Surgical Strategy According to Anatomy in ccTGA
#4 Atrial Switch and Rastelli Procedure
Rationale: ccTGA with pulmonary stenosis or atresia
In congenitally corrected TGA (ccTGA) with significant pulmonary stenosis or pulmonary atresia (PS/PA+), the morphologic left ventricle (LV) faces a narrowed pulmonary valve and/or subpulmonary outflow tract. In this setting, a standard arterial switch operation (ASO) is not appropriate, because the stenotic pulmonary valve cannot reliably serve as the neo-aortic valve and would expose the systemic circulation to residual obstruction and regurgitation [1].
Many of these patients also have a large ventricular septal defect (VSD) that opens toward the aorta. This VSD can be exploited as a channel to reroute LV outflow directly to the aorta, bypassing the obstructed LV–PA pathway. When this intracardiac rerouting is combined with an atrial switch, the result is an anatomic repair with a systemic LV and a subpulmonary RV—the Senning (or Mustard) + Rastelli operation [1,2].
Anatomic prerequisites
A Senning–Rastelli strategy is considered when all of the following conditions are met:
- Pulmonary stenosis or atresia (PS/PA+) is present, such that the native pulmonary valve is unsuitable as a neo-aortic valve.
- A VSD is present and sufficiently large to accommodate an unobstructed LV outflow tunnel.
- The VSD is favourably aligned with the aorta (subaortic or doubly committed), allowing construction of a short, straight baffle from the LV to the aortic valve without excessive angulation or narrowing [1,3].
If the VSD is small, remote, or absent, it cannot be used as a reliable systemic outflow pathway. In such cases, options are limited to physiologic repair (relieving PS while leaving the morphologic RV systemic) or alternative complex strategies, and the long-term advantages of an anatomic repair may not be achievable [3,4].
Operative concept: Senning + Rastelli
- Atrial switch (Senning or Mustard)
- Systemic venous return (SVC/IVC) is redirected to the morphologic LV, and pulmonary venous return is channeled to the morphologic RV.
- After this step, the LV becomes the inlet for systemic venous blood, and the RV receives pulmonary venous blood, restoring physiologic AV connections [2,3].
- Intracardiac rerouting of LV outflow (Rastelli component)
- Through the large, favourably positioned VSD, an intraventricular baffle is constructed from the LV to the aorta, committing the VSD to the systemic outflow.
- The LV now ejects directly into the aorta, completely bypassing the stenotic subpulmonary outflow and pulmonary valve.
- The baffle is designed to be short, wide, and smoothly contoured to minimize turbulence and prevent late left ventricular outflow tract (LVOT) obstruction [1–3].
- RV–PA reconstruction with a conduit
- With the LV connected to the aorta, the morphologic RV must now supply the pulmonary arteries.
- A valved RV–PA conduit (homograft or valved prosthetic conduit) is inserted from the RV outflow to the main or branch pulmonary arteries, re-establishing an unobstructed pulmonary circulation [1,2].
The final configuration achieves full anatomic correction: the morphologic LV supports the systemic circulation via the aorta, and the morphologic RV functions as the subpulmonary ventricle, ejecting through a conduit to the pulmonary arteries, while the native PS/PA is effectively excluded from the systemic pathway.
Outcomes and long-term considerations
Contemporary series of anatomic repair for ccTGA—including patients treated with Senning–Rastelli—report excellent early survival and good functional status in the majority of survivors [2–4]. In large single-centre cohorts, overall survival after anatomic correction exceeds 90% at 10–20 years, although reintervention is common, particularly for RV–PA conduits and baffle-related issues [2,4]. Meta-analyses comparing anatomic and physiologic strategies suggest that, in appropriately selected patients, anatomic repair provides superior systemic ventricular function and lower risk of systemic AV valve failure, at the cost of higher rates of reoperation [3,4].
Key long-term issues after Senning–Rastelli include:
- RV–PA conduit deterioration – Structural degeneration, stenosis, or regurgitation of the conduit is expected, with many patients requiring one or more conduit replacements during adolescence or early adulthood [2–4].
- Baffle-related obstruction – Narrowing or kinking of the LV–aorta tunnel can lead to LVOT gradients; careful initial geometry and surveillance with imaging are essential.
- Atrial switch sequelae – As with other Senning/Mustard procedures, patients remain at risk for sinus node dysfunction and atrial arrhythmias, and some will require pacemaker implantation or antiarrhythmic therapy [2,4].
- Tricuspid valve and RV function – Because the morphologic RV is placed on the low-pressure pulmonary side, progression of tricuspid regurgitation and systemic RV failure is generally mitigated compared with physiologic repair, but detailed follow-up of AV valve function remains important [3,4].
Summary
For ccTGA patients with pulmonary stenosis or atresia and a large, favourably aligned VSD, an atrial switch combined with a Rastelli procedure offers a coherent anatomic solution. The VSD is used as a pathway to reroute LV outflow to the aorta, while an RV–PA conduit restores pulmonary blood flow. When these anatomic prerequisites are met, Senning + Rastelli:
- corrects the AV and VA discordance,
- bypasses the obstructed pulmonary outflow,
- and places the morphologic LV in the systemic position, which is associated with better long-term ventricular performance compared with leaving the morphologic RV systemic.
These benefits must be balanced against the predictable need for future conduit and baffle reinterventions and the rhythm-related risks inherent to an atrial switch, underscoring the importance of careful patient selection and lifelong surveillance.
References
[1] Ilbawi MN, DeLeon SY, Backer CL, Zales VR, Duffy CE, Muster AJ, Idriss FS. An alternative approach to the surgical management of physiologically corrected transposition with ventricular septal defect and pulmonary stenosis or atresia. J Thorac Cardiovasc Surg. 1990;100(3):410-415.
[2] Brizard CP, Lee A, Zannino D, Davis AM, Fricke TA, d’Udekem Y, Konstantinov IE, Brink J, Cheung MMH. Long-term results of anatomic correction for congenitally corrected transposition of the great arteries: A 19-year experience. J Thorac Cardiovasc Surg. 2017;154(1):256-265.e4.
[3] He X, Shi B, Song Z, Pan Y, Luo K, Sun Q, Zhu Z, Xu Z, Zheng J, Zhang Z. Congenitally corrected transposition of the great arteries: Mid-term outcomes of different surgical strategies. Front Pediatr. 2022;9:791475.
[4] Jacob KA, van der Ven JPG, Bouma BJ, Koolbergen DR, Blom NA, Hazekamp MG, et al. Outcomes after anatomic versus physiologic repair of congenitally corrected transposition of the great arteries: A systematic review and meta-analysis. World J Cardiol. 2020;12(8):427-444.