An atrial switch procedure for heart transplantation in an infant with heterotaxy-dextrocardia

From the Divisions of Pediatric Cardiothoracic Surgery and Pediatric Cardiology, University of Iowa Hospitals and Clinics, Iowa City, Iowa. This work is supported by the Department of Surgery, University of Iowa. Disclosures: The authors reported no conflicts of interest. The Journal policy requires editors and reviewers to disclose conflicts of interest and to decline handling or reviewing manuscripts for which they may have a conflict of interest. The editors and reviewers of this article have no conflicts of interest. Received for publication May 6, 2021; accepted for publication May 7, 2021; available ahead of print May 18, 2021. Address for reprints: Yuki Nakamura, MD, Division of Pediatric Cardiothoracic Surgery, University of Oklahoma Health Sciences Center, 800 Stanton L. Young Blvd, Oklahoma City, OK 73104 (E-mail: Yuki-Nakamura@ ohusc.edu). JTCVS Techniques 2021;8:189-91 2666-2507 Copyright 2021 The Author(s). Published by Elsevier Inc. on behalf of The American Association for Thoracic Surgery. This is an open access article under the CC BY license (http://creativecommons.org/licenses/by/4.0/). https://doi.org/10.1016/j.xjtc.2021.05.003 Heart transplantation in heterotaxy-dextrocardia using an atrial switch procedure.

aborted. Considering the atypical clinical course and persistent coronary fistulas, he was listed for a heart transplantation on day 45 of life. Ductal stenting and balloon angioplasty for banded pulmonary arteries were successfully performed at 4 months while waiting for a donor heart. At 8 months with a body weight of 8.3 kg, the patient underwent a heart transplantation using a heart from a 10.1-kg donor. The aortic arch was reconstructed using a pulmonary homograft. The recipient cardiectomy was performed preserving the entire atrial tissue. The donor heart was implanted in a levocardia position, performing an atrial switch procedure using a donor pericardial patch to develop the PV pathway (Video 1 and Figure 1). The patch was sutured starting from the right side anterior to the right PV orifices, moving forward to an opening made in the left-sided recipient atrium anterior to the left PV orifices. The left atrial anastomosis was performed using the opening. The pulmonary artery and the ascending aorta anastomoses were performed. Finally, the right atrial anastomosis was performed using a right-sided opening of the recipient atrial cuff. Weaning from cardiopulmonary bypass was smooth. Delayed sternal closure with opening of the left pericardium was performed on postoperative day 4. The patient was extubated on postoperative day 9. The cardiac catheterization Heart transplantation in heterotaxy-dextrocardia using an atrial switch procedure.

CENTRAL MESSAGE
Biatrial anastomosis with intraatrial baffling using an atrial switch procedure may be a useful tool for heart transplantation in small pediatric patients with heterotaxy-dextrocardia. study on posttransplant day 45 demonstrated widely patent anastomoses with no pressure differences in the systemic and PV pathways ( Figure 2). Unfortunately, the patient died due to acute respiratory distress syndrome from human rhinovirus/enterovirus pneumonia at 4 months posttransplant after 2 weeks of venovenous extracorporeal life support. An echocardiogram at his demise showed widely patent systemic and PV pathways.

DISCUSSION
Surgical techniques described for heart transplantation in patients with dextrocardia/situs inversus are trichotomized: bicaval anastomosis with or without extracardiac conduits or flaps keeping levocardia, 1 bicaval anastomosis keeping dextrocardia, 2 and biatrial anastomosis with intraatrial baffling keeping levocardia. 3,4 The rarity of the condition rendered previous reports small-scale with limited follow-up.
Biatrial anastomosis was adopted in our patient instead of bicaval anastomosis due to concerns over the risk of SVC pathway stenosis. Our patient was an infant who needed aortic arch reconstruction. His single left-sided SVC was short because of interrupted IVC with hemiazygos  connection. Sequelae of the SVC pathway stenosis would have been greater compared with patients with bilateral SVC or without interrupted IVC.
Previous reports described biatrial anastomosis with intra-atrial baffling. 3,4 Michler and colleagues 3 described a technique creating 2 intra-atrial baffles using autologous atrial tissue. Our technique was unique, albeit a similar method was described by Huddleston and colleagues. 4 There are a few technical pearls for our technique in small patients. First, the intra-atrial baffle patch cannot be redundant but has to be properly sized and rectangle-shaped to avoid systemic vein pathway obstruction. Second, the opening made on the recipient's left-sided atrium cannot be extended too far superiorly and inferiorly due to a concern over systemic vein pathway obstruction. If needed, the opening can be extended rather posteriorly. Third, during the left atrial anastomosis, the recipient's left-sided atrial wall remote from the opening (not the opening itself) can be sutured to the donor left atrium if there is a concern over the purse-string effect along the suture line in tiny patients. Fourth, as described by Boston and colleagues, 5 choosing an appropriately sized donor is critical. We performed a similar volumetric analysis as described by them. 5 A donor-to-recipient weight ratio was 1.2:1 in our case.