Management of Acute Cholecystitis in a Patient With Eisenmenger Syndrome and Abdominal Heterotaxy: A Case Report

BACKGROUND Eisenmenger syndrome presents a unique challenge for the acute care surgeon. Even routine operations such as laparoscopic appendectomy or cholecystectomy become challenging due to the cardiopulmonary physiologic changes and anatomic anomalies associated with Eisenmenger syndrome. The care of these patients can be further complicated by the severity of disease, surgical complexity, and the abnormal anatomy associated with the syndrome. CASE REPORT A 35-year-old patient with Eisenmenger syndrome and abdominal heterotaxy presented with acute cholecystitis. She underwent percutaneous cholecystostomy tube placement during her index hospitalization, which was complicated by atrial fibrillation and a cerebrovascular accident due to air embolism. Three months after presentation, she underwent an uncomplicated open cholecystectomy. She was discharged on post-operative day 5, and her course was notable only for a superficial surgical site infection requiring incision and drainage and antibiotics. CONCLUSIONS Our experience managing acute cholecystitis in a patient with Eisenmenger syndrome, abdominal heterotaxy with interrupted inferior vena cava, and bilateral superior vena cava, highlights critical aspects of care of such patients in the context of routine acute surgical care. Pre-operative planning should include optimization of cardiopulmonary function, an individualized anesthetic plan to maintain systemic vascular resistance, and bailout maneuvers in the event of cardiovascular collapse, such as planning for extracorporeal membrane oxygenation with axillary cannulation in the case of our patient.