Anesthetic Challenges in an Adult With Single-Ventricle Physiology and Incomplete Cavopulmonary Palliation Undergoing Noncardiac Interventions.

BACKGROUND

Patients with single-ventricle physiology may present with complex forms rendering Fontan palliation impossible.

CASE SUMMARY: After multidisciplinary discussion, a 57-year-old man with a history of a right Glenn shunt and a left nonfunctional modified Blalock-Taussig shunt and left aortopulmonary collaterals for pulmonary atresia, hypoplastic right heart and discontinuous pulmonary arteries, required general anesthesia for noncardiac procedures. Intraoperatively, pulmonary blood flow was maintained by avoiding any increase in pulmonary vascular resistance and any reduction in cavopulmonary venous return on the right side and by maintaining systemic blood pressure and adequate cardiac output on the left side.

DISCUSSION: Although the long-term survival of patients with single-ventricle physiology without Fontan palliation is described, there is little known about their anesthetic management.

TAKE-HOME MESSAGE: The intraoperative management of patients not palliated with the Fontan must be tailored to the existing anatomy and physiology and carried out in collaboration with congenital cardiac anesthesiologists.