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
References
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