ccTGA: Surgical Strategy Overview

1. Core concept

In congenitally corrected transposition of the great arteries (ccTGA), operative planning should be based not simply on the diagnostic label, but on the functional anatomy and ventricular preparedness. The principal determinants are:

1. the presence or absence of pulmonary stenosis (PS) or morphologic LV outflow obstruction
2. the presence, size, and spatial suitability of a ventricular septal defect (VSD), and
3. whether the morphologic left ventricle (LV) is capable of sustaining the systemic circulation [1].

The central surgical question is therefore:

Can the morphologic LV be reassigned safely to the systemic circulation, and by which reconstructive route?

If the answer is yes, an anatomic repair is generally favored. If not, a physiologic repair may still be required in selected patients [1].

2. Why this decision matters

The long-term weakness of unrepaired or physiologically repaired ccTGA is that the morphologic right ventricle (RV) remains the systemic ventricle. Over time, this systemic RV is vulnerable to progressive dysfunction, worsening systemic tricuspid regurgitation, arrhythmia, heart failure, and late clinical deterioration [1,8]. These natural-history concerns are the major reason why contemporary strategy has increasingly shifted toward anatomic repair whenever anatomy and timing permit [1-3].

Recent pooled evidence supports this shift. In a 2023 systematic review and meta-analysis of 44 studies including 1,857 patients, anatomic repair was associated with lower post-discharge mortality, lower reoperation rates, and substantially less postoperative ventricular dysfunction than physiologic repair [2]. A 2024 meta-analysis of 47 studies including 2,844 patients similarly showed better long-term survival and reintervention-free survival after anatomic repair, with a 10-year mortality advantage favoring anatomic over physiologic repair [3].

3. The major anatomic pathways of repair

From a practical surgical standpoint, the anatomy of ccTGA usually directs the surgeon toward one of four broad strategies:

• Senning + arterial switch operation (double switch)

for patients without PS in whom the pulmonary valve/LV outflow can support arterial switch and the LV is adequately prepared [1].

• Pulmonary artery banding (PAB) followed by double switch

for patients without PS but with an underprepared LV that requires retraining before systemic reassignment [1,4,5].

• Senning + Rastelli

for patients with PS or LV outflow obstruction in whom the pulmonary valve cannot be used in an arterial switch, but a large and favorably positioned VSD allows intraventricular rerouting of LV flow to the aorta [1].

• Conventional (physiologic) repair

for patients in whom anatomic reassignment is not feasible because of unfavorable VSD anatomy, inadequate LV preparedness, or prohibitive outflow/coronary relationships [1,7].

This framework is conceptually simple, but actual decision-making remains highly individualized because ccTGA is anatomically heterogeneous and surgical suitability depends on geometry as much as on diagnosis [1,7].

4. ccTGA without pulmonary stenosis: the Senning + ASO pathway

4.1 Fundamental rationale

In ccTGA without pulmonary stenosis, the pulmonary valve and subpulmonary LV outflow may be suitable for arterial switch, making Senning + ASO the principal anatomic option. In this setting, the key issue is not merely the absence of PS, but whether the morphologic LV has been exposed to enough pressure load to function as a systemic ventricle after repair [1].

4.2 When LV pressure is preserved

If LV pressure remains near systemic level, the patient may proceed directly to an anatomic repair. This commonly occurs when a VSD is present, because the VSD helps maintain LV pressure and prevents the low-pressure regression seen in isolated ccTGA [1]. In such patients, an elective double switch can often be planned without prior LV retraining.

4.3 When no VSD is present

In ccTGA without PS and without VSD, the morphologic LV is committed to the low-pressure pulmonary circulation. As pulmonary vascular resistance falls after birth, LV mass and pressure may progressively decline, making late systemic reassignment more difficult or impossible [1,5]. This is why timing becomes crucial: either the operation should be performed before LV deconditioning becomes advanced, or the LV must first be retrained [1,5].

5. Pulmonary artery banding and LV retraining

5.1 Why PAB is used

When the anatomy is otherwise favorable for anatomic repair but the LV is underprepared, pulmonary artery banding (PAB) can be used to increase LV afterload and stimulate hypertrophy, geometric remodeling, and pressure adaptation before a later double switch [1,4,5]. In this sense, PAB is not merely a temporizing maneuver; it is a deliberate strategy to restore systemic competence to the morphologic LV.

5.2 What must be assessed

The adequacy of LV retraining should be judged serially by integrating:

• LV pressure relative to systemic pressure,
• LV wall thickness and mass,
• septal geometry,
• global LV systolic function,
• atrioventricular valve competence, and
• overall clinical tolerance [1,5].

