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

¹ Section of Cardiology, Dept of Medicine, University of Chicago, Chicago, IL

^* To whom correspondence should be addressed. E-mail: mgomberg{at}medicine.bsd.uchicago.edu.

	Abstract

We aimed to define the epidemiology of World Health Organization (WHO) Group I pulmonary arterial hypertension (PAH) in a large referral center in the United States (US).

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

578 patients, age 48±14 years, 77% female, and 80% with class III or IV symptoms were entered. Over time, connective tissue disease (CTD)-associated PAH increased, while referrals for human immunodeficiency virus (HIV) remained low. One-third of patients were referred on calcium channel blocker (CCB) 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 has improved over time, with a 1-year survival of 85% in the incident cohort.

In the US, PAH patients are still referred to tertiary centers late. Referral of CTD is increasing, while referral of HIV remains low. Inappropriate CCB treatment is common. Survival rates have increased but remain low suggesting that prognosis is improving but PAH is still a progressive, fatal disease.

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