Superior ​Approach for ​Repair ​of​ Obstruct​ed​ Mixed ​​TAPVC in Situs Inversus With Dextrocardia

A 2.8 kg newborn with signs of congestive heart failure was admitted to the authors’ center. He was diagnosed with situs inversus totalis, dextrocardia, bilateral superior vena cava, atrial septal defect (ASD II), and biventricular anatomy. Additionally, there was mixed total anomalous pulmonary venous connection (TAPVC) with an obstructive horizontal vein draining into the right-sided (morphologically left) superior vena cava (SVC) and coronary sinus (CS). At four days of age, the patient was referred for complete surgical repair. This video case report demonstrates the superior approach for the repair of TAPVC. 

Surgical Technique 

Through a standard median sternotomy, the right-sided (morphologically left) superior vena cava was dissected, and the entrance of the horizontal pulmonary vein confluence into the right-sided SVC was mobilized. The anatomy was examined and verified. There was a left-sided (morphologically right) SVC and inferior vena cava draining into the left-sided right atrium. Additionally, a separate right hepatic vein entered into the inferior segment of the right-sided SVC and into the coronary sinus. 
 
While on cardiopulmonary bypass (CPB), the separate right hepatic vein was controlled with a Yasargil clip. The horizontal confluence, along with both right and left collecting pulmonary veins, was dissected. The blood return from both lungs was controlled by two Yasargil clips, and the right-sided SVC was permanently occluded by hemoclips above and under the entry of the horizontal pulmonary vein. 

After aortic cross-clamp and cardioplegia administration, the horizontal pulmonary vein confluence was widely opened, and a parallel incision was performed on the roof of the left atrium right at the base of the left atrial appendage (LAA). A side-to-side anastomosis was created between the left atrium and the opening of the pulmonary vein confluence. Working through the right atriotomy, the atrial septal defect was repaired with a pericardial patch, and a 3 mm fenestration was left open to provide access for potential later catheter reintervention. The right hepatic vein was left intact, draining into the lower part of the right-sided SVC and coronary sinus. 
 
CBP and aortic cross-clamp times were 81 and 42 minutes, respectively. The patient was extubated on the ninth postoperative day and was discharged home on postoperative day 22. Postoperative transthoracic echocardiography showed free inflow from the confluence of the pulmonary veins into the left atrium and a small patent foramen ovale with a left-to-right shunt. The patient was doing well two years postoperatively.  

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