What does this research mean for the field?

Staged surgical management using a systemic-to-pulmonary shunt and mitral valve repair can facilitate right ventricular growth, enabling successful definitive Senning-Rastelli anatomical repair in congenitally corrected transposition of the great arteries with borderline ventricular volume and atrioventricular valve regurgitation. Novelty: ClaimNovelty.INCREMENTAL. Consensus alignment: ConsensusAlignment.NEUTRAL.

What question did this study set out to answer?

To report a staged surgical approach for anatomical repair in a child with congenitally corrected transposition of the great arteries complicated by dual atrioventricular valve regurgitation and inadequate ventricular volume.

October 7, 2026General Thoracic and Cardiovascular Surgery CasesOpen Access

Successful staged anatomic repair for congenitally corrected transposition with both atrioventricular valves’ regurgitation: a case report

Q: What is the clinical evidence from this study?

Study design: Case Report. Population: Congenitally corrected transposition of the great arteries with mitral and tricuspid regurgitation (n=1). Intervention: Staged anatomical repair (initial palliative systemic-to-pulmonary shunt with mitral valve repair followed by Senning-Rastelli operation). Primary outcome: Preservation of biventricular function and atrioventricular valve competence.

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Key result

Staged Senning-Rastelli repair preserves biventricular function with minimal valve regurgitation in complex transposition.

Why the study?

Management of congenitally corrected transposition with both atrioventricular valves’ regurgitation and inadequate right ventricular volume poses surgical challenges requiring staged repair strategies.

Does a staged anatomic repair improve biventricular function and oxygenation in pediatric patients with ccTGA, pulmonary stenosis, and significant atrioventricular valve regurgitation?

Key Points

To report a staged surgical approach for anatomical repair in a child with congenitally corrected transposition of the great arteries complicated by dual atrioventricular valve regurgitation and inadequate ventricular volume.
Evaluated a 4-year-old boy presenting with dextrocardia, congenitally corrected transposition of the great arteries, pulmonary valve stenosis, ventricular septal defect, and dual atrioventricular valve regurgitation using echocardiography and cardiac MRI.
Performed initial palliative surgery at 2 years and 2 months of age, consisting of a 4-mm systemic-to-pulmonary shunt from the innominate artery to the right pulmonary artery and direct-closure mitral valve repair.
Completed definitive anatomic repair at 4 years and 7 months of age using a Senning atrial switch, Rastelli operation, and Damus–Kaye–Stansel anastomosis with patch closure of the ventricular septal defect.
Initial palliation increased percutaneous oxygen saturation from 85% to approximately 90% and expanded right ventricular end-diastolic volume from 29.4 mL (118% of normal) to 55.3 mL (138% of normal), while reducing mitral regurgitation to trivial.
Definitive repair resolved hypoxemia with preserved biventricular function, minimal atrioventricular valve regurgitation, and no evidence of outflow tract or systemic venous pathway obstruction.
Cardiac catheterization at 8 months postoperatively demonstrated trivial mitral and tricuspid regurgitation, a left ventricular outflow tract pressure gradient of approximately 15 mmHg, and a left ventricular end-diastolic pressure of 11 mmHg.

Study Design

Type

Case Report (n=1)

Structured PICO

Does a staged anatomic repair improve biventricular function and oxygenation in pediatric patients with ccTGA, pulmonary stenosis, and significant atrioventricular valve regurgitation?

Population

A 4-year-old boy with dextrocardia, congenitally corrected transposition of the great arteries, pulmonary valve stenosis, ventricular septal defect, and progressive hypoxemia with mitral and tricuspid regurgitation.

Intervention

Staged anatomic repair (initial palliative approach at 2 years of age consisting of systemic-to-pulmonary shunt placement and mitral valve repair, followed by definitive Senning-Rastelli anatomic repair at 4 years and 7 months of age).

Outcome

Preserved biventricular function with minimal atrioventricular valve regurgitation and no evidence of outflow or pathway obstruction at 8-month follow-up.surrogate

Staged anatomical repair involving initial mitral valve repair and systemic-to-pulmonary shunt followed by definitive Senning-Rastelli operation is a viable strategy for ccTGA patients with significant atrioventricular valve regurgitation and inadequate ventricular volume.

Limitations

Long-term follow-up remains necessary
Case report with a single patient

Discussion

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Implication

Supports feasibility of staged repair in select ccTGA cases; leaves open need for larger studies on durability and applicability.

Cite This Study

Fuchigami et al. (2026) conducted a case report in Congenitally corrected transposition of the great arteries with mitral and tricuspid regurgitation (n=1). Staged anatomical repair (initial palliative systemic-to-pulmonary shunt with mitral valve repair followed by Senning-Rastelli operation) was evaluated on Preservation of biventricular function and atrioventricular valve competence. Staged anatomical repair comprising an initial palliative shunt and mitral valve repair followed by a Senning-Rastelli procedure resulted in preserved biventricular function with minimal regurgitation.

synapsesocial.com/papers/6ac5e087b2dceb1c429fa98b https://doi.org/10.1186/s44215-026-00281-2

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Successful staged anatomic repair for congenitally corrected transposition with both atrioventricular valves’ regurgitation: a case report

Key Points

Study Design

Structured PICO

Limitations

Discussion

Implication

Cite This Study

Successful staged anatomic repair for congenitally corrected transposition with both atrioventricular valves’ regurgitation: a case report

Key Points

Study Design

Structured PICO

Limitations

Discussion

Implication

Cite This Study

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