Anesthetic Management of an Adult Male with Fontan Physiology for Thoracic Arachnoid Cyst Excision: A Case Report

Background: Patients with successful corrections for congenital cardiac anomalies present into adulthood with complex cardiac physiology. Here, we report the successful management of an adult with tricuspid atresia, post– Fontan surgery who underwent excision of a thoracic arachnoid cyst with intraoperative motor evoked potential (MEP) monitoring.

Case Description: A 19-year-old, male patient weighing 58 kg was scheduled for an elective D1–D2 laminectomy and excision of an arachnoid cyst under general anesthesia. Diagnosed with tricuspid atresia at birth, he underwent modified right Blalock–Taussig shunt as a neonate and a hemi-Fontan procedure at 8 months of age. He was NYHA class 2 until 1 month ago until he developed muscle weakness with grade-4 power. The ECHO done preoperatively showed functional hemi-Fontan’s shunt, restrictive VSD 2 mm, small ASD, rudimentary RV, dilated LV, and a normal LV systolic function. After establishing standard ASA monitors, induction, and tracheal intubation were performed with intravenous fentanyl, ketamine, etomidate, and atracurium. Anesthesia was maintained with sevoflurane, MAC 0.5 and propofol infusion titrated with BIS. The patient was positioned carefully in prone with transcutaneous pacing pads and MEP was monitored intraoperatively to guide excision.

Conclusion: Understanding the Fontan physiology, the single ventricle physiology and pulmonary blood flow is critical to maintain hemodynamic stability. Anesthetic management, enabling intraoperative MEP monitoring, while maintaining cardiac function in the setting of altered cardiac physiology is essential for successful management.

Publication History

Article published online:
25 March 2020