Overcoming Barriers to Using Risk Assessment Tools in Pulmonary Arterial Hypertension

Medical understanding of pulmonary arterial hypertension (PAH) has come a long way in the last 20 years.1,2 Today, the medical community has a much clearer picture of the pathophysiology of this disease.1

PAH is a rare, progressive disease with a traditionally poor prognosis.2-4 It causes narrowing of the arteries from the right side of the heart to the lungs, leading to high pressure in the arteries of the lungs.4-6 Over time, PAH can progress to right ventricular failure and death.3,4 Currently, there is no known cure.2,3

Once diagnosed, the overall treatment goal for patients with PAH is to achieve and maintain a low-risk status (risk of one-year mortality <5%), which has been associated with improved outcomes.3,7

Fortunately, tools are available to help healthcare professionals assess risk in PAH patients, which can aid in decisions about when to escalate therapy.2-4,7 Yet real-world evidence indicates that some physicians encounter barriers to practical PAH risk assessment implementation,8,9 and treatment and management of PAH remains suboptimal.10 The majority of patients with PAH experience substantial delays between initial onset of symptoms and clinical diagnosis of the disease.11

Standardizing PAH Risk Assessment

Today, many healthcare providers (HCPs) indicate that they assess risk in PAH patients primarily by relying on clinical judgment.8,9 While clinical judgment can play an important role in clinical practice, use of a standardized, structured PAH risk assessment tool is crucial in ensuring that patients’ degree of disease progression is being adequately measured and tracked,12 and that relying on clinical judgment can lead to over- or underestimation of risk when compared with assessments made using objective methods.8,9

In fact, one retrospective analysis showed that, of patients considered low risk by clinical judgment, 11% to 36% were categorized as intermediate risk and 4% to 28% were categorized as high risk when assessed using formal risk assessment tools.9 The unintended consequence of relying on clinical judgment can be that patients in intermediate or higher risk categories who may require treatment escalation to achieve low-risk status may not be escalated as quickly as necessary, which can impede outcomes.9,13

To help HCPs assess PAH risk, Janssen supported the development of the US Registry to Evaluate Early and Long-term PAH Disease Management (REVEAL 2.0) calculator, a clinically validated tool to assess risk status of disease progression in patients with PAH, including those newly diagnosed and previously diagnosed.14,15 Janssen then supported the development of the complementary REVEAL Lite 2 to help inform physicians’ decision-making. REVEAL Lite 2 applies six non-invasive, modifiable variables that may be implemented routinely in clinical practice to help differentiate PAH patients among low, intermediate, and high risk of one-year mortality.15

The full REVEAL 2.0 score should be used at baseline, four to six months, and during annual evaluations in treatment-naïve patients. The abridged REVEAL Lite 2 calculator may be used in between these time points and is intended to complement, rather than replace, REVEAL 2.0.15

Risk calculators such as REVEAL Lite 2 that assess fewer non-invasive variables may help physicians facilitate regular, consistent assessment of patient risk status, which can lead to improved management of PAH.15

Integrating PAH Risk Assessment Tools into EHRs

Despite the availability of such tools, a recent survey of 121 PAH decision-makers revealed another barrier to widespread and consistent HCP adoption of formal PAH risk assessment tools is the lack of technological integration within their healthcare systems.9

Based on this evidence, the company created instructional resources for health systems with input from clinicians to help educate health systems so that they can better integrate PAH risk assessment tools into their health system’s electronic health records (EHRs). These build guides offer step-by-step instructions to build the REVEAL 2.0 and REVEAL Lite 2 risk calculators into Cerner or Epic EHR systems, enabling health systems to have a standardized, more readily available tool for risk stratification of PAH patients. This resource is available here.

“Our goal is to help educate health systems so that they can better integrate PAH risk assessment into their EHRs amongst a PAH rare disease population, which can have an impact on their prognosis and outcomes,” said Sean Studer, Vice President, Medical Affairs, Pulmonary Hypertension at the Janssen Pharmaceutical Companies of Johnson & Johnson.

The reality is that PAH remains a serious, progressive disease with no cure.15 Janssen is committed to working with the healthcare community to help change that.

Disclaimer: The purpose of the EHR Build Guides is to serve as an educational resource for HCPs. Each practice is solely responsible for implementing, testing, monitoring and ongoing operation of their EHR tools. The instructions are not intended to replace a health system’s processes or protocols and are not intended to be an endorsement or recommendation.

© 2021 Actelion Pharmaceuticals US, Inc. All rights reserved. cp-249067v1 11/21


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2.            Vachiéry JL, Gaine S. Challenges in the diagnosis and treatment of pulmonary arterial hypertension. Eur Respir Rev. 2012;21(126):313-320.

3.            Galiè N, Humbert M, Vachiery JL, et al. 2015 ESC/ERS guidelines for the diagnosis and treatment of pulmonary hypertension. Eur Heart J. 2016;37(1):67-119.

4.            Hoeper MG, Gibbs SR. The changing landscape of pulmonary arterial hypertension and implications for patient care. Eur Respir Rev. 2014;23(134):450-457.

5.            McLaughlin VV, et al. ACCF/AHA 2009 expert consensus document on pulmonary hypertension: a report of the American College of Cardiology Foundation Task Force on Expert Consensus Documents and the American Heart Association: developed in collaboration with the American College of Chest Physicians, American Thoracic Society, Inc., and the Pulmonary Hypertension Association. Circulation. 2009;119(16):2250-2294.

6.            Schermuly RT, et al. Mechanisms of disease: pulmonary arterial hypertension. Nat Rev Cardiol. 2011;8(8):443-455.

7.            Galie N, McLaughlin VV, et al. Risk stratification and medical therapy of pulmonary arterial hypertension. Eur Respir J. 2019;53(1):1802148.

8.            Simons JE, Mann EB, Pierozynski A. Assessment of risk of disease progression in pulmonary arterial hypertension: insights from an international survey of clinical practice. Adv Ther. 2019;36(9):2351-2363.

9.            Wilson M, Keeley J, Kingman M, Wang J, Rogers F. Current clinical utilization of risk assessment tools in pulmonary arterial hypertension: a descriptive survey of facilitation strategies, patterns, and barriers to use in the United States. Pulm Circ. 2020;10(3):2045894020950186.

10.          Simonneau G, Hoeper M, et al. Future perspectives in pulmonary arterial hypertension. Eur Respir Rev. 2016;25(142):381-389.

11.          Brown L, Elliott C. Time to diagnosis of pulmonary arterial hypertension. Adv Pulm Hypertens. 2012;11(2):69-72.

12.          Gaine S, McLaughlin V. Pulmonary arterial hypertension: tailoring treatment to risk in the current era. Eur Respir Rev. 2017;26(146):170095.

13.          Sahay S, Tonelli A, Selej M, et al. Risk assessment in patients with functional class II pulmonary arterial hypertension: comparison of physician gestalt with ESC/ERS and the REVEAL 2.0 risk score. PLoS One. 2020;15(11):e0241504.

14.          Benza RL, Gomberg-Maitland M, Miller DP, et al. The REVEAL Registry risk score calculator in patients newly diagnosed with pulmonary arterial hypertension. Chest. 2012;141(2):354-362.

15.          Benza RL, Kanwar MK, Raina A, 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. Chest. 2021;159(1):337-346.

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