Pulmonary Arterial Hypertension Highlights

Pulmonary arterial hypertension (PAH) is a chronic and progressive condition with significant clinical symptoms, poor quality of life, and early mortality. According to DelveInsight’s epidemiology analysis for PAH, there were nearly 69,605 prevalent cases in the 7MM [the US, EU-5 (Germany, France, Italy, Spain, and the UK), and Japan] in the year 2021. The estimations suggest a rise in cases in future years.

Over the last decade, the treatment and understanding of PAHs has improved significantly. However, there is much to be learned about the clinical course of both idiopathic PAH and associated PAH, including clinical symptoms, rate of progression, important parameters to monitor, effects of treatment, predictors of outcome, among others.

At the American Thoracic Society (ATS) International Conference 2022 held in San Francisco, California, real-world treatment and outcomes relating to the advancements in the PAH management and well-established treatment guidelines to improve the PAH prognosis in patients were discussed.

One of the key topics detailed was the Comprehensive Patient Record (CIPDR) a new type of comprehensive, integrated patient data repository. Registries contribute significantly to the knowledge of PAH epidemiology, risk factors, prognosis and treatment and CIDPR was created to enable this assessment of actual treatment and outcomes.

The TRIO CIPDR was created under the guidance of six pulmonologists.  CIPDR contains data elements on demographics, illness, comorbidities, test data, respiratory function tests, functional status, PAH treatment, reasons for discontinuation / switching of treatment, hospitalization, and death collected through the HIPAA secure online form. 11 Pulmonary Hypertension Association certified care centers initially contributed to the CIPDR though 2 centers were unable to continue participation due to COVID-19 impact.

To facilitate the enrolment, specialty pharmacy data corresponding to each site were used to identify potential patients and prepopulate qualification forms. Each site reviewed and qualified patients who met repository criteria: age >18 years, prescribed PAH specific medications, and confirmed of PAH diagnosis by right heart catheterization (mean Pulmonary Arterial Pressure ≥25mmHg, Pulmonary Capillary Wedge Pressure ≤15 mmHg, and Pulmonary Vascular Resistance ≥3.0 Wood Units at rest). The initial data collection included care encounters between Jan 2019 and Dec 2020 and data concerning diagnosis, onset of symptoms, procedures, and laboratory values closest to enrolment. After completion of data collection, all data were reviewed by Trio Health and adjudicated with each site.

Of the 3200 potentially qualified patients in the CIPDR Registry, 1009 were enrolled encompassing a total of 4489 visits, including 3103 office visits and 1386 telemedicine visits. Of all the enrolment, majority patients (83%) underwent Echocardiography examination as it provides important prognostic data in the assessment of pulmonary vascular hemodynamic and right heart load, followed by 81% patients who underwent 6 minute walk distance as an inexpensive, easy-to-use, reproducible, standardized test and well tolerated by PAH patients. RHC and CT Scans were preferred by almost 50% of the enrolled patient, whereas Serologic Tests was the least preferred diagnostic choice. The diagnosis of suspicious PH / PAH is based on non-invasive diagnostic imaging, but a confirmatory diagnosis with a right cardiac catheter (RHC) is essential. Amongst all the data recorded through various diagnostic modalities a major patient pool received a combination of diagnostic approaches to ensure complete PAH evaluation as ignorance among clinicians about the importance of early and accurate PAH diagnosis is persistent.

Of all the confirmed and clinically diagnosed patients with PAH, Tadalafil was the most preferred treatment option that was well tolerated and had improved QoL measures by reducing clinical worsening. Macitentan (orphan drug in treating PAH) (new dual endothelin receptor antagonist) was the second preferred treatment choice in improving the prognosis of PAH patients by delaying the progression of the disease. Along with the above-mentioned therapies, Treprostinil, Selexipag, Sildenafil, Ambrisentan, and Riociguat have successfully shown improved prognosis of PAH by delaying the progression of the disease and managing the condition by preventing permanent damage to the pulmonary arteries. Ever-increasing experience in treating PAH, coupled with the availability of evolving treatment options, has led to improvements in patient outcomes.

In recent years, we have come a long way in understanding pulmonary hypertension and pulmonary arterial hypertension. Patient outcomes have continuously improved as a result of increased experience in the treatment of pulmonary arterial hypertension (PAH) and the availability of new treatment options. As patients live longer with the disease, the focus of the CIPDR repository is on assessing the impact of PAH on quality of life and increasing awareness that PAH management should aim to mitigate this impact.

The overall study of the Trio CIPDR repository is an important step in clearly characterizing the patient's journey, treatment patterns, and outcomes for PAH patients.

According to DelveInsight, the market of PAH looks quite interesting with several patents expiry and newer therapies coming in market, overall market is anticipated to witness a boost. The particular frame of understanding generated through the TRIO CIPDR grows, new understandings, insights, and remedies will emerge to enhance the lives of sufferers with PAH.

For more detailed analysis, visit: Pulmonary Arterial Hypertension market