Introduction: Congenitally corrected transposition of the great arteries (ccTGA) is a rare congenital heart defect characterized by atrioventricular and ventriculo-arterial discordance. CcTGA can be diagnosed at any stage of life. The natural history of the disease depends on concomitant anomalies present in most of the cases, progression of systemic ventricular dysfunction and conduction disturbances.
Areas covered: This review describes diagnosis of the anomaly and summarizes the current knowledge on etiology and prognosis in ccTGA patients. Furthermore, interventional and pharmacological approaches to ccTGA management are discussed. The areas requiring further research are highlighted.
Expert opinion: Although advances in diagnosis and treatment continue to improve outcomes for ccTGA, patients are burdened with significant morbidity and mortality. Optimal approaches to surgical management of the anomaly, as well as prevention and management of heart failure, are still not established. Future research should focus on the long-term effect of anatomic repair, potential benefits of novel pharmacological strategies for heart failure therapy, and the optimal mode of pacing in ccTGA patients. However, the issues might be difficult to address due to rarity of the disease and its heterogenous clinical presentation. As the life-expectancy of ccTGA patients improves, acquired cardiovascular disorders will become another serious concern.
Keywords: Congenital heart disease; cardiac surgery; congenitally corrected transposition of the great arteries; heart failure; pacing; pediatric cardiology; systemic right ventricle.