Congenitally corrected transposition of the great arteries or l-transposition of the great arteries is characterized by discordance of both the atrioventricular and ventriculoarterial connections. Physiologic repair of associated conditions, whereby the morphologic right ventricle remains the systemic ventricle, has resulted in unsatisfactory long-term outcomes due to the development of right ventricular failure and tricuspid valve regurgitation. While intuitively attractive, anatomic repair also has inherent challenges and risks, particularly for those patients who present late and require left ventricular retraining. Although early and intermediate-term outcomes for anatomic repair have been encouraging, longer-term follow-up has demonstrated concern for late left ventricular dysfunction in this subgroup of patients. Continued monitoring of this challenging patient population will clarify late outcomes and inform clinical management in the future.
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