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Complete Repair of Total Anomalous Venous Return

Complete repair of a total anomalous pulmonary venous return. Also shown is a primary closure of a patent foramen ovale and patent ductus arteriosus. The patient is placed on cardiopulmonary bypass (CPB) in the standard fashion. The patient is then crash cooled to 20 degrees celsius with ice placed on the head and administration of steroids. Antegrade cardioplegia is then administered. The large confluent vein (vertical vein) is dissected and an arteriotomy is made, a subsequent atriotomy is made in the left atrial appendage. A side to side anastomosis using polypropylene suture in a continuous running fashion. The right atrium is then opened and the patent foramen ovale is closed. The patient was warmed to a satisfactory temperature and once adequate hemostasis was achieved the vertical vein is ligated at its insertion into the innominate vein.

We present a case of a newborn male at 8 days of age with supracardiac total anomalous pulmonary venous return (TAPVR). Upon admission the patient was mildly desaturated with a pulse ox in the 80s though he did not clinically exhibit cyanosis. Echocardiography demonstrated unobstructed supracardiac TAPVR along with a patent foramen ovale (PFO). Standard surgical approach is through midline sternotomy using cardiopulmonary bypass under deep hypothermic circulatory arrest (DHCA). The vertical vein was identified and observed coursing posterior to the left atrial appendage with its insertion point in the innominate vein. The vertical vein was entered, identifying several pulmonary veins returning to its confluence.  An incision is made in the atrial appendage in proximity to the vertical vein incision and side to side anastomosis is performed in a continuous running fashion. The atrium is entered and the PFO is then closed primarily, cardiopulmonary bypass is resumed and the patient is rewarmed, during that time the vertical vein is ligated as high as possible near the innominate vein.
Total anomalous pulmonary venous return is a rare congenital heart disease with a mortality rate of approximately 80% if not treated surgically during the first year of life [2]. The standard approach is a midline sternotomy. The patient is placed on aorto-atrial cardiopulmonary bypass in the standard fashion. The large innominate vein with an ascending vertical vein can be observed on the left side of the mediastinum, coursing posterior to the left atrium. The vertical vein is dissected to its insertion at the innominate vein. For this case the patient is systemically cooled to 20 degrees celsius with ice on the head, steroids are administered on cardiopulmonary bypass and  a single dose del Nido antegrade cardioplegia is administered. The large confluence posterior to the left atrium is then identified and partially mobilized and an veinotomy is performed using a 15 blade scalpel. A corresponding atriotomy is performed on the left atrial appendage. The vertical vein and left atrium are joined in a side to side anastomosis using 6.0 Prolene suture in a continuous running fashion. The right atrium is then opened and the patent foramen ovale is closed primarily using 6.0 Prolene suture. The right atrium is closed and cardiopulmonary bypass is reinitiated. As patient is rewarmed, the vertical vein is ligated at its insertion point in the innominate vein. Once the patient reaches appropriate temperature the patient is weaned off cardiopulmonary bypass.  DHCA presents unique challenges to poet procedure hemostasis, appropriate use of blood products are required to aid in hemostasis, once achieved the patient is closed in the usual fashion.
No specific materials are required for this procedure, however, blood replacement products are essential post procedure. Methods are described in the discussion.
The patient remained intubated until post op day 3. Mediastinal and pleural drains were removed on post op day 2. The patient was discharged on post op day 9. At 4 week follow up he was doing well on no medications. Echocardiogram demonstrated no obstruction at the site of repair. The patient is feeding well and the parents deny tachypnea, cyanosis, shortness of breath or any other cardiac symptoms.
The superior approach was demonstrated by Tucker et al in 1976 [3]. This approach used a side to side anastomosis but was done through the transverse sinus [3]. The approach we used allows for better visualization of the left atrium as well as the vertical vein. New techniques such as the sutureless technique are gaining prominence. The sutureless technique involves creating a neo-LA through pericardial flaps [4]. This prevents suture from contacting the pulmonary veins and therefore decreases the instances of postoperative obstruction [4]. This method also prevents suture from creating distortions in the small pulmonary veins and disrupting flow [4]. Risk of postoperative obstruction is ambiguous and this patient was an ideal candidate for primary side to side anastomosis [1]. There have been cases reported, such as with Cooley et al. where patients are middle aged with no signs of obstruction or other abnormalities [5].
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1. White BR, Ho DY, Faerber JA, et al. Repair of total anomalous pulmonary venous connection: Risk factors for postoperative obstruction. The Annals of Thoracic Surgery. 2019;108(1):122-129. doi:10.1016/j.athoracsur.2019.02.017 2.Shi G, Zhu Z, Chen J, et al. Total anomalous pulmonary venous connection. Circulation. 2017;135(1):48-58. doi:10.1161/circulationaha.116.023889 3. Tucker BL, Lindesmith GG, Stiles QR, Meyer BW. The superior approach for correction of the SUPRACARDIAC type of total anomalous pulmonary venous return. The Annals of Thoracic Surgery. 1976;22(4):374-377. doi:10.1016/s0003-4975(10)64970-3 4. Yanagawa B, Alghamdi AA, Dragulescu A, et al. Primary sutureless repair for “simple” total anomalous pulmonary venous connection: Midterm results in a single institution. The Journal of Thoracic and Cardiovascular Surgery. 2011;141(6):1346-1354. doi:10.1016/j.jtcvs.2010.10.056 5. Cooley DA, Cabello OV, Preciado FM. Repair of total anomalous pulmonary venous return: results after 47 years. Tex Heart Inst J. 2008;35(4):451-453.

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