, Simonneau G: Long-term intravenous epoprostenol infusion in primary pulmonary hypertension: prognostic factors and survival. J Am Coll Cardiol 2002;40:780–788.
20.
McLaughlin VV, Shillington A, Rich S: Survival in primary pulmonary hypertension: the impact of epoprostenol therapy. Circulation 2002;106:1477–1482.
21.
Benza RL, Miller DP, Gomberg-Maitland M, Frantz RP, Foreman AJ, Coffey CS, Frost A, Barst RJ, Badesch DB, Elliott CG, Liou TG, McGoon MD: Predicting survival in pulmonary arterial hypertension: insights from the Registry to Evaluate Early and Long-Term Pulmonary Arterial Hypertension Disease Management (REVEAL). Circulation 2010;122:164–172.
22.
Humbert M, Sitbon O, Yaïci A, Montani D, O’Callaghan DS, Jaïs X, Parent F, Savale L, Natali D, Günther S, Chaouat A, Chabot F, Cordier JF, Habib G, Gressin V, Jing ZC, Souza R, Simonneau G, French Pulmonary Arterial Hypertension Network: Survival in incident and prevalent cohorts of patients with pulmonary arterial hypertension. Eur Respir J 2010;36:549–555.
23.
Veldtman GR, Dearani JA, Warnes CA: Low pressure giant pulmonary artery aneurysms in the adult: natural history and management strategies. Heart 2003;89:1067–1070.
24.
Bland JM, Altman DG: Statistical methods for assessing agreement between two methods of clinical measurement. Lancet 1986;327:307–310.
Copyright / Drug Dosage / Disclaimer
Copyright: All rights reserved. No part of this publication may be translated into other languages, reproduced or utilized in any form or by any means, electronic or mechanical, including photocopying, recording, microcopying, or by any information storage and retrieval system, without permission in writing from the publisher.
Drug Dosage: The authors and the publisher have exerted every effort to ensure that drug selection and dosage set forth in this text are in accord with current recommendations and practice at the time of publication. However, in view of ongoing research, changes in government regulations, and the constant flow of information relating to drug therapy and drug reactions, the reader is urged to check the package insert for each drug for any changes in indications and dosage and for added warnings and precautions. This is particularly important when the recommended agent is a new and/or infrequently employed drug.
Disclaimer: The statements, opinions and data contained in this publication are solely those of the individual authors and contributors and not of the publishers and the editor(s). The appearance of advertisements or/and product references in the publication is not a warranty, endorsement, or approval of the products or services advertised or of their effectiveness, quality or safety. The publisher and the editor(s) disclaim responsibility for any injury to persons or property resulting from any ideas, methods, instructions or products referred to in the content or advertisements.
You do not currently have access to this content.