Helping to facilitate screening and risk assessment in pulmonary arterial hypertension

At Janssen, our mission is to put the needs and well-being of the people we serve as a top priority. As part of the larger healthcare ecosystem, we also recognize our responsibility to listen to and learn from the medical community by working to address unmet needs and identifiable gaps in patient care by providing educational support to HCPs. 

Pulmonary arterial hypertension (PAH) is a rare, progressive disease that causes narrowing of the arteries from the right side of the heart to the lungs, leading to high pressures in the arteries of the lungs.^1-3 PAH currently has no known cure and can progress to right ventricular failure and death.^1,5

For people living with this disease, risk assessment is utilized to evaluate disease progression and inform treatment decisions.⁵ While the need for timely and regular risk assessment in PAH is widely acknowledged, real-world evidence indicates that risk assessment in the clinical setting may be suboptimal; evidence suggests that some physicians encounter barriers to practical implementation, including the complexity of tools and invasive procedures.^6,7  

One of our goals is to help to raise awareness of screening and risk assessment in PAH. Recognizing a need to improve screening for PAH amongst an at-risk patient population, as well as a need for more timely and regular risk assessment for those living with PAH, ⁸ Janssen funded and supported the development of two tools: the REVEAL Lite 2 risk calculator and the DETECT Screening Tool app. 

Coupled with the original REVEAL 2.0 calculator, an analysis showed that REVEAL Lite 2 offers a simplified method of risk assessment using six non-invasive and modifiable variables that may be implemented routinely in clinical practice and helps differentiate PAH patients among low, intermediate and high risk of one-year mortality.⁹ Risk calculators such as REVEAL Lite 2 that assess fewer, non-invasive variables may help physicians expedite and enhance their strategic decision-making.^6,9

Janssen has also provided another resource through funding and developing the DETECT Screening Tool app to assist physicians screening patients for PAH associated with connective tissue disease (referred to as PAH-CTD), who are known to be at a greater risk of developing PAH.¹⁰  PAH associated with CTD is the second most prevalent type of PAH (after idiopathic PAH) in the US and Europe,⁶ affecting up to 15 percent of patients with CTD.¹⁰ Of the CTDs, PAH occurs most commonly in systemic sclerosis (SSc), a chronic, inflammatory, autoimmune disease characterized by endothelial dysfunction and microvascular disease.^11-14 Patients with SSc can develop PAH at any time, and they experience a median delay in diagnosis of 4 years.¹⁵ Prognosis in PAH-SSc is poor and may partially be explained by the delay in diagnosis.¹⁶  

The DETECT screening tool is designed to assist physicians screening patients with SSc with the goal of providing a recommendation as to which patients should be referred to echocardiography (ECG) as a first step, and if applicable, for right heart catheterization (RHC) as a second step for the diagnosis of PAH. Use of this app based on the DETECT algorithm can help HCPs identify patients with SSc who are at risk of developing PAH and may minimize missed PAH-SSc diagnoses.¹⁶ In the international DETECT study, the DETECT algorithm had a 4% (n=3) rate of missed PAH diagnoses compared with 29% (n=24) using the 2015 ESC/ERS Guidelines.¹⁶ The DETECT algorithm is limited for use only by specialists who are treating SSc patients, and who are familiar with PAH as a complication of SSc. It is not intended to replace the medical or professional judgment of healthcare providers. Healthcare professionals using the tool should exercise clinical judgment as to the information they provide to the patient based upon the tool. 

Both REVEAL Lite 2 and the DETECT Screening Tool app are non-invasive tools that have the potential to help inform clinician treatment decisions.^9,16 Our hope is that healthcare professionals and providers will consider integrating these tools into their practice to identify patients who should be screened for PAH, assess risk in the course of their patients’ disease and facilitate disease monitoring.  We look forward to continuing to listen, learn and collaborate with the medical community to continue to find ways to help raise awareness of screening and risk assessment in PAH. 

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