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Original Research: Pulmonary Vascular Disease |

Functional Class Improvement and 3-Year Survival Outcomes in Patients With Pulmonary Arterial Hypertension in the REVEAL RegistryFunctional Class Improvement and Survival Outcomes

Robyn J. Barst, MD; Lorinda Chung, MD; Roham T. Zamanian, MD; Michelle Turner, MS; Michael D. McGoon, MD
Author and Funding Information

From the Columbia University College of Physicians and Surgeons (Dr Barst), New York, NY; the Vera Moulton Wall Center for Pulmonary Vascular Disease (Dr Zamanian), the Stanford University School of Medicine (Drs Chung and Zamanian), Stanford, CA; the Palo Alto VA Health Care System (Dr Chung), Palo Alto, CA; ICON Late Phase & Outcomes Research (Ms Turner), San Francisco, CA; and the Mayo Clinic (Dr McGoon), Rochester, MN. †Died April 2013.

Correspondence to: Michael McGoon, MD, Mayo Clinic, Department of Cardiovascular Diseases, 200 1st St SW, Rochester, MN 55902; e-mail: mmcgoon@mayo.edu


Funding/Support: Funding and support for the REVEAL Registry were provided by CoTherix, Inc, and its affiliate, 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. 2013;144(1):160-168. doi:10.1378/chest.12-2417
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Objective:  New York Heart Association/World Health Organization functional class (FC) is associated with outcomes in pulmonary arterial hypertension (PAH). We assessed whether patients with PAH who improve from FC III to FC I/II have improved survival vs patients who remain at FC III or worsen to FC IV.

Methods:  Patients aged ≥ 19 years with FC III PAH from the Registry to Evaluate Early and Long-Term PAH Disease Management (REVEAL Registry) (N = 982) were categorized as improved, unchanged, or worsened according to their change in FC from enrollment to first follow-up assessment within 1 year of enrollment. Kaplan-Meier estimates of 3-year survival from first follow-up and changes in 6-min walk distance (6MWD) from enrollment to first follow-up were determined. Subgroup analyses were conducted by cause (ie, idiopathic/familial, connective tissue disease [CTD], congenital heart disease) and time of diagnosis (ie, newly or previously diagnosed [diagnostic right-sided heart catheterization within or ≥ 3 months of enrollment, respectively]).

Results:  Overall, 27% of patients improved FC. Survival was better in patients whose FC improved (84% ± 2%, n = 263) vs those who remained unchanged (66% ± 2%, n = 645) or worsened (29% ± 6%, n = 74) (all P < .001). Survival was also better in patient subgroups whose FC improved vs those who remained unchanged (idiopathic/familial [P < .001], CTD-associated PAH [P = .009], whether newly [P = .004] or previously diagnosed [P < .001]). 6MWD improvements were greater in patients whose FC improved vs those who remained unchanged in the overall (P < .001) and CTD (P = .028) cohorts.

Conclusion:  Patients with PAH who improve from FC III to I/II, whether newly or previously diagnosed and regardless of PAH cause, have better survival vs patients who remain FC III.

Trial registry:  ClinicalTrials.gov; No.: NCT00370214; URL: www.clinicaltrials.gov

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