Abstract

The coupling of the right ventricle (RV) to the pulmonary circulation is an indicator of RV functional performance, easily estimated by echocardiography asRV global longitudinal strain (RV-GLS) and systolic pulmonary artery pressure (sPAP) ratio. We aimed to analyze the RV to arterial coupling in a real-life prospective cohort of patients affected by fibrotic interstitial lung diseases (f-ILDs) at the time of first diagnosis andto evaluate its prognosis value. Fifty-two f-ILD patients, including 27 cases with idiopathic pulmonary fibrosis (IPF), (M=37; mean age 67±7 yrs) were enrolled and compared to 30 healthy and age-matched volunteers. Standard and speckle tracking echocardiography was performed with a Vivid E95 devise (GE Healthcare, Horten, Norway). Patients follow-up wasof 69.6±22 months. Compared to controls, f-ILD patients had a larger ventricle and a worst diastolic function as RV-GLS was significantly lower along with higher sPAP estimates. Mean values of RV to arterial coupling, while remaining above the reference cut-off of 0.35%/mmHg, were significantly reduced in f-ILDs patients (0.58±0.19 vs. 0.91±0.2, p=0.0001). Also, f-ILD patients withRV to arterial uncoupling (<0.35%/mmHg) hada significantly lower survival when compared to those with values above the cut-off (36.4±13 vs.71.8±3, p=0.0001). RV uncoupling and diagnosis of IPF were independent prognosis predictors after correction for age, body mass index and sPAP. RV to arterial uncoupling is an early biomarker of RV failure and correlate with mortality in f-ILDs. Itsapplicationcan help to discriminate the clinical phenotype of these patients with significant management and prognosis repercussions

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