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Left ventricular wall stress and contractile function were determined by echocardiographic methods in 11 patients studied 0.7 to 13.8 years (mean +/- standard error of the mean = 5.6 +/- 1.2 years) after undergoing the Rastelli operation for transposition of the great arteries associated with ventricular septal defect and left ventricular outflow tract obstruction. Age at operation ranged from 4.6 to 11.3 years (mean +/- standard error of the mean = 7.4 +/- 0.7 years). Data were compared with data of 24 normal subjects of similar age and heart rate. Left ventricular end-diastolic dimension and end-diastolic volume were significantly higher than normal, averaging 134% +/- 8% of normal dimension (p less than 0.004) and 106 +/- 13 ml/m2 versus a normal volume of 60 +/- 3 ml/m2) (p less than 0.007). In addition left ventricular wall mass was 215 +/- 40 gm/m2 versus a normal value of 72 + 6 gm/m2 (p less than 0.004). Both meridional and circumferential end-systolic and peak systolic stress values were not significantly different between normal subjects and Rastelli patients. Estimates of ventricular pump function including shortening fraction, rate-corrected velocity of circumferential fiber shortening, and ejection fraction were all depressed when compared with normal values. Velocity of fiber shortening, evaluated as a function of end-systolic stress, demonstrated abnormal contractile function in eight of 11 (73%) patients. These data indicate that left ventricular function is usually abnormal and residual left ventricular dilation and wall hypertrophy remain despite successful use of the Rastelli operation for repair in patients with transposition of the great arteries, ventricular septal defect, and left ventricular outflow tract obstruction.

Left ventricular wall stress and contractile function were determined by echocardiographic methods in 23 patients with tricuspid atresia after palliation only (group 1), in 19 patients after Fontan repair (group 2), and in 24 age-matched normal subjects. End-diastolic dimension was increased above normal in both groups with tricuspid atresia (group 1 [mean +/- SEM] 141 +/- 4%, group 2 129 +/- 5% of normal; both p less than .001) but were not different from each other. Left ventricular end-diastolic volume and wall mass also were increased above normal in both groups (group 1, 126 +/- 9 ml/m2 and 194 +/- 19 g/m2, respectively, p less than .001 and p less than .004; group 2, 80 +/- 7 ml/m2 and 128 +/- 10 g/m2, p less than .02 and p less than .001), and group 2 patients showed significantly lower values than group 1 patients (p less than .001 and p less than .004). Meridional end-systolic stress was increased above normal in both groups with tricuspid atresia but was not different between groups (normal, 43 +/- 3 g/cm2; group 1, 56 +/- 54 g/cm2, p less than .02; group 2, 59 +/- 7 g/cm2, p less than .001). Contractile function estimated by rate-corrected circumferential fiber shortening velocity was abnormal in nine of 23 (39%) group 1 patients and in five of 19 (26%) group 2 patients (percentages not different from each other by nonparametric testing). Contractile function was depressed in one of 11 group 1 patients under 5 years old and in eight of 12 over 5.3 years old.(ABSTRACT TRUNCATED AT 250 WORDS)

This article describes the determination of small systemic ventricular volume by quantitative cineangiocardiography in two patients with congenitally corrected transposition of great arteries, Ebstein's malformation of the systemic atrioventricular valve, and ventricular septal defect. A subjectively small systemic ventricle and an Ebstein-like abnormality of the systemic atrioventricular valve have been described previously in association with congenitally corrected transposition, but in vivo quantification of ventricular size and pump function has not been reported. An initial palliative rather than a totally corrective procedure is probably desirable in patients with this combination of findings. Ventricular volume calculations may prove useful as an aid in selection of operation in this setting.