Management Options for Congenitally Corrected Transposition: Which, When, and for Whom?

Area of Science:

• Cardiology
• Pediatric Cardiology
• Congenital Heart Disease

Background:

• Congenitally corrected transposition of the great arteries (ccTGA) management historically favored physiologic repair, using the morphologic right ventricle (mRV) for systemic circulation.
• This approach persisted due to acceptable short-term results and concerns about anatomical repair outcomes, despite eventual systemic mRV failure.

Observation:

• Long-term systemic mRV dysfunction is a significant risk associated with physiologic repair.
• Improved outcomes with anatomical repair and feasibility of morphologic left ventricle retraining are changing management perspectives.
• Key considerations include managing a failing mRV, systemic tricuspid valve regurgitation, left ventricle outflow tract obstruction, and pacing strategies.

Findings:

• Biventricular pacing is superior to univentricular pacing in ccTGA.
• Tricuspid regurgitation requires early management, potentially involving anatomical repair or tricuspid valve replacement, not repair.
• Recent long-term data increasingly support anatomical repair as the preferred strategy for most ccTGA patients.

Implications:

• The shift towards anatomical repair in ccTGA aims to improve long-term patient survival and quality of life.
• Further research is needed to fully elucidate optimal management strategies for complex ccTGA cases.
• Standardizing management protocols across centers could improve the interpretation of outcomes data and patient care.