日本小児循環器学会雑誌 Pediatric Cardiology and Cardiac Surgery

Left isomerism is frequently associated with anomalous systemic venous connection and common atrium, requiring systemic venous abnormality correction and common-atrium septation in biventricular repair. Our case was a 4-year-old girl diagnosed with left isomerism, dextrocardia, complete atrioventricular septal defect, common atrium, bilateral superior vena cava, and interruption of the inferior vena cava with hemiazygos vein connection. She underwent intracardiac repair with intra-atrial rerouting. Preoperative computed tomography suggested that extracardiac reconstruction of the anomalous systemic vein was inappropriate, and showed that all the pulmonary veins drain to the common chamber in the common atrium. Thus, the blood flow from the right superior vena cava was diverted into the left side of the common atrium through an intra-atrial tunnel. Complete atrioventricular septal defect was repaired with modified one-patch method, and the patch divided the common atrium into the right and left atria. She was discharged on postoperative day 11 without obstruction in systemic and pulmonary venous return. Therefore, if all the pulmonary veins drain to the common chamber, intra-atrial rerouting may effectively correct anomalous systemic venous connection in the septation of the common atrium.