• Koichi Yuki, Alfonso Casta, and Shoichi Uezono.
• Department of Anesthesiology, Perioperative and Pain Medicine, Childrens Hospital Boston, 300 Longwood Avenue, Bader 3, Boston, MA 02115, USA. yuki.koichi@gmail.com
• J Anesth. 2011 Apr 1; 25 (2): 247-56.

AbstractPatients with congenital heart diseases are a growing population, and noncardiac surgeries will become an important health care issue. Patients with single ventricle physiology are a particularly challenging population who will undergo staged, palliative repair toward a final step of Fontan circulation. Although Fontan surgery creates a serial circulation in which the ventricle pumps blood to the systemic circuit, pulmonary blood flow occurs without a dedicated ventricle. Despite progress in outcomes, this abnormal circulation remains associated with various co-morbidities such as ventricular dysfunction, arrhythmias, protein losing enteropathy, and plastic bronchitis. Health care professionals must prepare for these patients to present to noncardiac surgery at any stage of intervention, possibly with complications. Given that staged, palliative repair has undergone multiple modifications, patients who present for surgery can vary in types and timing of the repair. Anesthesiologists who care for them must be familiar with perioperative issues to optimize outcomes, especially because congenital heart disease is a risk factor for increased mortality for noncardiac surgery.

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