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Transposition of the Great Arteries with Ventricular Septal Defect (TGA-VSD) with or without Left Ventricular Outflow Tract Obstruction (LVOTO)

Aditya Suryakumar, MD, Viktoria H. Weixler, MD, Sitaram M. Emani, MD

Key Points

  • Anatomical and Embryological Basis: D-Transposition of the Great Arteries (D-TGA) is characterized by concordant atrioventricular connections but discordant ventriculo-arterial connections, with the pulmonary artery arising from the left ventricle and the aorta from the right ventricle. The presence of a ventricular septal defect (VSD) and, in some cases, left ventricular outflow tract obstruction (LVOTO), defines the complexity of the lesion.
  • Pathophysiology and Clinical Presentation: The fundamental pathophysiological issue in TGA is parallel circulation: systemic venous blood is delivered to the systemic arterial system, and pulmonary venous blood is recirculated to the lungs. Survival depends on mixing at the atrial, ventricular, or ductal level. The degree of cyanosis and clinical symptoms (i.e. heart failure) is determined by the extent of mixing and the presence or absence of LVOTO.
  • Diagnostic Approach: Echocardiography is the primary diagnostic tool, essential for defining the ventriculo-arterial relationships, VSD characteristics, coronary artery anatomy, and any associated LVOTO, guiding both clinical management and surgical planning. The key features to establish the diagnosis include discordant ventriculo-arterial connections with concordant atrioventricular connections. It is also important to define the location and size of the VSD, and any anatomic structures that cause restriction to flow across the VSD or to pulmonary or systemic blood flow should be identified.
  • Clinical and Surgical Management: Initial stabilization includes prostaglandin E1 infusion to maintain ductal patency and, when necessary, balloon atrial septostomy to improve mixing. Definitive surgical management is determined by the specific anatomy:
    • Arterial Switch Operation (ASO): Preferred with prepared LV, good pulmonary valve, and left ventricular outflow tract can be adequately relieved. This involves transecting and re-anastomosing the great arteries to their correct ventricles, with coronary artery transfer and VSD closure as needed.
    • Rastelli Procedure: Used when there is significant LVOTO that cannot be relieved. The left ventricle is baffled to the aorta via the VSD, and a right ventricle-to-pulmonary artery conduit is placed.
    • REV procedure (Réparation à l’Etage Ventriculaire) (Conal septal resection + LV to aorta intraventricular tunnel + RV to PA direct anastomosis): Used when there is significant LVOTO that cannot be relieved. The REV Procedure includes (1) conal septal resection and (2) LV to aorta intraventricular tunnel (3) RV to PA direct anastomosis.
    • Pulmonary Root Translocation: Used when there is LVOTO that cannot be relieved, unfavorable coronary anatomy, and moderately dysplastic PV, but pulmonary root of sufficient quality.
    • Aortic Root Translocation (Nikaidoh Procedure): With favorable coronary anatomy and an unsalvageable pulmonary valve, the Nikaidoh procedure (Aortic root translocation over left ventricle) involves posterior translocation of the aortic root to the left ventricle, and reconstruction of the right ventricular outflow tract.
    • Double Root Translocation: Includes translocation of the aortic root and the pulmonary root.
    • Atrial Switch (Mustard or Senning): Considered for palliation in select patients not suitable for anatomic repair. Surgical planning is highly individualized, based on the relationship of the VSD, the size and morphology of the pulmonary valve, and coronary anatomy.
  • Long-Term Outcomes and Prognosis: Long-term survival for TGA/VSD +/- LVOTO is generally excellent, with greater than 90% survival at 15 years for most anatomic repairs. However, outcomes vary by surgical strategy:
    • Arterial Switch Operation (ASO): Associated with 85–90% freedom from LVOT reoperation at 10 years, and a 96% long-term survival rate. Late complications can include neo-aortic root dilatation, neo-aortic regurgitation, and coronary artery issues, though reoperation rates for these are low.
    • Nikaidoh Procedure: Demonstrates 95% mid-term survival, with low rates of LVOT reintervention.
    • Rastelli Procedure: Has the lowest long-term survival (52% at 20 years), primarily due to conduit obstruction, LVOTO, and arrhythmias. Freedom from RVOT reoperation is only about 20% at 15 years.
    • Atrial Switch (Mustard/Senning): 30-year survival is around 60%, with late complications including sinus node dysfunction, arrhythmias, and RV dysfunction. Adult survivors may experience reduced exercise capacity, cognitive challenges, and decreased quality of life. The need for pacemaker implantation is associated with higher mortality.

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Last updated: January 13, 2026