Specific cardiovascular morphology on computed tomography angiography in patients with right isomerism.

BACKGROUND

Children with right isomerism usually accompany complex congenital heart disease, which is associated with pulmonary atresia, common atrioventricular valve, and total anomalous pulmonary venous connection. This study aimed to explore specific cardiovascular morphology associated with right isomerism on computed tomography angiography (CTA).

METHODS

We retrospectively reviewed CTA images to assess the atrio-ventricular connections and the spatial relationship between the systemic and pulmonary channels in children with right isomerism.

RESULTS

We studied 33 patients (17 females). Atrial situs was classified into solitus and inversus in 21 and 12 patients, respectively. Atrioventricular valve morphology included unilateral insertion in 15/33 (46%), balanced insertion into both ventricles in 11/33 (33%), unbalanced insertion into both ventricles in 7/33 (21%), and common atrioventricular valve in 27/33 (82%). The pulmonary connection included atresia and stenosis in 14/33 (42%) and 17/33 (52%), respectively. The anteroposterior relationship between the aorta and the pulmonary trunk was observed in 15/33 (46%). Total anomalous pulmonary venous connection concurred in 21/33 (63%), including supracardiac type in 16/21 (76%) and infracardiac type in 2/21 (10%). Preoperative pulmonary venous obstruction was found in 10/21 (47%). There were 14 patients with bilateral superior caval veins (42%). The anteroposterior relationship between the aorta and the pulmonary trunk was significantly correlated to the pulmonary arterial and venous channel anomalies (p = 0.001 and p = 0.027).

CONCLUSIONS

The spatial relationship between the systemic and pulmonary channels was associated with distinct cardiovascular morphology associated with right isomerism.