Echocardiographic abnormalities in patients with cystic fibrosis.

An echographic study was undertaken to evaluate left (LV) and right ventricular 
(RV) function in 30 patients with cystic fibrosis. Echographic recording of 
the pulmonary and aortic valve echogram permitted measurement of the phases 
of right and left ventricular systole. The ratio of the LV 
preejection period/LV ejection time (LPEP/LVET) and shortening of the LV internal 
dimension %SID was employed to reflect LV function, while RV preejection 
period/RV ejection time (RPEP/RVET) has excellent correlation with pulmonary artery diastolic 
pressure. RPEP/RVET and two other echographic measurements, right ventricular wall (RVW) 
and internal dimension (RVD) were compared with pulmonary function tests and 
clinical scores. RPEP/RVET correlated well with percent vital capacity(%VC), r = 
-0.73, percent residual volume (%RVol) r = +0.72, and clinical score, 
r = -0.77. Multilinear regression of RPEP/RVET, RVD, and RVW improved 
correlation for %VC (r = -0.80), %RVol, r = +0.82, and 
clinical score, r = -0.84. Patients in overt right heart failure 
exhibited elevated RPEP/RVET (mean = 0.48) when compared to patients not 
in right heart failure (mean = .33). Marked diminution of LV 
function was present in two patients. A variety of cardiovascular abnormalities 
were demonstrated echographically and were valuable in assessing the degree of 
cardiac involvement in patients with cystic fibrosis. 