Abstract

Introduction : Pulmonary hypertension ( PH) is common in patients with advanced Idiopathic Pulmonary Fibrosis (IPF),ranging from 30 to 50 percent.There remains a significant knowledge gap in association of lung fibrosis specifically in Usual Interstitial Pneumonia (UIP) patients and the risk of PH based on Echocardiography.

Aim: To assess whether the degree of fibrosis on HRCT scan Chest in UIP patients is associated with echocardiographic risk of PH. 

Methods : This is a retrospective study of 98 consecutive IPF patients between January 2015 and June 2023 at a tertiary center in UAE. 76 patients were identified as definite or probable UIP based on 2022 ATS/ERS/JRS/ALAT Clinical Practice Guideline. The data analyzed includes comorbid conditions, Forced Vital Capacity (FVC),Diffusion capacity of Carbon Monoxide (DLCO). UIP diagnosis was based on multidisciplinary team of 2 expert Pulmonologists and Radiologist. PH risk was determined using 2022 ECS/ERS guideline.The degree of fibrosis was defined as mild, moderate & severe (< 10%, 10-30% and > 30% respectively) based on HRCT predefined visual scoring.

Results:

  

Conclusion: There was no association between degree of lung fibrosis and the risk of PH based on echocardiographic imaging. Fourteen patients (18%) had high risk of PH which warrants further investigation with right heart catheterization.