Congenitally Corrected Transposition of the Great Arteries

Abstract

Congenitally corrected transposition of the great arteries (ccTGA) can occur in isolation, or in combination with other structural cardiac anomalies, most commonly ventricular septal defect, pulmonary stenosis and tricuspid valve disease. Clinical recognition can be challenging, so echocardiography is often the means by which definitive diagnosis is made. The tricuspid valve and right ventricle are on the systemic arterial side of the ccTGA circulation, and are therefore subject to progressive functional deterioration. The natural history of ccTGA is also greatly influenced by the nature and severity of accompanying lesions, some of which require surgical repair. The traditional surgical approach (physiologic repair) of ccTGA attempts at restoring normal physiology by repairing the associated lesions. It fails to address the most serious anatomic abnormality, mainly ventriculoarterial discordance, and results in less than optimal long-term outcomes. Anatomic repair was introduced to incorporate the left ventricle into the systemic circulation. The excellent short-term and intermediate results of the double switch operation and its modifications make it the procedure of choice for the treatment of ccTGA. Occasionally, the anatomic peculiarities of ccTGA do not allow straightforward biventricular repair, and univentricular palliation is a reasonable option. Regardless of the approach selected, late cardiovascular complications are relatively common, so ongoing outpatient surveillance is required. This chapter provides a comprehensive overview of the pathology, pathophysiology and surgical management of ccTGA.