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1179 ECHOCARDIOGRAPHIC EVALUATION OF RIGHT VENTRICULAR FUNCTION IN PATIENTS WITH CYSTIC FIBROSIS

Non-invasive evaluation of right ventricular (RV) function has been difficult in patients with cystic fibrosis (CF). Echographic recording of the pulmonary valve permits measurement of the phases of RV systole, and the ratio of the RV pre-ejection period to the RV ejection time (RPEP/RVET) correlates well with pulmonary artery pressure. RPEP/RVET and other echographic measurements including RV wall (RVW) and dimension (RVD) were compared with pulmonary function tests and clinical scores in 28 CF patients ranging in age from 4-35 years. RPEP/RVET correlated well with percent vital capacity (%VCA) r=-0.74, residual volume (%RVol) r=0.72, and clinical score r=-0.77. RVW and RVD correlated poorly (r<0.50) with %VCA, %RVol, and clinical score. Multilinear regression of RPEP/RVET and RVD improved correlations significantly for %RVol (r=0.82 and clinical score (r=0.84). Patients in right heart failure (RHF) exhibit significantly higher RPEP/RVET (mean 0.43) than those without RHF (mean=0.33) indicating higher pulmonary artery pressure or diminished RV contractility in patients with RHF. The use of digoxin did not significantly change this value. At the conclusion of the study satisfactory pulmonary valve echograms could be obtained in 2/3 of patients tested and success was independent of the severity of the pulmonary disease.