The reliability and practicality of single crystal echocardiography in the evaluation of single ventricle. Angiographic and pathological correlates.

A prospective clinical and echocardiographic diagnosis of single ventricle was made in 42 patients. Each was evaluated for the number of atrioventricular (A/V) valves, presence of an outflow chamber (OC), A/V valve-semilunar continuity, and orientation of the great arteries. Angiographic correlations were subsequently obtained in 40 and autopsies in 12. The overall diagnosis of single ventricle was substantiated in 39. Two other patients diagnosed as single ventricle by angiography were thought to have large ventricular septal defects on echocardiography. Tricuspid valve was interpreted as septum in one. The angiographic diagnosis of single ventricle was incorrect in another, correctly diagnosed by echocardiography and confirmed at pathology. The differential diagnosis also included A-V canal, L-transposition of the great arteries, double outlet right ventricle, and tricuspid atresia. This last condition has to be differentiated on clinical evidence. The echocardiograms were of particular value in determining the number of A/V valves. Two great arteries were demonstrated in 74% of patients and their relationship was correctly determined in 79% of these. Both imaging techniques agreed closely as to A/V valve-semilunar continuity and presence of an OC, but both showed some inaccuracies compared to pathological specimens. The echocardiogram helps both in planning catheterization and in evaluating the overall diagnosis.