• Antonello D'Andrea, Anna Agnese Stanziola, Rajan Saggar, Rajeev Saggar, Simona Sperlongano, Marianna Conte, Michele D'Alto, Francesco Ferrara, Luna Gargani, Patrizio Lancellotti, Eduardo Bossone, and RIGHT Heart International NETwork (RIGHT-NET) Investigators.
• Division of Cardiology, Department of Cardio-thoracic and Respiratory Sciences, University of Campania "Luigi Vanvitelli," A.O. dei Colli Monaldi Hospital, Naples, Italy. Electronic address: antonellodandrea@libero.it.
• Chest. 2019 Feb 1; 155 (2): 297-306.

BackgroundThe most important determinant of long-term survival in patients with idiopathic pulmonary fibrosis is the right ventricular (RV) adaptation to the increased pulmonary vascular resistance. Our aim was to explore RV contractile reserve during stress echocardiography in early-stage IPF.MethodsFifty early-stage patients with IPF and 50 healthy control patients underwent rest and stress echocardiography, including RV two-dimensional speckle tracking echocardiography. At peak exertion, blood gas analysis and spirometry were also assessed.ResultsAt rest, RV diameters were mildly increased in IPF; however, although RV conventional systolic function indexes were similar between the IPF and control groups, RV global longitudinal strain and RV lateral wall longitudinal strain (LWLS) were significantly reduced in the IPF cohort. During physical exercise, patients with IPF showed a reduced exercise tolerance with lower maximal workload (P < .01), level of oxygen saturation (P < .001), and peak heart rate (P < .01). Systolic and diastolic BP values were similar in both groups. Systolic pulmonary artery pressure (PAPs) increase (ΔPAPs) during exertion was higher in IPF vs healthy subjects (P < .0001); RV LWLS increase (ΔRV LWLS) during exercise was lower in patients with IPF vs control patients (P < .00001). By multivariable analysis, RV LWLS at rest and ΔRV LWLS were directly related to peak exertion capacity, PAPs, and blood oxygen saturation level (Spo2; P < .0001). Δ RV LWLS was directly related to diffusion lung carbon monoxide (P < .0001).ConclusionsRV myocardial dysfunction is already present at rest in early-stage IPF and worsens during exertion as detected by two-dimensional speckle-tracking echocardiography. The RV altered contractile reserve appears to be related to reduced exercise tolerability and impaired pulmonary hemodynamic.Copyright © 2018 American College of Chest Physicians. Published by Elsevier Inc. All rights reserved.

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