[A case of successful surgery for tetralogy of Fallot with single atrium, azygous connection and partial anomalous pulmonary venous drainage in atrio-visceral heterotaxic syndrome].

A 2-year-old girl of heterotaxia associated with complex anomalies was reported. Major cardiac anomaly was tetralogy of Fallot (TOF), which associated with the interruption of the inferior vena cava, persistent left superior vena cava (PLSVC), common atrium and partial anomalous pulmonary venous drainage (PAPVD) to the coronary sinus. The two dimensional echocardiogram displayed the characteristic views of TOF and the additional interesting abnormalities near the mitral ring. The markedly enlarged and protruded coronary sinus by the association of PLSVC and PAPVD overlay along the posterior mitral leaflet and the abnormal muscular septum surrounded immediately above the anterior mitral leaflet. These structures created a sort of supramitral ring structure, although this was not important hemodynamically because of the association of single atrium and TOF preoperatively. At the operation the coronary sinus roof was resected along the posterior mitral leaflet as well as the abnormal muscular flange. The atrial septum was created with a porcine pericardium and the left superior vena cava was ligated. The postoperative course was not eventful.