Adult Congenital Heart Disease Patient Presenting for Non-Cardiac Surgery.

A 26 year old female with a history of transposition of great arteries, ventricular septal defect, and severe pulmonary stenosis, status post Fontan repair at the age of 12 years old, initially presented with abdominal pain, diarrhea, and persistent nausea. CT scan subsequently identified paragangliomas on core biopsy, and patient was scheduled for laparoscopic resection. After uneventful induction, intubation, and line placement, open laparotomy with planned excision of four abdominal paragangliomas ensued. Patient was hemodynamically tenuous throughout the case, yet was successfully extubated at the completion of surgery.

Due to advances in prenatal diagnosis, interventional cardiovascular procedures, pediatric cardiac anesthesia, post-operative critical care, and efficacy of cardiovascular pharmacologic agents, there has been an overall increase in post-repair survival rates in patients with congenital heart diseases.Therefore, a growing number of adults with congenital heart disease (ACHD) may require non-cardiac surgeries as they age. Since these ACHD patients experience an increased rate of perioperative morbidity and mortality, it is recommended that all elective surgical procedures be performed in a referral center with skilled surgeons and anesthesiologists. Knowledge of pathophysiology of various congenital heart anomalies, as well as the effect of these chronic pathologic changes on other organ systems, aids in individualized and safe management of ACHD patients. A thorough and comprehensive preoperative evaluation is often required, including assessment of cardiac lesions and resultant altered physiology. Here we review hemodynamics and anesthetic management of sympathetic surges during paraganglioma resection in a patient with complicated cardiac physiology.