2025 Pulmonary and Critical Care Medicine Fellows Course (INVITE ONLY)-D LEVINE - CHEST FELLOW PH ILD 2025

D LEVINE - CHEST FELLOW PH ILD 2025 - Read-Only

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This case study presented at CHEST 2025 by Dr. Deborah Jo Levine focuses on advanced pulmonary hypertension associated with interstitial lung disease (PH-ILD), highlighting diagnostic challenges, disease progression, and management strategies. A 49-year-old woman with ILD showed progressive worsening shortness of breath (SOB) over months, increasing oxygen requirements, and signs of right heart dysfunction such as jugular venous distension and lower extremity edema. Despite no evidence of pulmonary embolism or infection, elevated NT-proBNP, deteriorating renal function, worsening right ventricular size and function on echocardiography, and right heart catheterization indicating severe pulmonary hypertension confirmed PH-ILD progression.<br /><br />PH-ILD is challenging to assess due to its unpredictable clinical course, frequent late diagnosis, and comorbidities that mimic or exacerbate symptoms (e.g., sleep-disordered breathing, left heart disease, reflux). Progressive pulmonary hypertension in ILD often leads to right heart failure and further functional decline. Serial imaging and hemodynamic assessment are essential for monitoring.<br /><br />Management requires a multidisciplinary approach, including optimizing ILD therapy, careful diuretic use, and early referral for lung transplantation. In the case described, the patient was hospitalized, received intravenous diuretics, optimized ILD treatment, initiated inhaled Treprostinil, and promptly evaluated and listed for bilateral lung transplantation. Early discussions and referral to transplantation centers are crucial to maximize candidacy and improve outcomes, given the unpredictable trajectory and limited treatment options for advanced PH-ILD.<br /><br />Key takeaways emphasize the necessity for early recognition and intervention of PH-ILD, identification and management of comorbidities, and timely lung transplant evaluation to address this progressive and life-limiting condition effectively.

Keywords

pulmonary hypertension

interstitial lung disease

PH-ILD

right heart dysfunction

diagnostic challenges

lung transplantation

Treprostinil therapy

oxygen therapy

multidisciplinary management

disease progression