Anesthesia for the Patient with a Single Ventricle

Since first described in 1971, the principle of the Fontan operation has been applied to the full spectrum of cardiac lesions with one functional ventricle. Essentially all patients with a single functional ventricle now undergo staged palliation, resulting in completion of the Fontan circuit at 18 months to 4 years of age. The majority undergo a neonatal palliative procedure, followed by a superior cavopulmonary connection (bidirectional Glenn or hemi-Fontan procedure) at 3–6 months of age in all patients. This chapter will initially present these principles by using hypoplastic left heart syndrome (HLHS) as the most common single-ventricle congenital cardiac lesion. Tricuspid atresia and other forms of single-ventricle lesions will then be discussed. Finally, the growing recognition of the long-term complications and sequelae of the Fontan circulation, along with management strategies and outcomes, will be described.