HOME HELP FEEDBACK SUBSCRIPTIONS ARCHIVE SEARCH TABLE OF CONTENTS		QUICK SEARCH:   [advanced] Author: Keyword(s): Year:  Vol:  Page:

This Article Full Text Full Text (PDF) Alert me when this article is cited Alert me if a correction is posted Citation Map Services Email this article to a friend Similar articles in this journal Similar articles in ISI Web of Science Similar articles in PubMed Alert me to new issues of the journal Download to citation manager Citing Articles Citing Articles via HighWire Citing Articles via ISI Web of Science (3) Citing Articles via Google Scholar Google Scholar Articles by Thenappan, T. Articles by Gomberg-Maitland, M. Search for Related Content PubMed PubMed Citation Articles by Thenappan, T. Articles by Gomberg-Maitland, M.

A USA-based registry for pulmonary arterial hypertension: 1982–2006

Section of Cardiology, Dept of Medicine, University of Chicago, Chicago, IL, USA.

CORRESPONDENCE: M. Gomberg-Maitland, 5841 S Maryland Ave, MC 2016, Chicago, IL, 60637, USA. Fax: 1 7738341764. E-mail: mgomberg{at}medicine.bsd.uchicago.edu

Keywords: Aetiology, epidemiology, medications, pulmonary arterial hypertension, survival

Received: April 6, 2007
Accepted August 20, 2007

The aim of this study was to define the epidemiology of World Health Organization (WHO) Group I pulmonary arterial hypertension (PAH) in a large referral centre in the USA.

The Pulmonary Hypertension Connection registry, initiated in 2004, evaluated all patients in a single USA practice from 1982–2006. For comparison, the authors divided the group by incident versus prevalent cohorts, aetiology and by treatment era.

In total, 578 patients (77% female) aged 48±14 yrs were entered. Of these, 80% had class III or IV symptoms. Over time, connective tissue disease-associated PAH increased, while referrals for HIV remained low. One-third of patients were referred on calcium channel blocker therapy even though only 4.6% had an acute response to vasodilator challenge. When compared by treatment era, there were no differences in the severity of PAH. However, survival had improved over time, with a 1-yr survival of 85% in the incident cohort.

In the USA, pulmonary arterial hypertension patients are still referred to tertiary centres too late. Referral of connective tissue disease is increasing, while referral of HIV remains low. Inappropriate calcium channel blocker treatment is common. Survival rates have increased but remain low suggesting that prognosis is improving but PAH is still a progressive, fatal disease.

This article has been cited by other articles:

				R. W. Schrier and S. Bansal Pulmonary Hypertension, Right Ventricular Failure, and Kidney: Different from Left Ventricular Failure? Clin. J. Am. Soc. Nephrol., September 1, 2008; 3(5): 1232 - 1237. [Abstract] [Full Text] [PDF]

				M. Gomberg-Maitland Traditional and Alternative Designs for Pulmonary Arterial Hypertension Trials Proceedings of the ATS, July 15, 2008; 5(5): 610 - 616. [Abstract] [Full Text] [PDF]

				K. M. Chin and L. J. Rubin Pulmonary arterial hypertension. J. Am. Coll. Cardiol., April 22, 2008; 51(16): 1527 - 1538. [Abstract] [Full Text] [PDF]

				M. Humbert Update in Pulmonary Arterial Hypertension 2007 Am. J. Respir. Crit. Care Med., March 15, 2008; 177(6): 574 - 579. [Full Text] [PDF]

				S. L. Archer, M. Gomberg-Maitland, M. L. Maitland, S. Rich, J. G. N. Garcia, and E. K. Weir Mitochondrial metabolism, redox signaling, and fusion: a mitochondria-ROS-HIF-1{alpha}-Kv1.5 O2-sensing pathway at the intersection of pulmonary hypertension and cancer Am J Physiol Heart Circ Physiol, February 1, 2008; 294(2): H570 - H578. [Abstract] [Full Text] [PDF]