Arterial Switch Operation for Complex D-Transposition of the Great Arteries with Aortopulmonary Window

Central MessageTransposition of the great arteries with aortopulmonary window is an exceedingly rare congenital heart defect. We demonstrate the arterial switch operation for a neonate with this diagnosis. Transposition of the great arteries with aortopulmonary window is an exceedingly rare congenital heart defect. We demonstrate the arterial switch operation for a neonate with this diagnosis. Dextro-transposition of the great arteries (d-TGA) is commonly associated with other congenital heart defects. Among the more frequent lesions are ventricular septal defects (VSDs) and coronary artery anomalies. Aortopulmonary (AP) window rarely occurs with d-TGA, and only 20 cases have been formally published.1Mishra A. Gandhi H. Sharma P. Patel H. Parmar D. Sheth M. et al.Transposition of great arteries with aortopulmonary window: our surgical experience.Ann Thorac Surg. 2014; 97: 196-201https://doi.org/10.1016/j.athoracsur.2013.07.012Abstract Full Text Full Text PDF PubMed Scopus (2) Google Scholar In this case report, we describe a neonate at our institution diagnosed with d-TGA, AP window, and outlet VSD who underwent the arterial switch operation and patch closure of the VSD. Institutional review board approval was waived, and informed consent for publication of study data was obtained. A term female neonate was born with a prenatal diagnosis of presumed double outlet right ventricle. On day of life 1, she was transferred from an outside hospital to our institution where she underwent echocardiography. Her postnatal imaging showed complex d-TGA with AP window (type II) and outlet VSD. The patient maintained normal respirations with oxygen saturations in the high 90s and did not require prostaglandin E. At age 6 days, she experienced an episode of acute lactic acidosis that resolved with intubation. Preoperative 3-dimensional computed tomography reconstruction illustrates the AP window, aberrant left subclavian artery, and right-sided aortic arch (Figure 1). Computed tomography imaging also revealed the patient's coronary artery anatomy: 1RL, 2Cx according to the Leiden convention (Figure 2). On day of life 10 weighing 3.0 kg, she underwent an arterial switch operation under deep hypothermic circulatory arrest. Intraoperatively, the AP window was divided, followed by transection of the aorta and pulmonary arteries at the distal wall of the AP window. The coronary buttons were excised, and the outlet VSD was repaired with a decellularized patch. The coronary arteries were anastomosed to the neoaorta. Next, the branch pulmonary arteries were mobilized toward the hilum, and the LeCompte maneuver was performed. The aorta was reanastomosed to construct the left ventricular outflow tract, followed by right atriotomy and primary closure of the patent foramen ovale. Two U-shaped patches of extracellular matrix were fashioned to complete the neopulmonary root where the coronary buttons were excised. The distal anastomosis with the branch pulmonary arteries was performed to complete the right ventricular outflow tract (Video 1). Cardiopulmonary bypass time was 105 minutes, and aortic crossclamp time was 93 minutes. The patient was transferred to the cardiovascular intensive care unit where she was extubated on postoperative day 6. She is growing and developing well 9 months postoperatively.Figure 2Intraoperative view of the aorta (Ao) lying anterior to the pulmonary artery (PA). The large aortopulmonary window (star) measures 9.1 × 7.6 mm. The left coronary artery (LCA) and right coronary artery (RCA) are seen arising from a single orifice. The left circumflex coronary artery arose from a separate sinus (not shown).View Large Image Figure ViewerDownload Hi-res image Download (PPT) d-TGA with AP window is an uncommon form of congenital heart disease. Patients with d-TGA and aortopulmonary