5.3 What contemporary data suggest

Modern series support the feasibility of this approach, particularly in younger patients. Ibrahimiye and colleagues reported that LV retraining followed by double switch was effective in selected patients, with successful progression to definitive repair in most of their cohort and excellent midterm outcomes after double switch [4]. Myers and colleagues further showed that earlier banding and earlier anatomic repair were associated with more favorable LV and neo-aortic valve function, whereas delayed training was associated with a higher incidence of postoperative LV dysfunction [5]. These findings align with more recent comparative analyses showing better outcomes when anatomic repair is performed at younger ages and when preoperative PAB has been used appropriately [3].

6. Double switch operation: current position and limitations

Among anatomic strategies, the double switch operation has emerged as the preferred option when anatomy permits arterial switch and the LV is or can be made systemically competent [2,3]. In the 2023 meta-analysis by Anzai et al., double-switch procedures had lower in-hospital mortality and lower reoperation rates than Senning + Rastelli among patients undergoing anatomic repair [2].

At the same time, double switch is not free of late burden. Contemporary outcome data show generally favorable survival, but also emphasize the importance of late surveillance for:

• neo-aortic valve regurgitation,
• baffle-related reintervention,
• conduction disease and pacemaker requirement, and
• residual or recurrent ventricular dysfunction [6].

In a recent large single-center series, transplant-free survival after double switch remained high at midterm follow-up, but neo-aortic valve intervention and rhythm-device therapy were important components of late management [6]. Thus, the operation is best understood not as a definitive cure in all cases, but as the most durable anatomy-restoring strategy in appropriately selected patients.

7. ccTGA with pulmonary stenosis: why the pathway changes

When pulmonary stenosis is present, the operative logic changes fundamentally. The stenotic pulmonary valve/subpulmonary outflow cannot serve as an acceptable systemic outflow pathway after arterial switch, so a standard Senning + ASO is generally not feasible [1].

At this point, the next question is whether a VSD exists and is usable for rerouting. This is not simply a binary issue of presence versus absence. The VSD must be:

• large enough,
• favorably located,
• alignable with the aortic outflow, and
• suitable for tunnel construction without causing residual LV outflow obstruction or distortion of adjacent structures [1].

This geometric suitability determines whether the patient is a candidate for Senning + Rastelli or must instead undergo conventional physiologic repair.

8. Senning + Rastelli: when PS coexists with a usable VSD

If PS is present and the VSD is large and favorably positioned, an anatomic repair can still be achieved by combining:

1. an atrial switch to redirect venous return appropriately, and
2. a Rastelli-type intraventricular rerouting to direct LV flow through the VSD to the aorta, together with an RV-to-PA conduit for pulmonary blood flow [1].

The conceptual strength of this strategy is that it still places the morphologic LV in the systemic position, which remains the overarching goal of anatomic repair [1-3]. However, its technical success depends heavily on intracardiac geometry. A remote, restrictive, malaligned, or otherwise unfavorable VSD may make the tunnel hazardous, obstructive, or impossible.

This limitation is reflected in comparative outcome data. Although Senning + Rastelli remains an important reconstructive option, contemporary pooled evidence suggests that it carries a greater reintervention burden and less favorable early comparative metrics than arterial-switch-based anatomic pathways [2]. This is likely related in part to VSD geometry, conduit-related reintervention, and the complexity of the reconstructed outflow tracts.

9. Conventional (physiologic) repair: when anatomic reassignment is not feasible

If PS is present but the VSD is absent, too small, or not suitable for rerouting, the anatomy may not support an anatomic repair. In that setting, a conventional physiologic repair remains an appropriate option [1,7]. Depending on the anatomy, this may include closure of the VSD, relief or bypass of outflow obstruction, and conduit-based pulmonary blood flow reconstruction, while leaving the morphologic RV in the systemic position.

This strategy is less anatomically corrective, but it may be the safer and more rational option when:

• the LV cannot be adequately retrained,
• VSD geometry is unsuitable for Rastelli-type baffling,
• coronary or outflow anatomy is prohibitive,
• or overall surgical risk outweighs the theoretical long-term benefit of anatomic reassignment [1,7].

The tradeoff, however, is clear: the systemic ventricle remains the morphologic RV, and therefore the patient remains exposed to the longer-term risks of systemic RV failure and progressive systemic tricuspid regurgitation [1,8].

10. A practical anatomy-based decision algorithm

A useful operative framework is as follows:

Step 1. Is pulmonary stenosis / LV outflow obstruction present?

• No → evaluate for double switch (Senning + ASO).
• Yes → ASO is generally not feasible; evaluate for Senning + Rastelli or physiologic repair [1].

Step 2. Is the morphologic LV adequately prepared for systemic work?

