Anesthetic Management of Resection of Metastatic Occipital Malignancy in a Patient With Fontan Physiology

Significant advances in surgical management have allowed patients with congenital heart disease to survive to adulthood. Often, these patients present for non-cardiac surgeries, including patients who have undergone the three-staged Fontan repair for congenital single ventricle. The primary aim in the anesthetic management of adult patients with Fontan physiology is to maintain adequate venous pressure, low pulmonary vascular resistance (PVR) and normal contractility to maintain the cardiac output. We present the case of a 26-year-old female with Fontan physiology following a three-staged Fontan repair for tricuspid atresia who underwent a stealth-guided left occipital craniotomy for the palliative resection of a metastatic brain tumor. This case highlights the importance of understanding Fontan physiology and its implications in the anesthetic management of a patient undergoing an open craniotomy. These patients require a high central venous pressure and low PVR to maintain optimum venous return to the left atrium. A rise in PVR can result in the shunting of the deoxygenated blood from the Fontan shunt to the systemic circulation. Hence, alpha agonists and high airway pressure are to be avoided. To minimize the risk of perioperative mortality, there is an increased need to optimize systemic to pulmonary blood flow ratios and maintain normal arterial saturation and euvolemic fluid status.