Laparoscopic cholecystectomy in a patient with Fontan circulation.

Background: Fontan circulation is created when a baby is born with only one functioning cardiac ventricle. A series of surgeries are performed to allow the ventricle to provide oxygenated blood to the systemic circulation and to create passive flow of venous blood to the pulmonary circulation via a conduit. Laparoscopic surgery poses several hemodynamic challenges to a patient with Fontan physiology attributable to carbon dioxide insufflation, positive pressure ventilation, and reverse Trendelenburg positioning.

Clinical Features: A 39-yr-old male with a Fontan physiology was referred to our tertiary care centre because of repeated bouts of cholecystitis requiring a percutaneous drain and now elective laparoscopic cholecystectomy. Because of repeated cardiac surgeries, the patient also had complete heart block and was pacemaker dependent. We placed an arterial catheter prior to induction of general anesthesia with tracheal intubation. Transesophageal echocardiography allowed for real-time intraoperative assessment of venous blood flow through the patient's extracardiac diversion system throughout the surgery. This information was used to guide management and determine circulation tolerance during the various stages of laparoscopy. Inhaled milrinone resulted in the shunt fraction returning to the patient's baseline. Intraperitoneal pressure was kept below 10 mm Hg, and systemic blood pressure was supported with a low-dose norepinephrine infusion.

Conclusions: Intraoperative transesophageal echocardiography is a useful monitoring device during laparoscopic surgery when a patient has Fontan circulation. Knowing how to administer inhaled milrinone is a useful skill to decrease the shunt fraction through a patient's conduit, increasing pulmonary blood flow while avoiding hypotension.