The optimal procedure for the great arteries and left ventricular outflow tract obstruction. An anatomical study
Mark Hazekamp1, Francisco Portela, Margot Bartelings
1Department of Cardiothoracic Surgery, Leiden University Medical Center, Leiden, The Netherlands. m.g.hazekamp@lumc.nl
Insights
Optimal surgical strategies for transposition of the great arteries (TGA) with left ventricular outflow tract obstruction (LVOTO) depend on specific anatomical features. Procedures like arterial switch operation (ASO) are often feasible, but complex cases may require Nikaidoh or Rastelli procedures.
Area of Science:
- Congenital Heart Surgery
- Pediatric Cardiology
- Cardiac Anatomy
Background:
- Transposition of the great arteries (TGA) is a critical congenital heart defect.
- Left ventricular outflow tract obstruction (LVOTO) presents a significant surgical challenge in TGA patients.
- Accurate anatomical assessment is crucial for determining the best surgical approach.
Purpose of the Study:
- To define optimal surgical strategies for TGA with concurrent LVOTO.
- To analyze the anatomical variations contributing to LVOTO in TGA.
- To evaluate the feasibility of different surgical interventions.
Main Methods:
- Retrospective analysis of 33 heart specimens with TGA and LVOTO.
- Categorization of specimens into TGA with intact ventricular septum (TGA/IVS), TGA with ventricular septal defect (TGA/VSD), and Taussig-Bing anomaly.
- Detailed examination of anatomical structures causing LVOTO and associated ventricular septal defects (VSDs).
Main Results:
- LVOTO in TGA/IVS involved pulmonary valve abnormalities, fibrous ridges, and muscular obstructions.
- Arterial switch operation (ASO) with LVOTO resection was feasible in most TGA/IVS cases.
- In TGA/VSD and Taussig-Bing, LVOTO involved complex combinations of valve, septal, and mitral valve anomalies.
- Surgical options included ASO with VSD closure, Rastelli, Nikaidoh procedures, or univentricular palliation, depending on anatomical constraints.
- Mitral valve anomalies significantly impacted the possibility of biventricular repair.
Conclusions:
- LVOTO resection and pulmonary valvotomy often allow for ASO in TGA.
- The Nikaidoh procedure is preferred for specific anatomical challenges like inlet VSDs or straddling mitral valves.
- Complex mitral valve anomalies can preclude biventricular repair in TGA with LVOTO.
Objective:
To describe the optimal surgical strategy in heart specimens with transposition of the great arteries (TGA) and left ventricular outflow tract obstruction (LVOTO).
Methods:
Thirty-three specimens with LVOTO were selected: TGA with intact ventricular septum (TGA/IVS) (10), TGA/VSD (21), and Taussig-Bing (2).
Results:
LVOTO in TGA/IVS consisted of combinations of bicuspid pulmonary valve (four), subpulmonary fibrous ridge (four), obstructive muscular conus (two) and bulging muscular septum (four). Arterial switch operation (ASO) with LVOTO resection/valvotomy was feasible in nine hearts. Obstructive anterior papillary muscle prohibited LVOTO relief in one specimen. In TGA/VSD and Taussig-Bing LVOTO consisted of combinations of bicuspid (nine) or unicommissural (one) pulmonary valve, fibrous ridge (three), obstructive muscular conus (five), malaligned outlet septum (six), accessory mitral valve tissue (two), straddling mitral valve (two) and anterior mitral valve rotation (four). VSDs were subpulmonary in 13 (9 perimembranous, 4 muscular), subaortic in 3 (2 perimembranous, 1 anterior muscular), doubly committed in 2, inlet in 3 (2 perimembranous, 1 muscular), non-committed and anterior in 1, and finally 1 VSD extended both into inlet and subpulmonary outlet septum. LVOTO resection and ASO with VSD closure was possible in 10. In six specimens, both a Rastelli and a Nikaidoh operation were feasible. For two hearts, a Nikaidoh procedure was the only option, while Rastelli was considered optimal in another specimen. Mitral valve anomalies prevented LVOTO relief in four, only permitting for Senning/VSD closure (one) or univentricular palliation (three).
Conclusions:
LVOTO resection and pulmonary valvotomy frequently permits an ASO. Inlet VSD, impossibility of VSD enlargement, straddling mitral valve, distant aorta and small right ventricle make the Nikaidoh procedure the best option. Mitral anomalies preventing LVOTO relief can make biventricular repair impossible.

