Impact of pulmonary hypertension in baseline characteristics and survival of patients with idiopathic pulmonary fibrosis

<bold>Background:</bold> Pulmonary hypertension (PH) may complicate patients with idiopathic pulmonary fibrosis (IPF) and affect their outcome. <bold>Aims and Objectives:</bold> To investigate the impact of PH on functional and imaging parameters as well as survival in patients with IPF. <bold>Methods:</bold> This is a retrospective case-control study including routine clinical, functional and echocardiographic data at diagnosis between 2005 and 2023. <bold>Results:</bold> In total, 65 adults with IPF and precapillary PH and 65 controls (IPF without PH, excluded with echocardiography and/or RHC) matched for age, sex and forced vital capacity were included (Figure 1A). Compared to their matched controls, patients with IPF-PH had a significantly lower diffusion lung capacity for carbon monoxide, walked a shorter distance and desaturated significantly at the end of 6-minute walk test. They presented also elevated brain natriuretic peptide levels and right ventricle dilatation with impaired function. More than half of patients with IPF-PH (n=36, 55.4%) had severe PH (pulmonary vascular resistance >5 wood units). Survival was significantly reduced among patients with IPF-PH compared to their controls, while patients with severe PH presented an even worse survival compared to those with non-severe PH (Figure 1B, C). <bold>Conclusions:</bold> PH greatly impacts functional parameters and survival among patients with IPF. Widely available non-invasive tools for upfront screening are needed to timely detect PH in IPF and refer patients for further management at specialist PH centres. <fig><object-id>erj;64/suppl_68/PA3420/F1</object-id><object-id>F1</object-id><object-id>F1</object-id><graphic></graphic></fig>