Specific morphological features of congenital heart disease relevant to outcomes in patients with right isomerism

Abstract

Abstract Purpose Children with right isomerism accompany complex congenital heart disease, which is related to high mortality and morbidity, especially total anomalous pulmonary venous connection and common atrioventricular valve regurgitation. This study aimed to explore specific cardiac morphological features relevant to poor outcomes based on computed tomography (CT) in children with right isomerism. Methods We retrospectively reviewed contrasted CT images in patients with right isomerism, and assessed cardiac morphological features including the atrial and ventricular connections, the special relationship between the aorta and the pulmonary trunk, the appendages, pulmonary venous channels, and caval veins. In particular, we focused symmetry of the heart base which was defined as the atrial appendages were symmetrically positioned on either side of the antero-posteriorly great arteries. We analyzed the morphological features relevant to total anomalous pulmonary venous connection which is the risks for poor outcomes. Results Thirty patients with right isomerism were studied. Atrial situs was classified into situs in 11, and inversus in 19 patients. Atrioventricular and ventriculoarterial discordances were observed in 17 and 26 patients, respectively. Ventricular morphological features included single ventricle in 14, unbalanced atrioventricular septal defect in 6, pulmonary atresia in 11, pulmonary stenosis in 17, and bilateral superior caval veins in 14. There were 18 patients (60%) with extracardiac total anomalous pulmonary venous drainage (preoperative obstruction: N = 8), which was significantly correlated to symmetry of the heart base (P < 0.001). Conclusions The heart base symmetry correlated to total anomalous pulmonary venous drainage.