P136 Pulmonary hypertension and outcomes in a single-centre IPF cohort

Introduction Idiopathic pulmonary fibrosis (IPF) often coexists with pulmonary hypertension (PH) and left heart disease (LHD). Estimates of PH prevalence vary, from 8–15% at initial assessment to 30–50% at transplant evaluation. PH conveys increased risk of mortality and acute exacerbation. Ischaemic heart disease (IHD) affects 4–25% of patients. Novel diagnosis during follow-up is associated with increased mortality. The majority of data predate general clinical uptake of antifibrotics. We reviewed prevalence and impact of PH and LHD in our single-centre IPF cohort in the antifibrotic era. Methods We collected cardiac data from electronic patient records of patients with MDT-diagnosed IPF from 2013 onwards, diagnosed and treated, with antifibrotics as indicated, as per national guidelines. Significant echocardiographic features were defined as: systolic pulmonary artery pressure (sPAP) ≥50 mmHg, right ventricular dilatation or dysfunction for PH; and at least moderate left ventricular systolic dysfunction, valvular disease, or grade 2 diastolic dysfunction for LHD. We compared patient characteristics and outcomes of those without PH, with PH at presentation and developing PH. Differences in proportions between PH and no PH groups were calculated using Fisher’s exact test. Results See table 1. 59 IPF patients had at least one echocardiogram. 36 (61%) had echocardiographic features of significant PH; 16 (27%) at presentation. 35 (59%) received one or both antifibrotics (sequentially). 52-week and overall mortality were not significantly different with or without PH (5/36 versus 2/23, p=0.69, and 21/36 versus 11/23, p=0.59, respectively). Discussion PH prevalence is comparatively high but without appearing to influence mortality. Assessment in all patients was undertaken non-invasively, limiting conclusions on degree and aetiology of any pulmonary vascular disease. Further, the cohort is small. Antifibrotics could theoretically directly and indirectly modulate the pulmonary vasculature. Together with improved access to specialist services facilitating early work-up and appropriate management, the negative impacts of cardiovascular comorbidity are potentially modifiable. Further study is warranted. References Cottin V, Price LC, Valenzuela C. The unmet medical need of pulmonary hypertension in idiopathic pulmonary fibrosis. Eur Resp J 2018;51:1702596 Corte TJ, Wort SJ, Gatzoulis MA, et al. Elevated brain natriuretic peptide predicts mortality in interstitial lung disease. Eur Resp J 2010;36:819–825.