Perioperative management of emergency non-cardiac surgery in a pediatric patient with Eisenmenger syndrome: a case report.

Background: Eisenmenger syndrome (ES) is a late-stage complication of untreated congenital heart disease. It is characterized by irreversible pulmonary arterial hypertension (PAH), right-to-left shunting, and cyanosis. Pediatrics with ES who undergo emergency non-cardiac surgery are at an extremely high perioperative risk. Potential complications include pulmonary hypertension crisis (PHC), right ventricular failure, and sudden cardiac death. There is limited evidence guiding perioperative management in this population. Case Description: An 11-year-old girl required emergency surgery under general anesthesia for right ovarian torsion. She had a history of congenital heart disease, which was complicated by severe pulmonary hypertension (pulmonary artery pressure: 105/49 mmHg) and ES. Anesthesia was inducted with etomidate, midazolam, vecuronium, and sufentanil, and maintained with propofol, remifentanil and sevoflurane. Intraoperatively, hemodynamic parameters remained stable. However, the patient developed a postoperative PHC, which was characterized by ventricular tachycardia, hypotension, and hypoxemia. Prompt treatment with amiodarone, treprostinil, sodium bicarbonate, and supplemental oxygen successfully restored sinus rhythm and hemodynamic stability. The patient was discharged on postoperative day 11. Conclusions: Emergency non-cardiac surgery poses an extremely high anesthetic risk for pediatrics with ES. It is critical to avoid factors that elevate pulmonary vascular resistance, ensure meticulous monitoring, and recognize and treat PHC early. This case underscores the importance of personalized anesthetic planning and multidisciplinary perioperative management for this vulnerable population.