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Original Research: Pulmonary Vascular Disease |

An Evaluation of Long-term Survival From Time of Diagnosis in Pulmonary Arterial Hypertension From the REVEAL RegistrySurvival From Time of Diagnosis in REVEAL Registry

Raymond L. Benza, MD; Dave P. Miller, MS; Robyn J. Barst, MD, FCCP; David B. Badesch, MD, FCCP; Adaani E. Frost, MD, FCCP; Michael D. McGoon, MD, FCCP
Author and Funding Information

From Allegheny General Hospital (Dr Benza), Pittsburgh, PA; ICON Late Phase and Outcomes Research (Mr Miller), San Francisco, CA; Columbia University College of Physicians and Surgeons (Dr Barst), New York, NY; University of Colorado (Dr Badesch), Denver, CO; Baylor College of Medicine (Dr Frost), Houston, TX; and Mayo Clinic (Dr McGoon), Rochester, MN.

Correspondence to: Raymond L. Benza, MD, The Gerald McGinnis Cardiovascular Institute, Allegheny General Hospital, 320 E N Ave, 16th Floor, S Tower, Pittsburgh, PA 15212; e-mail: rbenza@wpahs.org


Funding/Support: Preparation of this manuscript was supported by Actelion Pharmaceuticals US, Inc. Funding for the REVEAL Registry is provided by Actelion Pharmaceuticals US, Inc.

Reproduction of this article is prohibited without written permission from the American College of Chest Physicians. See online for more details.


Chest. 2012;142(2):448-456. doi:10.1378/chest.11-1460
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Background:  The Registry to Evaluate Early and Long-term Pulmonary Arterial Hypertension Disease Management (REVEAL Registry) was established to characterize the clinical course, treatment, and predictors of outcomes in patients with pulmonary arterial hypertension (PAH) in the United States. To date, estimated survival based on time of patient enrollment has been established and reported. To determine whether the survival of patients with PAH has improved over recent decades, we assessed survival from time of diagnosis for the REVEAL Registry cohort and compared these results to the estimated survival using the National Institutes of Health (NIH) prognostic equation.

Methods:  Newly or previously diagnosed patients (aged ≥ 3 months at diagnosis) with PAH enrolled from March 2006 to December 2009 at 55 US centers were included in the current analysis.

Results:  A total of 2,635 patients qualified for this analysis. One-, 3-, 5-, and 7-year survival rates from time of diagnostic right-sided heart catheterization were 85%, 68%, 57%, and 49%, respectively. For patients with idiopathic/familial PAH, survival rates were 91% ± 2%, 74% ± 2%, 65% ± 3%, and 59% ± 3% compared with estimated survival rates of 68%, 47%, 36%, and 32%, respectively, using the NIH equation.

Conclusions:  Comprehensive analysis of survival from time of diagnosis in a large cohort of patients with PAH suggests considerable improvements in survival in the past 2 decades since the establishment of the NIH registry, the effects of which most likely reflect a combination of changes in treatments, improved patient support strategies, and possibly a PAH population at variance with other cohorts.

Trial Registry:  ClinicalTrials.gov; No.: NCT00370214; URL: clinicaltrials.gov.

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