window are at increased risk for pulmonary vascular remodeling, which is exacerbated by pulmonary overcirculation. Surgical repair of this condition can be achieved through the arterial switch operation. Different techniques can be applied depending on the size of the AP window and the presence of associated anomalies. Some surgeons report exclusion of the AP window with a homograft or pericardial patch during arterial switch, similar to the repair of a typical AP window.2Backer C.L. Mavroudis C. Surgical management of aortopulmonary window: a 40-year experience.Eur J Cardio Thorac Surg. 2002; 21: 773-779https://doi.org/10.1016/s1010-7940(02)00056-8Crossref PubMed Scopus (0) Google Scholar,3Najm H.K. Jijeh A.M. El Moazamy Y.M. Mufti H.N. Abu-Sulaiman R. Mutairi M.A. Dextrocardia, aortopulmonary window with transposition of the great arteries, case report.J Saudi Heart Assoc. 2010; 22: 61-64https://doi.org/10.1016/j.jsha.2010.02.007Crossref PubMed Scopus (4) Google Scholar For patients with a VSD or PFO, others have chosen to use a patch with a 4-mm fenestration instead of closing the defect completely. These modified patches were intended to reduce the risk of pulmonary hypertension postoperatively by decreasing pulmonary blood flow (fenestrated patent foramen ovale) or enabling decompression of the right ventricle (fenestrated VSD).4Duca V. Sulliotti G. Maggio C. Corsello G. Transposition of the great arteries and aortopulmonary window in the same patient: clinical report and follow-up.Pediatr Cardiol. 2002; 23: 474-475https://doi.org/10.1007/s00246-002-1433-4Crossref PubMed Scopus (11) Google Scholar,5Chen J. Zhou C. Zhuang J. Surgical repair for d-transposition of the great arteries associated with an aortopulmonary window using the fenestrated ventricular septal defect patch as a safety adjunct.Pediatr Cardiol. 2011; 32: 78-80https://doi.org/10.1007/s00246-010-9800-zCrossref PubMed Scopus (1) Google Scholar Chen and colleagues5Chen J. Zhou C. Zhuang J. Surgical repair for d-transposition of the great arteries associated with an aortopulmonary window using the fenestrated ventricular septal defect patch as a safety adjunct.Pediatr Cardiol. 2011; 32: 78-80https://doi.org/10.1007/s00246-010-9800-zCrossref PubMed Scopus (1) Google Scholar indicated that their fenestrated VSD had closed spontaneously after 12 months, and the systolic pulmonary artery pressure was 27 mm Hg. Although these techniques were not utilized in our repair, their physiologic implications should not be overlooked. Perhaps the most variable step in the arterial switch is translocation of the coronary arteries to the neoaorta. Several different approaches have been described in the literature and each is specific to the origin of the coronary arteries. Although d-TGA with AP window represents a small subset of patients, the arterial switch operation remains the gold standard for surgical repair. eyJraWQiOiI4ZjUxYWNhY2IzYjhiNjNlNzFlYmIzYWFmYTU5NmZmYyIsImFsZyI6IlJTMjU2In0.eyJzdWIiOiJlMGIxYWE3ZWE3OTg5NTc5MTkyODdkYWQzOGMwM2EyOSIsImtpZCI6IjhmNTFhY2FjYjNiOGI2M2U3MWViYjNhYWZhNTk2ZmZjIiwiZXhwIjoxNjk0ODgwNTc5fQ.TkJ_x5-nbtEme3w0T21ANxj3b3Xdswl-hUB8yvcBX5AxP0m9s_sJrDn6cBZIo3Bxb1HHzFK4dGhLkqjPUItSBbKr0F_t-DVi8B_JXTa2bGYkRzS1ChMNL3LLH7b43CAwe3iR61Q1X1DyHiy3CpiTCRTrZhJovmV9rWDwZOhxXwKtgP669I0s5RBP5uJBhSBdRaOYrudJXBDymEPflaBSl2nRvIZBjsDkd0RMZlr7ioy5Fw68CqW49bvFhuoSRwvhS-Tt8put2FXZcOTXxcR8XP3VZBcqHrDgNHk9lIdK6TCdVMpYMmmjm7vZW0M6Tp4V7QxPHERg1h7SLxRro5drJg Download .mp4 (100.34 MB) Help with .mp4 files Video 1Arterial switch operation for complex dextro-transposition of the great arteries with aortopulmonary window. Video available at: https://www.jtcvs.org/article/S2666-2507(23)00064-0/fulltext. Download .jpg (.24 MB) Help with files Video 1Arterial switch operation for complex dextro-transposition of the great arteries with aortopulmonary window. Video available at: https://www.jtcvs.org/article/S2666-2507(23)00064-0/fulltext.