Echocardiography in cystic fibrosis: A proposed scoring system.

An echocardiographic scoring system was developed on the basis of an observed sequence of echo abnormalities, beginning with RV hypertrophy, through RV dilation, to abnormal RV systolic time intervals, noted to be associated with the progressive pulmonary disease of cystic fibrosis. This score correlated significantly with both the Shwachman-Kulczycki (r = 0.87, P < 0.001) and Taussig-NIH (r = 0.86, P < 0.001) clinical scoring systems, the Brasfield chest roentgenogram score (r = 0.86, P < 0.001), and pulmonary function test results. The scoring of echos appears to be useful for the early detection and systematic quantitation of the cardiac effects of the progressive pulmonary disease. Preliminary sequential echo studies suggest that this system provides a method for assessing the progression of cardiac disease and evaluating prognosis in individual patients, and may prove to be useful in monitoring therapeutic interventions for cor pulmonale.