Surgical case of partial anomalous pulmonary venous connection to superior vena cava with cor triatriatum: Williams’ modification and excision of diaphragm

Abstract

We experienced a successful pediatric surgical case of partial anomalous pulmonary venous connection to the superior vena cava with cor triatriatum. Echocardiography and multidetector-row computed tomography showed partial anomalous pulmonary venous connection (right upper pulmonary vein connected to the high superior vena cava) and atypical cor triatriatum (analogue to type III-A2 of Lucas-Schmidt classification: left upper pulmonary vein had dual connection to the innominate vein via vertical vein and the accessory chamber). At 8 years of age, the male patient underwent extracardiac right atrial pedicle repair of partial anomalous pulmonary venous connection to the superior vena cava (Williams' modification) and excision of the diaphragm between the accessory chamber and the left atrium simultaneously. The postoperative course was uneventful in normal sinus rhythm and there was no stenosis of newer drainage root from right upper pulmonary vein.