Case Report Volume 8 • Issue 3 97 Successful Repair of a Complete Atrioventricular Septal Defect Associated with Transposition of the Great Arteries: A Rare Association and a Literature Review Torchio F 1 , Lo Rito M 1 , Bergonzoni E 1,3 , Gaspari A 2 , Giamberti A 1* Affiliation: 1 Department of Pediatric and Adult Congenital Cardiac Surgery, IRCCS San Donato Hospital, Milan, Italy 2 Department of Pediatric Cardiology and Adult with Congenital Heart Disease, IRCCS San Donato Hospital, Milan, Italy 3 Cardiac Surgery Unit, Department of Cardiac, Thoracic, Vascular Sciences and Public Health, University of Padua, Padua, Italy *Corresponding Author Alessandro Giamberti, Department of Pediatric and Adult Congenital Cardiac Surgery, IRCCS Policlinico San Donato, Via Morandi 30, 20097 San Donato Milanese (MI), Italy. Citation: Torchio F, Lo Rito M, Bergonzoni E, Gaspari A, Giamberti A. Successful Repair of a Complete Atrioventricular Septal Defect Associated with Transposition of the Great Arteries: A Rare Association and a Literature Review. Archives of Clinical and Medical Case Reports. 8 (2024): 97-100. Received: October 12, 2024 Accepted: January 23, 2024 Published: May 07, 2024 Abstract Complete atrioventricular septal defect associated with transposition of the great arteries is a relatively rare congenital heart disease, with only a few cases reported in the literature. Surgical strategy between one-stage or two-stage repair represents a challenging decision-making process. Herein, we report the case of an 11-month-old infant who received a postnatal diagnosis of a complete type A atrioventricular septal defect associated with malposition of the great arteries. The patient underwent a successful one-stage anatomic repair with arterial switch procedure and cAVSD repair with a two-patch technique. His post-operative course was uneventful and the patient was discharged home in stable condition. This case highlights the suitability of one-stage surgical repair in infants with cAVSD and TGA, resulting in successful outcomes with few complications. Keywords: Complete atrio-ventricular septal defect; Transposition of great arteries; One-stage repair Abbreviations: D-TGA = D-loop Transposition of Great Arteries; cAVSD = Complete Atrioventricular Septal Defect; VSD = Ventricular Septal Defects; CPB = Cardiopulmonary Bypass; ASD = Atrial Septal Defect; LVOT = Left Ventricular Outflow Tract; ASO = arterial switch operation; PAB = Pulmonary Artery Banding; LDA/LAD = Left Anterior Descending Artery; CX/LCX = Circumflex Coronary Artery; RCA = Right Coronary Artery; Ao/A = Aorta; PA/P = Pulmonary Artery Introduction Complete atrioventricular septal defect (cAVSD) associated with true transposition of the great arteries (TGA) has rarely been reported in the literature and the surgical management of these patients is still debated. We describe an 11-month-old male infant with cAVSD and TGA arrived from abroad and successfully treated by complete anatomic correction. A detailed literature review concerning this interesting and unusual combination is also provided. Case Report An 11-month-old boy weighing 6.5 kg was transferred to our center from abroad after a postnatal diagnosis of cAVSD with TGA. On admission, he had mild congestive heart failure symptoms and was on diuretic oral therapy.