Primary Sutureless Repair of Obstructed Total Anomalous Pulmonary Venous Connection (TAPVC) in a Neonate

This video presents a case of primary sutureless repair of obstructed total anomalous pulmonary venous connection (TAPVC) in a neonate. The patient is a full-term, 3.1 kg male neonate who was transferred to this center with possible diagnosis of TAPVC. He presented in extremis with cardiogenic shock and multisystem organ failure (MSOF). A chest X-ray showed bilateral pulmonary edema, and an echocardiogram confirmed the presence of an obstructed TAPVC with severe pulmonary hypertension. 

The patient was immediately placed on peripheral extracorporeal membrane oxygenator support (ECMO) via percutaneous cannulation of the right internal jugular vein and the right common carotid artery. This served to stabilize the patient, improve his oxygenation, and reverse his MSOF. A preoperative computed tomography scan (CTA) was done to confirm the anatomy of the pulmonary veins, which were all connected to a retrocardiac confluence that drained via a vertical vein to the portal vein after passing through the liver parenchyma. The decision was then made to proceed with surgical repair via median sternotomy. 

First, the ductus arteriosus was dissected and ligated. The vertical vein was then dissected at the level of the diaphragm and encircled with a 3-0 silk tie. Marking sutures with multiple 6-0 Prolene sutures were placed to delineate the suture line of the future left atriopericardial anastomosis. Heparin was then administered systemically and ECMO was switched to cardiopulmonary bypass (CPB) via the same neck cannulas. The patient was cooled down to 18 degrees Celsius. 

Next, an ascending aorta cardioplegia needle was placed. The ascending aorta was cross-clamped, and antegrade cardioplegia was administered. The heart was then delivered out of the pericardial cavity into the right pleural space. An incision was created in the left atrium from the base of the left atrial appendage, and the atrial septum was resected. The suture line was then started between the left atrium and the pericardium around the pulmonary veins and its confluence using running 7-0 Prolene sutures. 

Once half of the suture line was completed, the pulmonary venous confluence was opened along its long axis, and the incision was extended all the way along the length of the vertical vein. The incision was extended across the upper pulmonary veins to guarantee wide drainage of all veins into the pericardial cavity. The surgeons then completed the left atriopericardial anastomosis. 

Once the anastomosis was completed, the heart was repositioned back into the pericardial cavity. An oblique right atriotomy was then made, and the atrial septal defect was visualized. To close the defect, an appropriately sized bovine pericardial patch with a 4 mm fenestration was sewn in using running 6-0 Prolene sutures. 

The right atriotomy was then closed using running 6-0 Prolene sutures. CPB was then reinitiated, and the aortic cross-clamp was removed after routine deairing of the heart. The patient regained his normal sinus rhythm and was rewarmed back to normothermia. He was weaned off CPB and an epicardial echocardiogram showed excellent repair. The chest was temporary closed at this stage.

The aortic cross-clamp time was forty-nine minutes, cardiopulmonary bypass was 116 minutes, and the circulatory arrest time was forty-four minutes. 

Post-Operative Course

The patient was kept on ECMO initially until the lung conditions and all other organ systems recovered. The patient was subsequently weaned off ECMO two days later with repair of both the right internal jugular vein and the right common carotid artery, simultaneously with chest closure. He was extubated on the fourth postoperative day and was discharged on day fifty-five.
The patient continued to do well during his follow-up, with most recent echocardiogram (four months postoperatively) showing laminar flow from the pulmonary venous confluence into the left atrium. There was a mean gradient of 1 mmHg across the left atriopericardial anastomosis. Both ventricles were of normal size and functions.

References

1. Wang Z, Ding N, Yi H, Zhu Y, Li Z, Yan D, Li X, Bai S. Application of sutureless technique in total anomalous pulmonary venous connection repair. J Card Surg. 2022 Nov;37(11):3769-3775
2. Zhao L, Pan Z, Wu C, Shen L, Wu Y. Sutureless Technique for Primary Total Anomalous Pulmonary Venous Connection Repair: An Updated Meta-Analysis. Front Cardiovasc Med. 2022 Apr 28;9:890575
3. Thanh DQL, Giau HTN, Huong TNG, Linh TNU, Phuc VM, Vuong NL. Sutureless Closure Versus Conventional Technique in the Primary Surgery of Total Anomalous Pulmonary Venous Connection: A Systematic Review and Meta-analysis. Pediatr Cardiol. 2022 Jun;43(5):943-951

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