BACKGROUND: The 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.
METHODS: Fifty 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.
RESULTS: At 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).
CONCLUSIONS: RV 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.
- Adaptation, Physiological
- Blood Gas Analysis
- Case-Control Studies
- Disease Progression
- Echocardiography, Doppler/methods
- Echocardiography, Stress/methods
- Functional Residual Capacity
- Hypertension, Pulmonary/etiology
- Idiopathic Pulmonary Fibrosis/complications
- Image Interpretation, Computer-Assisted
- Kaplan-Meier Estimate
- Middle Aged
- Proportional Hazards Models
- Risk Assessment
- Severity of Illness Index
- Survival Analysis
- Vascular Resistance
- Ventricular Dysfunction, Right/diagnostic imaging