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Poster Presentations: Wednesday, October 26, 2011 |

Impact of Functional Class Change on Survival in Patients With Pulmonary Arterial Hypertension Associated With Congenital Heart Disease: Insights From REVEAL FREE TO VIEW

Robyn Barst, MD; D. Dunbar Ivy, MD; Robert Frantz, MD; A. Frost, MD; Erika Rosenzweig, MD; Aimee Foreman, MA; S. Shapiro, PhD; Darren Taichman, PhD; Michael McGoon, MD
Chest. 2011;140(4_MeetingAbstracts):716A. doi:10.1378/chest.1118232
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Abstract

PURPOSE: The Registry to EValuate Early And Long-term Pulmonary Arterial Hypertension (PAH) Disease Management (REVEAL), a 55-center observational, US study, provides current demographic, clinical, and treatment patterns in patients with PAH, including the PAH subgroup congenital heart disease-associated PAH (APAH-CHD). This analysis investigates the association between NYHA functional class (FC) and survival in APAH-CHD.

METHODS: Of the 374 APAH-CHD patients enrolled in REVEAL, 158 were FC III at enrollment; of these, 105 (66%; repaired [n = 29] and unrepaired/partially repaired [n = 76]) had ≥1 follow-up FC assessment within the first year after enrollment (at 3-, 6-, or 12-months). At enrollment, 7 patients were aged ≤18 years and 98 were >18 years. Patients were classified at first follow-up as follows: 1) “Improved” FC III improved to FC I/II; 2) “Unchanged” remained FC III; or 3) “Deteriorated” worsened to FC IV. Descriptive data is reported as mean ± SD. Two-year survival estimates of these 3 subgroups were compared from time of first follow-up FC assessment using the log-rank test.

RESULTS: Of these 105 APAH-CHD patients, 30% (n = 32) improved, 62% (n = 65) were unchanged, and 8% (n = 8) deteriorated at first follow-up (mean time to first follow-up, 4.5 ± 2.8 months). Among demographic, clinical, and hemodynamic parameters at enrollment, only 6-minute walk distance (6MWD; total cohort, 351 ± 105 m) was different among subgroups (improved, 394 ± 115 m; unchanged, 337 ± 95 m; deteriorated, 307 ± 109 m; P = 0.018). At first follow-up, the difference in 6MWD was even greater (improved to FC I/II, 434 ± 61 m; unchanged FC III, 342 ± 91 m; deteriorated to FC IV, 222 ± 90 m; P <0.001). Two-year survival estimates from first FC follow-up were 97 ± 3%, 86 ± 4%, and 50 ± 18% for improved (FC I/II), unchanged (FC III), and deteriorated (FC IV) patients, respectively (overall log-rank P = 0.003, follow-up FC I/II vs FC III P = 0.026).

CONCLUSIONS: These results suggest that APAH-CHD patients who improve from FC III to FC I/II have a better two-year survival compared with patients who remain FC III.

CLINICAL IMPLICATIONS: These data suggest that goal-oriented therapy should aim for FC I/II in patients with APAH-CHD who are FC III.

DISCLOSURE: Robyn Barst: Consultant fee, speaker bureau, advisory committee, etc.: Actelion, Bayer, GlaxoSmithKline, GeneraMedix, Gilead, Eli Lilly & Co., MondoBIOTECH, NIH/NHLBI, Novartis, and Pfizer, Grant monies (from industry related sources): Actelion, Gilead, Eli Lilly & Co., Novartis, Pfizer, and United Therapeutics, Grant monies (from sources other than industry): NIH/NHLBI

D. Dunbar Ivy: Consultant fee, speaker bureau, advisory committee, etc.: Actelion, Gilead, Pfizer & United Therapeutics

Robert Frantz: Consultant fee, speaker bureau, advisory committee, etc.: Actelion, Gilead, and United Therapeutics, Grant monies (from industry related sources): Actelion, Gilead, and United Therapeutics

A. Frost: Consultant fee, speaker bureau, advisory committee, etc.: Actelion, Gilead and Pfizer, University grant monies: Grants received through Baylor from Gilead, Actelion, United Therapeutics, Eli Lilly, Pfizer, Novartis and Bayer

Erika Rosenzweig: Consultant fee, speaker bureau, advisory committee, etc.: United Therapeutics, Actelion, Gilead, Grant monies (from industry related sources): Actelion, Gilead, United Therapeutics, Novartis, Pfizer, Eli Lilly, Bayer

Aimee Foreman: Other: Employed by ICON Late Phase and Outcomes Research, a company that receives research support from Actelion and other pharmaceutical companies

S. Shapiro: Grant monies (from industry related sources): Actelion, Pfizer, Gilead, United Therapeutics, and Bayer, Consultant fee, speaker bureau, advisory committee, etc.: Actelion, United Therapeutics, and Gilead

Darren Taichman: Grant monies (from industry related sources): Actelion

Michael McGoon: Consultant fee, speaker bureau, advisory committee, etc.: Actelion, Gilead, Lung Rx, and Medtronic, Grant monies (from industry related sources): Gilead and Medtronic

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