Refining the Definition for “Low Risk” in Pulmonary Arterial Hypertension: Time to Reduce Morbidity and Mortality (JACC Heart Failure August 2026)-Article: Refining the Definition for “Low Risk” in Pulmonary Arterial Hypertension: Time to Reduce Morbidity and Mortality

Article: Refining the Definition for “Low Risk” in Pulmonary Arterial Hypertension: Time to Reduce Morbidity and Mortality

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This study asked whether the current “low-risk” definition in pulmonary arterial hypertension (PAH), based only on having less than 5% 1-year mortality, is too permissive in an era of better treatments. Using patient-level data from 8 randomized clinical trials, the authors analyzed 1,925 PAH patients already classified as low risk by REVEAL 2.0.<br /><br />They proposed a stricter “refined low-risk” definition: less than 5% mortality at 3 years and less than 10% clinical worsening at 1 year. The analysis showed that patients with a REVEAL 2.0 score of 4 met this definition, with 2.6% 3-year mortality and 6.4% 1-year clinical worsening. In contrast, patients with scores 5–6 had worse outcomes, including 6.3% 3-year mortality and 13% 1-year clinical worsening.<br /><br />Clinical worsening included events such as PAH-related hospitalization, lung transplantation, oxygen therapy, significant functional decline, or need for additional PAH medication. The refined low-risk group also had better exercise capacity, lower NT-proBNP, lower heart rate, better hemodynamics, and fewer atrial fibrillation and depression diagnoses.<br /><br />Independent predictors of refined low-risk status were idiopathic PAH, NYHA class I–II, longer 6-minute walk distance, lower heart rate, lower NT-proBNP, and no prior atrial fibrillation. A similar refined definition was also supported using REVEAL Lite 2.0, with a score of 4 identifying patients at lower risk.<br /><br />The authors conclude that low risk in PAH should include both mortality and morbidity, not just short-term survival. They suggest REVEAL 2.0 score 4 may better define truly low-risk patients and could become a more ambitious treatment goal, especially for younger patients with idiopathic PAH.

Meta Tag

Concept Pulmonary Arterial Hypertension

Concept Low-Risk Definition

Concept Three-Year Risk

Concept Clinical Worsening

Concept One-Year Mortality

Keywords

pulmonary arterial hypertension

PAH

low-risk definition

REVEAL 2.0

clinical worsening

mortality risk

risk stratification

REVEAL Lite 2.0

idiopathic PAH

functional capacity

Pulmonary Arterial Hypertension

Low-Risk Definition

Three-Year Risk

Clinical Worsening

One-Year Mortality