Levo-transposition of the great arteries is a rare anomaly, comprising less than 1% of all forms of congenital heart disease and is characterized by both atrioventricular and ventriculoarterial discordance. Despite ventriculoarterial discordance, oxygenated and deoxygenated blood flow is in the correct physiologic direction, resulting in normal oxygenation and perfusion. Patients without other structural cardiac defects are asymptomatic at birth and during early childhood and usually present later in life. The long-term sequelae of patients with uncorrected levo-transposition include right ventricular dysfunction, systemic atrioventricular valve regurgitation, congestive heart failure, arrhythmias, and complete heart block. The natural history of the disease is largely determined by the presence of associated anatomic cardiac abnormalities and the progressive dysfunction of the morphologic right ventricle as the systemic pump. This chapter details the anesthetic management of a patient with levo-transposition presenting for an urgent appendectomy, including management of a pacemaker.

In “simple” dextro-transposition of the great arteries the great vessels originate from the wrong ventricle. Blood flows in a parallel fashion with deoxygenated blood recirculating to the body and oxygenated blood recirculating to the lungs. The resultant physiology is not compatible with life unless there is mixing between circulations. In the late 1960s, ingenious ways of rerouting atrial blood to the opposite ventricle were developed, and atrial switch procedures became the procedure of choice for the next 15–20 years until the arterial switch operation became standard treatment for this lesion. The atrial switch is no longer performed, but as of 2008, there were an estimated 9000 adult congenital heart disease patients in the United States who had undergone atrial switch procedures performed for the correction of dextro-transposition of the great arteries. This chapter discusses the assessment and management of an adult patient who underwent the atrial switch procedure and the perioperative concerns involved in care of these patients.