Robotic Adrenalectomy for Pheochromocytoma in a patient with Fontan Physiology

Abstract A strong association exists between cyanotic congenital heart disease and pheochromocytoma, with chronic hypoxia speculated as a likely pathogenic mechanism. Excess catecholamines secreted by the pheochromocytoma can induce heart failure and arrhythmias, particularly in patients with Fontan physiology. Minimally invasive (MIS) resection of pheochromocytomas can pose unique challenges in patients with Fontan physiology. Here we present a complex case of anesthetic management of a robotic transabdominal pheochromocytoma resection in a patient with Fontan palliation. A 36 year old female with prior Fontan palliation presented for robotic adrenalectomy for pheochromocytoma. The patient remained hemodynamically stable throughout induction and insufflation. Intraoperative transesophageal echocardiogram revealed pulmonic atresia with hypoplastic right ventricle, a non-restrictive secundum atrial septal defect, and normal systemic ventricular size and function. The patient experienced one episode of sudden and severe hypertension during clamping of the adrenal effluent vein, which responded to intravenous fentanyl, propofol, and nicardipine. The remainder of the procedure was uneventful. Pathology confirmed the diagnosis of pheochromocytoma. Pheochromocytoma can have lethal consequences for Fontan patients. MIS adrenalectomy can offer faster recovery afforded by amelioration of pain and fluid shifts associated with laparotomy. While abdominal insufflation can cause negative hemodynamic derangements, these authors present a successful robotic approach to pheochromocytoma resection in a patient with Fontan physiology.