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Am. J. Respir. Crit. Care Med., Volume 163, Number 5, April 2001, 1212-1218

Subclinical Right Ventricular Dysfunction in Cystic Fibrosis
A Study Using Tissue Doppler Echocardiography

ADRIAN A. IONESCU, ALINA-ANDREEA IONESCU, NICOLA PAYNE, IZASKUN OBIETA-FRESNEDO, ALAN G. FRASER, and DENNIS J. SHALE

Section of Respiratory Medicine and Department of Cardiology, University of Wales College of Medicine, Cardiff, United Kingdom

Patients with severe cystic fibrosis can develop cor pulmonale, but little is known about the function of the right ventricle (RV) early in the disease. We hypothesized that such patients might have subclinical RV dysfunction, detectable by tissue Doppler echocardiography, and related to the severity of lung disease. We studied 21 clinically stable patients (Group 1), five patients with severe lung disease (Group 2), and 23 age-matched healthy subjects. Patients had impaired RV systolic function. The mean (SD) systolic velocities of the RV free wall were 8.9 (1.7) cm/s in Group 1, 7.7 (1.0) in Group 2, and 10.8 (1.9) in healthy subjects (p < 0.001). The velocities of the tricuspid annulus were less in patients (p < 0.0001). Patients had a greater isovolumic relaxation time (p < 0.001), indicating RV diastolic dysfunction. RV wall thickness was greater in patients (0.4 [0.1] versus 0.3 [0.1] cm/m2, p < 0.01). RV systolic function was related to C-reactive protein (r = - 0.66, p < 0.001) and FEV1 (r = 0.62, p = 0.003) and diastolic function to interleukin-6 (r = 0.64, p < 0.005). Patients with cystic fibrosis have subclinical RV dysfunction, which correlates with the severity of lung disease. Tissue Doppler echocardiography provides a quantifiable indicator useful for detection and monitoring of disease progression.




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