2025 Pulmonary and Critical Care Medicine Fellows Course (INVITE ONLY)-VTEST

VTEST - CHEST PH-ILD

Pdf Summary

This case study by Dr. Victor J. Test explores pulmonary hypertension associated with interstitial lung disease (PH-ILD), focusing on diagnosis, interpretation of testing, treatment, and prognosis.<br /><br />The case involves a 73-year-old man with progressive severe dyspnea and hypoxemia years after COVID-19 pneumonia, now requiring oxygen. His exam reveals signs consistent with right heart strain and interstitial lung disease (ILD), including basilar crackles, clubbing, elevated jugular venous pressure, and echocardiogram findings such as right atrial and ventricular enlargement, elevated right ventricular systolic pressure (80 mm Hg), and paradoxical septal motion. Laboratory tests show elevated pro-NT-BNP (1260 pg/mL) and a severely reduced diffusion capacity, which is a key marker suggesting coexisting pulmonary hypertension in ILD.<br /><br />PH-ILD has a high incidence among ILD patients (14-44%, up to 86% at lung transplant) and significantly worsens prognosis. Mortality increases linearly with pulmonary artery pressure, with median survival of less than one year in patients with severe pulmonary hypertension.<br /><br />Screening strategies include diffusion capacity <40% predicted, FVC/DLCO ratio >1.6, elevated BNP, worsening oxygen desaturation, and imaging showing right heart dilation. Right heart catheterization confirms the diagnosis.<br /><br />Treatment options emphasize inhaled treprostinil, which has been shown in the INCREASE trial to improve outcomes in PH-ILD. Other agents like endothelin receptor antagonists (ambrisentan, bosentan), riociguat, and calcium channel blockers have not demonstrated benefit and may worsen outcomes. Sildenafil’s efficacy remains unconfirmed.<br /><br />In summary, recognizing and screening for PH-ILD in ILD patients is vital due to its prevalence and mortality risk. Inhaled treprostinil is currently the preferred treatment to improve symptoms and prognosis in idiopathic PH-ILD.

Keywords

pulmonary hypertension

interstitial lung disease

PH-ILD

dyspnea

hypoxemia

right heart strain

pro-NT-BNP

diffusion capacity

inhaled treprostinil

INCREASE trial