Health Tech

Overcoming Technological Barriers in Adopting Pulmonary Arterial Hypertension Risk Assessment Tools

By Sean Studer, MD, MSc, Vice President, Medical Affairs, Pulmonary Hypertension at the Janssen Pharmaceutical Companies of Johnson & Johnson

Pulmonary arterial hypertension (PAH) is a rare, progressive disease with a traditionally poor prognosis.1-3 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.3-5 Over time, PAH can lead to right ventricular failure and death.1,3 Currently, there is no known cure.1,2

Because PAH is progressive, ongoing risk assessment is an essential part of PAH management. Once diagnosed, the overall treatment goal for people with PAH is to achieve and maintain a low-risk status (risk of one-year mortality <5%), which has been associated with improved outcomes.1,6

Despite the clinical need for risk assessment in PAH populations, a recent survey published in Pulmonary Circulation7 of 121 PAH treatment decision-makers shows that only 59 percent reported using risk assessment tools in general, with fewer than two-thirds of physicians and fewer than half of healthcare professionals (HCPs), such as nurse practitioners and physician assistants, reporting the use of tools.7

When Clinical Judgment Alone Is Not Enough 

Research has found that there are differences between physicians’ clinical judgment of patient risk as calculated using an objective algorithm.8 While clinical judgment can play an important role in clinical practice, research shows that 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,9 and that relying on clinical judgment can lead to over- or underestimation of risk when compared with assessments made using objective methods.7,8,10  

A retrospective chart analysis of PAH patients considered low risk by clinical judgment shows that 11% to 36% were categorized as intermediate risk and 4% to 28% were categorized as high risk when assessed using various formal risk assessment tools.7 The analysis noted that a large proportion of World Health Organization (WHO) functional class (FC) II patients (who are often considered to be at low risk for disease progression, as they have only mild physical activity limitations) are at intermediate or high risk for disease progression when assessment is performed with standard risk assessment tools, and may require more intensive therapy.7,10

The Use of Standard PAH Risk Assessment Tools 

One of Janssen’s primary goals is to help improve the lives of people living with PAH. Janssen is introducing tools and educational resources for healthcare teams that may help them address and overcome barriers to delivering PAH care. 

One existing resource is the US Registry to Evaluate Early and Long-term PAH Disease Management (REVEAL).11 The REVEAL Registry is a multicenter, observational, US-based study of the clinical course and disease management of PAH patients.11 All consecutive consenting patients diagnosed with PAH (WHO Group I), according to specific hemodynamic criteria at participating institutions, were enrolled and followed for a minimum of five years from the time of enrollment. REVEAL is one of the largest and longest running PAH registries utilized to date.11,12

Based on data analysis from the REVEAL registry, Janssen supported the development of two PAH risk assessment tools: the REVEAL 2.0 and REVEAL Lite 2 calculators.12,13

REVEAL Lite 2, which is an abridged version of REVEAL 2.0 and is intended to supplement REVEAL 2.0, can enable risk assessment to be expedited in the clinical setting without compromising validity through the use of six non-invasive and modifiable variables that can be easily collected at every clinic visit (functional class, vital signs [both systolic blood pressure and heart rate], 6-minute walk distance, BNP or NT-proBNP levels, and renal insufficiency by estimating the glomerular filtration rate).13 The non-invasive and modifiable variables used with REVEAL Lite 2 may be implemented routinely in clinical practice to help differentiate PAH patients among low, intermediate and high risk of one-year mortality.13

Combatting Technological Barriers 

Despite the demonstrated benefit of formal PAH risk assessment tools, the Pulmonary Circulation survey shows that the widespread adoption of such tools by healthcare professionals has been hindered, in part due to lack of technical integration within health system technology platforms.

Based on this evidence, Janssen developed an educational resource called the Electronic Health Record (EHR) Build Guide. This build guide aims to help educate health systems so that they can better implement the REVEAL 2.0 and REVEAL Lite 2 risk calculators into their health system’s Cerner or Epic EHR system. This digital resource is available online here

Incorporating the REVEAL 2.0 and REVEAL Lite 2 risk-assessment calculators into an organization’s EHR may enable health systems to have a standardized, more readily available tool for risk stratification of PAH patients. 

The reality is that PAH remains a serious, progressive disease with no cure.1-3,13 When nearly one in three patients dies within five years of diagnosis,14 there’s more work to be done. We are committed to communicating with the HCP community to do that work. 


©2021 Actelion Pharmaceuticals US, Inc. All rights reserved. cp-249066v1 1/22

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.

References
1. 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.
2. Vachiéry JL, Gaine S. Challenges in the diagnosis and treatment of pulmonary arterial hypertension. Eur Respir Rev. 2012;21(126):313-320.
3. Hoeper MM, Gibbs JSR. The changing landscape of pulmonary arterial hypertension and implications for patient care. Eur Respir Rev. 2014;23(134):450-457.
4. 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.
5. Schermuly RT, et al. Mechanisms of disease: pulmonary arterial hypertension. Nat Rev Cardiol. 2011;8(8):443-455.
6. Galie N, McLaughlin VV, et al. Risk stratification and medical therapy of pulmonary arterial hypertension. Eur Respir J. 2019;53(1):1802148.
7. 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.
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. Gaine S, McLaughlin V. Pulmonary arterial hypertension: tailoring treatment to risk in the current era. Eur Respir Rev. 2017;26(146):170095.
10. 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. 
11. Benza RL, Miller DP, Gomberg-Maitland M, et al. Predicting survival in pulmonary arterial hypertension insights from the Registry to EValuate EArly and Long-term pulmonary arterial hypertension disease management (REVEAL). Circulation. 2010;122(2):164-172.
12. 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.
13. 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.
14. Hoeper MM, Kramer T, Pan Z, et al. Mortality in pulmonary arterial hypertension: prediction by the 2015 European pulmonary hypertension guidelines risk stratification model. Eur Respir Rev. 2017;50(2):1700740.

The editorial staff had no role in this post's creation.