Chest
Original ResearchPulmonary Vascular DiseaseValidation of the Pulmonary Hypertension Connection Equation for Survival Prediction in Pulmonary Arterial Hypertension
Section snippets
Study Population
Patients included in these analyses were enrolled in four double-blind randomized controlled trials and their associated open-label extension study arms13, 14, 15, 16, 17 that investigated two prostaglandin analogs (treprostinil and beraprost) in PAH. Detailed inclusion and exclusion criteria for these clinical trials have been reported previously.13, 14, 15, 16, 17 Briefly, patients were eligible for these clinical trials only if they met the following entry criteria: New York Heart
Baseline Characteristics
A total of 694 patients with PAH were included in these clinical trials. The mean age was 44 ± 15 years. Eighty-one percent (n = 559) were women. A majority of patients were WHO FC III or IV (81%, n = 560). Of the 694 patients, 449 patients had IPAH, HPAH, or anorexigen-associated PAH (65%); 114 patients had connective tissue disease-associated PAH (16%), 122 patients had congenital heart disease-associated PAH (18%), and nine patients had other associated PAH (1%). The mean 6MWD at baseline
Discussion
The PHC equation accurately predicted survival in a prospective IPAH, HPAH, and anorexigen-associated PAH population cohort. This was true despite multiple enrollment criteria and disease severity. The accurate prediction of survival by both the PHC equation and the French equation in this independent cohort supports their use as a survival comparator in the general population cohort studies. The PHC equation also performed well in the subset analyses (1) in sick patients with WHO FC III and
Acknowledgments
Author contributions: Dr Thenappan takes responsibility for the integrity of the work.
Dr Thenappan: contributed to study design, data analyses, and drafting of the manuscript.
Ms Glassner: contributed to drafting the manuscript and manuscript revisions.
Dr Gomberg-Maitland: contributed to study design, data analyses, drafting of manuscript, and manuscript revisions.
Financial/nonfinancial disclosures: The authors have reported to CHEST the following conflicts of interest: Actelion, Gilead,
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Funding/Support: The authors have reported to CHEST that no funding was received for this study.
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