Raymond L. Benza, MD, FACC, FAHA, FACP Professor of Medicine Temple University School of Medicine Philadelphia, PA

REVEAL Risk Calculator Allows Primary Care Physicians To Assess Pulmonary Arterial Hypertension Risk

MedicalResearch.com Interview with:

Raymond L. Benza, MD, FACC, FAHA, FACP Professor of Medicine Temple University School of Medicine Philadelphia, PA

Dr. Benza

Raymond L. Benza, MD, FACC, FAHA, FACP
Primary Study Investigator and Professor of Medicine at The Ohio State University

MedicalResearch.com: What is the background for this study? Would you briefly explain the significance of Pulmonary Arterial Hypertension?

Response: Pulmonary arterial hypertension (PAH) is a silently progressive disease with no known cure and is often fatal. It’s a specific form of pulmonary hypertension (PH) that causes the walls of the pulmonary arteries to become thick and stiff, narrowing the space for blood to flow, and causing an increased blood pressure to develop within the lungs. PAH has a variety of etiologies and long-term impact on patients’ functioning as well as their physical, psychological and social wellbeing.

Assessing a patient’s risk of 1-year mortality is a crucial component to the management and treatment of PAH, as the main treatment goal is for patients to achieve a low-risk status. Given the severity of the disease, physicians need to be able to risk stratify patients in order to characterize their disease better, know how to intelligently implement their medications, and when to refer them for lung transplantation.

There are different approaches to assessing risk in PAH, including the use of variables, equations, and calculator tools; however, real-world evidence indicates risk assessment in the clinical setting is suboptimal. This is why we conducted an analysis to determine the validity of the Registry to Evaluate Early and Long-Term PAH Disease Management (REVEAL) Lite 2 risk calculator, an abridged version of the REVEAL 2.0 risk calculator, in patients with PAH. 

MedicalResearch.com: What are the main findings? How can the risk calculator be incorporated into primary care practice?

Response: REVEAL Lite 2 is an abridged version of the REVEAL 2.0 risk calculator that uses six (rather than 13) exclusively noninvasive and modifiable variables, which include functional class (FC), vital signs (systolic BP [SBP] and heart rate), 6-min walk distance (6MWD), brain natriuretic peptide (BNP)/N-terminal prohormone of brain natriuretic peptide (NT-proBNP), and renal insufficiency (by estimated glomerular filtration rate [eGFR])—and was validated in a series of analyses (Kaplan-Meier, concordance index, Cox proportional hazard model, and multivariate analysis).

In total, 2,529 of the 3,515 patients enrolled in the REVEAL Registry™ were eligible for inclusion in this analysis. When REVEAL Lite 2 was applied at the time of patient enrollment (N=3,046 PAH patients), the c-index, known as the concordance index, was 0.71 (95% CI, 0.69-0.73), indicating good discrimination.

The analysis demonstrated that REVEAL Lite 2 offers a simplified method of risk assessment that may be implemented routinely in daily clinical practice and can be used in conjunction with REVEAL 2.0, and differentiates patients among low, intermediate and high risk of one-year mortality. And in today’s COVID-19 environment, REVEAL Lite 2 may also be adopted to use in remote telehealth. 

MedicalResearch.com: What should readers take away from your report?

Response: PAH is a silently progressive disease, and risk assessment can help detect disease progression and may have important implications for patient care, such as guiding treatment decisions and potentially improving outcomes.

Restrictions in data availability, along with time constraints, may make a risk assessment strategy that assesses fewer variables, such as REVEAL Lite 2, more practical than existing methods, and regular risk assessments with REVEAL Lite 2 may be incorporated into routine clinical practice to help clinicians identify modifications in disease progression and offer therapy to meet the specific needs of each patient.

MedicalResearch.com: What recommendations do you have for future research as a result of this work?

Response: Further research is needed around how REVEAL Lite 2 is used within a real-world clinical setting. There are also ongoing efforts to further improve the usability of REVEAL Lite 2 within the clinical setting, including potential incorporation of the tool into electronic medical records.

Our findings also should prompt discussion as to how risk categories are assigned and whether any additional categories are needed to represent patients with risk scores that do not fall into existing categories.

Any disclosures?

General: Dr. Raymond Benza received research support from Janssen Pharmaceuticals, Inc (a Janssen Pharmaceutical Company of Johnson & Johnson) and has served as a paid consultant to the company. 

Funding: The authors of this analysis acknowledge financial support from The American Heart Association , Bayer , The National Heart, Lung, and Blood Institute of National Institutes of Health (NHLBI/NIH), and United Therapeutics.

Role of sponsors: Janssen Pharmaceuticals, Inc is the sponsor of the REVEAL Registry™ and provided funding and support for the analysis presented. Medical writing support was provided by Twist Medical LLC and was funded by Janssen Pharmaceuticals, Inc.


Benza RL, Kanwar MK, et al. Development and validation of an abridged version of the REVEAL 2.0 Risk Score Calculator, REVEAL Lite 2, for use in patients with pulmonary arterial hypertension [published online ahead of print September 1, 2020]. Chest. doi: 10.1016/j.chest.2020.08.2069.



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Last Updated on February 12, 2021 by Marie Benz MD FAAD