Echocardiographic profiles of the long-term cardiac changes in cystic fibrosis.

In this echocardiographic study, assessment of the heart in children with cystic 
fibrosis has shown that changes occur in not only the right 
ventricle but also in the left ventricle and aorta, as compared 
with normal. Echocardiograms could be successfully performed in 34 of 37 
patients. The thickness of the right ventricular anterior wall and the 
dimension of the right ventricular cavity were abnormal, even in patients 
with mild disease (National Institutes of Health [NIH] score for severity 
of disease of 85 or greater). Larger, older children with lower 
NIH score had disproportionately larger right ventricular anterior walls and cavities. 
The thickness of the septal wall, the thickness of the left 
ventricular posterior wall, and the aortic dimensions were increased in those 
with severe disease. The dimensions of the left ventricular cavity for 
the population were slightly but significantly smaller than normal in systole 
and in diastole throughout the course of the disease. This study 
demonstrates that echocardiography is an effective noninvasive means of assessing the 
long-term changes in children with cystic fibrosis. These changes occur in 
both sides of the heart and appear to worsen as the 
disease progresses. 