Robyn J Barst1, Lorinda Chung2, Roham T Zamanian3, Michelle Turner4, Michael D McGoon5. 1. Columbia University College of Physicians and Surgeons, New York, NY. 2. Stanford University School of Medicine, Stanford, CA; Palo Alto VA Health Care System, Palo Alto, CA. 3. Vera Moulton Wall Center for Pulmonary Vascular Disease, Stanford, CA. 4. ICON Late Phase & Outcomes Research, San Francisco, CA. 5. Mayo Clinic, Rochester, MN. Electronic address: mmcgoon@mayo.edu.
Abstract
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.
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.
Authors: Lea Ann Matura; Corey E Ventetuolo; Harold I Palevsky; David J Lederer; Evelyn M Horn; Stephen C Mathai; Diane Pinder; Christine Archer-Chicko; Emilia Bagiella; Kari E Roberts; Russell P Tracy; Paul M Hassoun; Reda E Girgis; Steven M Kawut Journal: Ann Am Thorac Soc Date: 2015-03
Authors: R Mark Grady; Matthew W Canter; Fei Wan; Anton A Shmalts; Ryan D Coleman; Maurice Beghetti; Rolf M F Berger; Maria J Del Cerro Marin; Scott E Fletcher; Russel Hirsch; Tilman Humpl; D Dunbar Ivy; Edward C Kirkpatrick; Thomas J Kulik; Marilyne Levy; Shahin Moledina; Delphine Yung; Pirooz Eghtesady; Damien Bonnet Journal: J Am Coll Cardiol Date: 2021-08-03 Impact factor: 27.203