• Yes → proceed toward anatomic repair.
• No → consider PAB for LV retraining, especially in younger patients [1,4,5].

Step 3. Is there a VSD, and is it usable?

• Present and favorable → may preserve LV pressure or permit intraventricular rerouting.
• Absent or unfavorable → narrows the options and may preclude Rastelli-type repair [1].

Step 4. Can the systemic circulation ultimately be assigned to the morphologic LV?

• If yes → anatomic repair is usually preferred.
• If no → physiologic repair may be necessary [1-3].

This stepwise logic reflects both contemporary surgical philosophy and real-world practice patterns observed in multicenter data [7].

11. Contemporary perspective

The modern literature increasingly supports the principle that anatomic repair offers superior long-term survival and ventricular preservation compared with physiologic repair, provided that patient selection is appropriate [2,3]. The strongest current evidence favors:

• early recognition of candidates
• timely LV retraining when needed, and
• anatomy-specific selection of double switch versus Rastelli-based pathways [1-6].

At the same time, the best operation is still not universal. The heterogeneity of ccTGA means that decision-making must remain individualized, taking into account age, ventricular conditioning, VSD geometry, outflow relationships, tricuspid valve competence, and institutional expertise [1,7]. Emerging reconstructive strategies, including selected root translocation-based approaches, may further expand anatomic options in highly specific anatomies, but these remain specialized solutions rather than standard pathways [9].

12. Take-home summary

• Surgical strategy in ccTGA is fundamentally anatomy-driven.
• The key variables are PS/LV outflow anatomy, VSD anatomy, and LV preparedness.
• No PS + preserved/preparable LV favors Senning + ASO.
• No PS + underprepared LV may require PAB before double switch.
• PS + large usable VSD favors Senning + Rastelli.
• PS + absent or unusable VSD often necessitates conventional physiologic repair.
• The overall objective, whenever feasible, is to place the morphologic LV in the systemic position, thereby avoiding the long-term liabilities of a systemic morphologic RV [1-3,8].

References

[1] Miller JR, Sebastian V, Eghtesady P. Management Options for Congenitally Corrected Transposition: Which, When, and for Whom? Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu. 2022;25:38-47.

[2] Anzai I, Zhao Y, Dimagli A, Pearsall C, LaForest M, Bacha E, Kalfa D. Outcomes After Anatomic Versus Physiologic Repair of Congenitally Corrected Transposition of the Great Arteries: A Systematic Review and Meta-Analysis. World J Pediatr Congenit Heart Surg. 2023;14(1):70-76.

[3] Jacob KA, Hörer J, Hraska V, Agbor VN, Duchateau S, van Wijk A, Barron DJ, Schoof PH. Anatomic and Physiologic Repair of Congenitally Corrected Transposition of the Great Arteries. J Am Coll Cardiol. 2024;84(25):2471-2486.

[4] Ibrahimiye AN, Mainwaring RD, Patrick WL, Downey L, Yarlagadda V, Hanley FL. Left Ventricular Retraining and Double Switch in Patients With Congenitally Corrected Transposition of the Great Arteries. World J Pediatr Congenit Heart Surg. 2017;8(2):203-209.

[5] Myers PO, del Nido PJ, Geva T, Bautista-Hernandez V, Chen P, Mayer JE Jr, Emani SM. Impact of Age and Duration of Banding on Left Ventricular Preparation Before Anatomic Repair for Congenitally Corrected Transposition of the Great Arteries. Ann Thorac Surg. 2013;96(2):603-610.

[6] Marathe SP, Chávez M, Schulz A, Sleeper LA, Marx GR, Emani SM, Del Nido PJ, Baird CW. Contemporary Outcomes of the Double Switch Operation for Congenitally Corrected Transposition of the Great Arteries. J Thorac Cardiovasc Surg. 2022;164(6):1980-1990.e7.

[7] Chew JD, Hill KD, Soslow JH, Jacobs ML, Jacobs JP, Eghtesady P, Thibault D, Chiswell K, Bichell DP, Godown J. Congenitally Corrected Transposition Cardiac Surgery: Society of Thoracic Surgeons Database Analysis. Ann Thorac Surg. 2022;114(5):1715-1722.

[8] Prieto LR, Hordof AJ, Secic M, Rosenbaum MS, Gersony WM. Progressive Tricuspid Valve Disease in Patients With Congenitally Corrected Transposition of the Great Arteries. Circulation. 1998;98(10):997-1005.

[9] Zubrzycki M, Schramm R, Costard-Jäckle A, Morshuis M, Grohmann J, Gummert JF, Zubrzycka M. Pathogenesis and Surgical Treatment of Congenitally Corrected Transposition of the Great Arteries (ccTGA): Part III. J Clin Med. 2024;13(18):5461.