ILD as a Cardiopulmonary Disease: Cardiology's Role in Management

Interstitial lung disease (ILD) is frequently framed as a pulmonary problem, but the cardiovascular complications it generates — particularly pulmonary hypertension (PH)— can be as clinically consequential as the lung disease itself. At the Association of Pulmonary Advanced Practice Providers (APAPP) National Conference, held June 28-20 in Colorado Springs, John Giacona, PhD, PA-C, CHC, Assistant Professor in the Department of Applied Clinical Research and the Division of Cardiology at UT Southwestern Medical Center, Dallas, and President-Elect of the Academy of Physician Associates in Cardiology, made the case that cardiology APPs have a defined and underutilized role in ILD care — and that systematic gaps in collaboration between specialties are costing patients.1

"ILD — we view it as just a lung disease, but it really is a cardiopulmonary disease," Giacona said.

He identified 3 core contributions a cardiology APP brings to the ILD multidisciplinary team: screening for pulmonary hypertension, distinguishing cardiac from pulmonary causes of dyspnea, and longitudinal right ventricular surveillance via echocardiography. In his experience, ILD patients frequently present first to cardiology with symptoms initially attributed to heart failure or other cardiac causes — meaning the cardiology encounter may represent an unrecognized opportunity for earlier diagnosis and routing. He noted that collaboration quality varies substantially by institution and geography, with audience members at APAP highlighting significant deficiencies at their own institutions, and APPs in rural settings or without access to a pulmonary hypertension center facing the steepest barriers.

Once PH is suspected, distinguishing WHO group 2 (left heart disease) from group 3 (lung disease–related) pulmonary hypertension is clinically essential, since standard PAH therapies have demonstrated harm in the ILD setting, and management diverges meaningfully by type.2 Inhaled treprostinil, the only therapy currently approved for pulmonary hypertension associated with ILD (PH-ILD), has robust evidence from the INCREASE trial — a phase 3, randomized, placebo-controlled, 16-week study in 326 patients that demonstrated a 31-meter improvement in 6-minute walk distance, a 15% reduction in NT-proBNP levels with treprostinil versus a 46% increase with placebo, and a reduction in clinical worsening events.3 Giacona also flagged that amiodarone use in patients with atrial fibrillation confers elevated ILD risk — 1 of several medication-related ILD triggers that make cross-specialty vigilance important.3

The persistent challenge is identifying which patients have PH-ILD in the first place. Standardized screening algorithms have historically been lacking, with clinical suspicion relying on signals such as symptoms disproportionate to the degree of fibrosis or desaturation on exertion. The recently completed PHINDER study (NCT05776225), a prospective multicenter observational study, published preliminary results in early 2026 suggesting that a combination of pulmonary function testing, lung imaging, and echocardiography may support earlier and more accurate PH detection in ILD — a potential step toward the systematic screening protocol the field has needed.4 Giacona expressed hope that the final PHINDER data will prompt development of an expert consensus document to guide institutional screening pathways.

His vision for a minimum viable MDT in ILD: pulmonology, cardiology, and radiology as the core, with rheumatology added when underlying autoimmune disease is driving the ILD — he cited scleroderma as a prime example — or when patients are on antifibrotics or immunosuppressive biologics. "The onus is on any one provider who gets a patient with ILD or pulmonary hypertension to ensure that patient is plugged in with all the appropriate specialists," he said.

Giacona has no relevant disclosures to report.

References

1. Giacona J, Glennie J, Mixon A, Smith D. Bridging Specialties in ILD: A Multidisciplinary Bootcamp Series for Early Recognition and Targeted Treatment. Presented at APAPP 2026 National Conference, June 18-20; Las Vegas, Nevada.

2. Cottin V, Martinez FJ, Smith V, Walsh SLF. Multidisciplinary teams in the clinical care of fibrotic interstitial lung disease: current perspectives. Eur Respir Rev. 2022;31(166):220003. doi:10.1183/16000617.0003-2022

3. 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

4. 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