Urgent cesarean delivery with contingent ECPR preparation for a parturient with Eisenmenger syndrome and supra-systemic pulmonary hypertension: a case report

Parturients with pulmonary arterial hypertension (PAH) face an increased risk of morbidity and mortality surrounding the peripartum period. Patients with severe PAH and Eisenmenger syndrome have the highest risk for perioperative mortality and provide a significant perioperative challenge for anesthesiologists, necessitating a multidisciplinary approach throughout pregnancy. We present a case of an urgent cesarean section at 37 weeks of gestation in a patient with Eisenmenger syndrome, a large atrial septal defect, and supra-systemic pulmonary hypertension. Due to the patient’s severity of PAH, cardiothoracic surgery was present and placed femoral arterial and venous catheters in preparation for possible extracorporeal membrane oxygenation (ECMO) cannulation in the event of hemodynamic collapse. The procedure was performed under a carefully titrated epidural, thus avoiding general anesthesia and the dangers associated with inducing a patient with her comorbid conditions. An arterial line, internal jugular central line, and Swan-Ganz catheter were placed while the patient was awake to aid in monitoring. Additionally, transthoracic echocardiography (TTE) was used to assist in cardiac monitoring while the epidural level was gradually increased. She was maintained on vasopressin, epinephrine, and phenylephrine infusions in addition to inhaled nitric oxide. She tolerated the procedure well, and the fetus was delivered safely. Careful management across pre-epidural, post-epidural, intraoperative, and postoperative stages illustrates the dynamic adjustments and multidisciplinary communication required in this patient population. Advanced medical therapy optimization, careful neuraxial anesthesia titration, and early consideration of extracorporeal cardiopulmonary resuscitation (ECPR) together capture the critical elements of management in this complex setting.