Excerpt

A 4-year-old girl presented with cardiac dysrhythmia detected on a routine clinic visit. On physical examination the second heart sound was widely split and there was a grade 1/6 systolic ejection murmur at the left upper sternal border. Transthoracic echocardiography displayed a superior sinus venosus atrial septal defect, turbulent flow in the superior vena cava (SVC), and right heart enlargement. Partial anomalous-pulmonary venous connection (PAPVC) was suspected. Cardiac magnetic resonance imaging (MRI) clearly visualized the anomalous drainage of the right upper and middle pulmonary veins into the SVC and the right atrium (Figs. 1 and 2). No further studies were requested for surgery. Intraoperatively, the anomalous pulmonary veins were tunnelized to the left atrium via the septal defect, which was enlarged to provide for unrestricted flow. The SVC was transected and anastomosed to the right atrial appendage. The postsurgical course was uneventful. At 11-month follow-up there was no evidence of arrhythmias or pulmonary venous obstruction.

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