Cardiology's Role in Identifying Pulmonary Hypertension in ILD

Pulmonary hypertension complicating interstitial lung disease (PH-ILD) carries a markedly elevated risk of hospitalization and clinical worsening compared with ILD alone. Inhaled treprostinil remains the only approved therapy for PH-ILD, a status confirmed by the FDA in 2021 following the phase 3 INCREASE trial. The 326-participant trial showed inhaled treprostinil improved 6-minute walk distance by 31.12 m over placebo at week 16 and reduced NT-proBNP levels by 15%, compared with a 46% increase in the placebo arm.¹

Other pulmonary arterial hypertension (PAH) therapies have shown harm when applied to ILD populations, underscoring the importance of a therapy purpose-built for this overlap. Identifying candidates for treatment remains the larger obstacle, since no standardized screening pathway for PH-ILD currently exists. The ongoing PHINDER study is working to close this gap by evaluating pulmonary function testing, imaging, and echocardiography as tools to flag precapillary PH before right heart catheterization.²

A cardiology perspective can sharpen this detection process, distinguishing cardiac from pulmonary causes of worsening dyspnea and screening for right ventricular dysfunction pulmonology-only evaluation may miss. Collaboration between the two specialties still varies widely by institution, with rural centers sometimes lacking access to pulmonary hypertension programs altogether.

John Giacona, PhD, PA-C, CHC, an assistant professor in the Applied Clinical Research Department with a secondary appointment in the Division of Cardiology at UT Southwestern Medical Center, brings a hypertension specialist's viewpoint to this overlap. In the following interview, Giacona spoke with HCPLive at the APAPP 2026 National Conference in Las Vegas about where cardiology and pulmonology intersect in PH-ILD screening and management.

HCPLive: What cardiology perspective might pulmonary specialists miss in ILD recognition and management, and where are the gaps between the two fields?

John Giacona, PhD, PA-C: I think there's an opportunity for multidisciplinary treatment in patients with ILD. I don't come from a pulmonology viewpoint, but from a cardiology viewpoint, having a cardiologist or cardiology APP can provide significant contributions to the team and to the patient. One is screening for pulmonary hypertension. Another is distinguishing cardiac from pulmonary causes of dyspnea, especially if the patient has worsening symptoms, and longitudinal surveillance of the heart on echocardiogram, particularly the right ventricle. ILD patients frequently present first to cardiology, at least in my experience, where their symptoms are initially attributed to something like heart failure with preserved ejection fraction. Multidisciplinary assessment is the accepted diagnostic approach to interstitial lung disease, and I think the cardiologist can help determine whether that dyspnea is left heart disease, which would be group 2 pulmonary hypertension, whether it's pulmonary vascular disease, which is group 3, or both. Knowing which type of pulmonary hypertension the patient has is fundamental because it changes management.

Is there a need for better collaboration between cardiology and pulmonology, or is current coordination adequate?

Giacona: It very much depends on region and even institution. My experience at APAPP was that some audience members pointed out huge deficiencies in collaboration at their respective institutions. If they're in a rural area, they may not even have a pulmonary hypertension center, so they may have to refer patients far distances to tertiary centers. Across the board, I think it's probably not as collaborative as it should be. Some institutions may have that set up, but it's a huge need we have not met yet.

How has treprostinil's approval changed the clinical landscape for pulmonary hypertension associated with ILD, and what challenges remain?

Giacona: I do think it has changed the clinical landscape, because patients with pulmonary hypertension associated with ILD are at very high risk for worse outcomes, more hospitalization, and worsening symptoms. With the advent of treprostinil, as you mentioned, it's the only therapy approved right now, and it has robust evidence. The INCREASE trial from 2021 showed a significant improvement in 6-minute walk distance, reduction in heart failure markers like NT-proBNP, and reduction in clinical symptoms at 16 weeks. Another important thing to remember is that other pulmonary arterial hypertension therapies have actually shown harm in ILD, so it's important we have this tool in the toolbox for our patients. The bigger challenge is first identifying patients with pulmonary hypertension associated with ILD. We have the treatment, but we're not great at screening and identifying them. There's no standardized screening pathway. The PHINDER study was designed to try and address this. We have expert consensus on suspecting pulmonary hypertension, particularly when symptoms are disproportionate to degree of fibrosis or degree of desaturation a patient has when they ambulate, but we don't have a systematic algorithm we can advise and employ at many institutions, so it becomes difficult to identify them. It's important we have the right treatment, but we also have to find the right patients for that treatment.

Are guidelines being developed to help identify these patients, and what progress has been made?

Giacona: The PHINDER study had some initial findings presented a couple months ago. I don't have them off the top of my head, but I think once that study is complete, there will likely be some progress. My hope is there's some sort of expert consensus document that comes out of it to help set systematic screening in place.

Editor's Note: This transcript has been edited for grammar and clarity using artificial intelligence tools.

References

1. Waxman A, Restrepo-Jaramillo R, Thenappan T, et al. Inhaled treprostinil in pulmonary hypertension due to interstitial lung disease. N Engl J Med. 2021;384(4):325-334. doi:10.1056/NEJMoa2008470

2. Zisman D, Sahay S, Bandyopadhyay D, et al. Screening for pulmonary hypertension in interstitial lung disease: preliminary results from the PHINDER study. Adv Ther. 2026;43(5):2071-2089. doi:10.1007/s12325-026-03508-4