Echocardiography in cystic fibrosis

Echocardiograms were obtained for 25 patients with cystic fibrosis (CF) and 20 controls to evaluate this technique as a means of assessment of right ventricular changes in patients with CF. Right ventricular anterior wall thickness per square meter of body surface (RVAW/sq m) and right ventricular internal dimension per square meter of body surface (RVID/sq m) were compared with other techniques for detection of cor pulmonale. Significant correlations existed between both RVAW/sq m and RVID/sq m and the forced vital capacity, forced expiratory volume in one second, midmaximal expiratory flow rate, clinical score of severity of disease, and roentgenographic score of pulmonary involvement. The RVAW/sq m was slightly more sensitive than RVID/sq m; RVAW/sq m thickness on echocardiogram in vivo compared well with actual measurements at autopsy in five patients. No correlations were found between echocardiography and electrocardiograms, vectorcardiograms, thoracic index, or cardiothoracic ratio.