Echocardiography for the diagnosis of congenital cardiac anomalies with multiple lesions.

The purpose of this study was to determine the sensitivity and specificity of echocardiography for the diagnosis of congenital cardiac abnormalities with multiple lesions. The study was carried out on 80 patients (ages 1 day to 14 years). After clinical evaluation all patients were studied by echocardiography. Cardiac catheterization and angiocardiography were performed, and echocardiographic findings were compared with those obtained by cardiac catheterization. The sensitivity and specificity of echocardiographic diagnosis were determined based on the false-negative and false-positive results. Of the 80 patients, 19 had double-outlet right ventricle, 17 transposition of the great arteries, 10 common atrium, 9 atrioventricular septal defect, 7 single ventricle, 7 corrected transposition, 6 tricuspid atresia, 3 Ebstein's anomaly, and 2 cor triatriatum. All of these entities were visualized correctly by echocardiography (sensitivity 100%, specificity 100%). There were also 12 instances of atrial isomerism with one false-negative diagnosis, 6 pulmonary atresia with two false-negative diagnoses, and 5 total anomalous pulmonary venous connections with one false-negative diagnosis. The total number of individual cardiac lesions was 291. Nineteen false-negative and four false-positive echocardiographic diagnoses were obtained (sensitivity 93%, specificity 99%). It is concluded that double-outlet right ventricle, transposition of the great arteries, atrioventricular septal defect, single ventricle, corrected transposition, and tricuspid atresia can be accurately diagnosed by echocardiography. However, the role of echocardiography is limited for evaluation of right ventricular outflow tract and small patent ductus arteriosus, especially when associated with pulmonary hypertension.