Charles Fauvel, Priscilla Correa Jaque, Yongqi Liu, Sang Wan Lee, Sandeep Sahay, Nelson A. Villasmil Hernandez, Jason Weatherald, James White, Allen Everett, Megan Griffith, Thomas M. Cascino, Adam Perer, Katelyn Morrison, Michael Lewis, Yuri Matusov, Shili Lin, Raymond L. Benza
Rouen University Hospital and French National Institute for Health and Medical Research. Eastern Virginia Medical School, Old Dominion University and Sentara Health. Ohio State University. Houston Methodist Hospital. University of Alberta. University of Rochester. Johns Hopkins University. University of Texas Southwestern. University of Michigan. Carnegie Mellon University. Cedars Sinai Medical Center.
France, United States and Canada
Journal of the American College of Cardiology Heart Failure
JACC Heart Fail 2026;
DOI: 10.1016/j.jchf.2026.103151
Abstract
Background: “Low-risk” pulmonary arterial hypertension (PAH) patients are defined as having a risk of death <5% within 1 year. As treatment improves, accounting for the negative impact of short-term morbidity and considering extending the duration of <5% mortality in this definition seem prudent.
Objectives: This study sought to propose a more stringent definition of low risk that extends the low mortality risk to 3 years and specifies a low risk of morbidity at 1 year.
Methods: This study used patient-level data from a harmonized data set of 8 PAH randomized controlled trials that included patients with a REVEAL (Registry to Evaluate Early and Long-Term PAH Disease Management) 2.0 risk score ≤6 (ie, low risk using contemporary scores). Low risk was a priori defined as “risk of death <5% at 3 years and risk of clinical worsening <10% at 1 year.” Clinical worsening was defined at the individual trial level and included any of the following: lung transplantation, hospitalization for PAH, initiation of long-term oxygen therapy, 15% decrease in 6-minute walking distance (6MWD) from baseline with a worsening of NYHA functional class, or the addition of a new PAH medication.
Results: A total of 1,925 PAH patients were included: median age, 46 years (Q1-Q3: 34-57 years); mean pulmonary artery pressure, 47 mm Hg (Q1-Q3: 37-58 mm Hg); pulmonary vascular resistance, 8 WU (Q1-Q3: 6-12 WU), 6MWD, 409 m (Q1-Q3: 363-423 m); and N-terminal pro-B-type natriuretic peptide, 227 pg/mL (Q1-Q3: 101-621 pg/mL). Compared with patients with a REVEAL 2.0 score equal to 5 or 6, patients with a score ≤4 had a better clinical and hemodynamic presentation and met the definition of “refined low risk” (3-year mortality of 2.6% and 1-year clinical worsening of 6.4%). Idiopathic PAH, 6MWD, N-terminal pro-B-type natriuretic peptide, NYHA functional class I to II, heart rate, and the absence of previous atrial fibrillation were associated with refined low risk.
Conclusions: This study is the first to embed both morbidity and mortality within the definition of low -risk for PAH and suggests that patients with a REVEAL 2.0 risk score ≤4 define this condition
Category
Diagnostic Testing for Pulmonary Vascular Disease. Risk Stratification
Age Focus: Adult Pulmonary Vascular Disease
Fresh or Filed Publication: Fresh (PHresh). Less than 1-2 years since publication
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