Transposition of the great arteries is a common congenital heart defect causing cyanosis in neonates, occurring in 0.2 per 1000 live births. It has been reported to occur with other associated congenital heart lesions. However, its association with a superior sinus venosus defect and partially anomalous pulmonary venous return has not been reported. We present a neonate with transposition of the great arteries, superior sinus venosus defect with partially anomalous pulmonary venous return that underwent successful complete neonatal repair, and discuss important physiological and anatomic considerations.

Primary care cardiology, also known as ambulatory or outpatient cardiology, for the longitudinal management of patients with common arterial trunk or with transposition of the great arteries is both poorly described and has limited evidence to justify its basis. This article discusses some of the various complications that these patients can develop, reviews the medical literature, and describes a framework for care of these complex patients from infancy to transition to adult congenital cardiac specialist care.

The arterial switch operation has become the standard of surgical repair for total correction of complete transposition (concordant atrioventricular and discordant ventriculoarterial connections). A standard nomenclature for classification of the numerous anatomic relationships the coronary arteries may assume, however, has yet to be universally adopted. Inconsistent use of varied nomenclatures remain an obstacle to effective communication between physicians and surgeons. In this review we offer a unified nomenclature incorporating five important relationships: the configuration of the sinuses; their location; the configuration of the arterial orifices; the pattern of branching of the major coronary arteries; and the course of each artery.