Pregnant individuals with pulmonary arterial hypertension (PAH) have an increased risk of peripartum morbidity and mortality. Patients with severe PAH and Eisenmenger syndrome have the highest risk for perioperative mortality and warrant multidisciplinary care coordination throughout pregnancy. For most patients with congenital heart disease (CHD) with left-to-right shunt, PAH is a late stage finding and portends a poor prognosis. Pregnancy in patients with PAH is associated with high risk of maternal and neonatal mortality [1–4]. These patients are at an elevated risk of pulmonary hypertensive crisis, pulmonary embolism, and cerebrovascular accident [1]. For the anesthesiologist, whether the choice is made to deliver via vaginal delivery or cesarean section, the anesthetic plan must consider the increased risks and abnormal maternal physiology. Cesarean delivery (CD) requires achieving surgical anesthesia without significant decreases in systemic vascular resistance (SVR), avoiding increases in pulmonary vascular resistance (PVR), and preserving right heart function. For vaginal delivery, dense neuraxial analgesia and assisted second stage of labor avoid increases in PVR due to pain and decreased venous return caused by increased intrathoracic pressure while pushing.

Furthermore, patients with severe PAH and long-standing left-to-right shunt may develop Eisenmenger syndrome, wherein shunt flow reverses or becomes bidirectional due to the increase in PVR. Parturients with Eisenmenger syndrome are more likely to experience severe heart failure, pulmonary hypertensive crisis, or peripartum death [5]. We present the management of an urgent CD for a parturient with Eisenmenger syndrome, a large atrial septal defect (ASD), and supra-systemic pulmonary artery pressure who presented with acute decompensated right heart failure at 37 weeks gestation. The patient’s severe PAH placed her at high risk for right heart failure and pulmonary hypertensive crisis, with potential for hemodynamic collapse and impaired placental circulation. Accordingly, the cardiothoracic surgery team placed femoral arterial and venous catheters in preparation for possible extracorporeal cardiopulmonary resuscitation (ECPR). The procedure was performed under a carefully titrated epidural, thus avoiding general anesthesia and the associated risks.

The timeline of the case is summarized in Table 1.

A 31-year-old gravida 5 para 4 woman at 37 weeks gestation was admitted with worsening dyspnea, dizziness, and pre-syncopal episodes with exertion. Her cardiac history included a large ostium secundum ASD, Eisenmenger syndrome, severe pulmonary hypertension (WHO group-I, NYHA class 4), and chronic right ventricular failure (Figure 1). Her social history was significant for current tobacco smoking as well as former use of marijuana and methamphetamine. All previous obstetric deliveries occurred at other hospitals. Her first two deliveries were vaginal deliveries with no other outside hospital records. She was diagnosed with pulmonary hypertension during her fourth pregnancy three years prior, during which she developed progressive shortness of breath and hypoxemia and underwent an emergency cesarean section at an outside hospital. Prior to delivery, her transthoracic echocardiography (TTE) revealed a large secundum ASD and severe pulmonary hypertension with an estimated right ventricular systolic pressure (RVSP) of 94 mmHg. Systemic systolic blood pressures at that time were in the 90–110 mmHg range. Her postpartum therapy included selexipag, macitentan, and sildenafil. Cardiology planned for a repeat heart catheterization after three months of maintenance pulmonary hypertension therapy before possible closure of her ASD. However, she stopped taking her medications due to financial constraints. Prior workup did not identify any additional shunts, such as congenital malformations besides the ASD.

The patient was lost to follow up after her prior CD and was not taking any medications for pulmonary hypertension at the time of admission. She sought care at an outside hospital for shortness of breath and palpitations two weeks prior to admission; at that time her TTE estimated RVSP was 105 mmHg with systemic systolic pressures of 110 mmHg. On admission, her oxygen saturation was 90–95% on room air with desaturations to 84–85% on exertion.

A multidisciplinary team including maternal fetal medicine, anesthesiology, pulmonology, cardiology, cardiothoracic surgery, and intensivists convened to discuss her care. There was prevailing concern that vaginal delivery with repeated Valsalva could precipitate pulmonary hypertensive crisis, acute right ventricular failure, and hemodynamic collapse that could compromise maternal and fetal circulation. There was also concern for rapid decline and post-delivery cardiovascular complications in the event of uncontrollable bleeding during a vaginal delivery and potentially larger fluid shifts after delivery compared to a cesarean section. Alternatively, a planned cesarean section would provide controlled timing to ensure all necessary support staff were immediately available for escalation of care and reduce the risk of an emergent cesarean section. Overall, cesarean section created the most controlled environment for the multitude of potential complications. The consensus was that a repeat cesarean section combined with femoral sheath placement to facilitate ECPR was the safest option for delivery.

She was initially admitted to the labor and delivery unit. An epidural catheter was placed in anticipation of eventual CD. After a test dose of 3 mL of 1.5% lidocaine, the epidural was activated with an infusion of 0.15% ropivacaine and fentanyl 2.5 µg/mL at 2 mL/hour. She developed hypotension with systolic pressures in the low 90s. A vasopressin infusion was started at 0.04 units/min due to its ability to increase SVR with minimal effect on pulmonary vasculature. In addition, she required boluses of vasopressin and epinephrine to maintain her systolic blood pressure above 105 mmHg. The epidural infusion was discontinued, and she was transferred to the intensive care unit (ICU), where she continued to receive vasopressin at 0.04 units/min, inhaled nitric oxide by high flow nasal cannula (HFNC), and a Treprostinil intravenous infusion. TTE redemonstrated a large ~2.4 cm ostium secundum ASD with intermittent right-to-left shunting, RVSP 80 mmHg with systemic systolic pressure of 95 mmHg, a severely dilated and hypertrophied right ventricle, severely decreased right ventricular function, and preserved left ventricular function (Figure 2, Figure 3).

A radial arterial and central venous catheter was placed in the ICU, and she was transferred to the operating room. She arrived still on vasopressin at 0.04 units/min and inhaled nitric oxide. Her initial vitals were: systemic blood pressure 125/67 mmHg, heart rate 85 bpm, and oxygen saturation 99% with HFNC (80% FiO₂ at 18 L/min). A pulmonary artery catheter was placed in the operating room via the existing 9 French central venous catheter using sterile technique. TTE was used to guide the catheter placement in the setting of an ASD. The initial pulmonary artery pressure was 101/28 mmHg and reached a maximum of 114/55 mmHg during the surgery. Given concern for further hypotension with epidural activation, vasopressin infusion was maximized to 0.06 units/min, and a phenylephrine infusion was started at 50 mcg/min. Phenylephrine was chosen to maintain SVR, thus supporting biventricular perfusion at the expense of increasing PVR. Her epidural was incrementally loaded with 2% lidocaine with epinephrine in 3 mL aliquots until a T4 sensory level was obtained. She developed persistent hypotension with epidural loading, and low dose epinephrine infusion was initiated at 3 mcg/min to support right heart function and to provide mild augmentation of SVR. She was positioned supine, and catheters were placed under epidural analgesia in the common femoral artery and common femoral vein to facilitate ECPR. Her baby was delivered by cesarean section with APGAR scores of 7 and 7 at 1 and 5 minutes, respectively. Her baby weighed 2.5 kg and was initially admitted to neonatal ICU (NICU) for continued need of continuous positive airway pressure (CPAP). Her baby was discharged home on day of life 9. Her pulmonary arterial systolic pressures were supra-systemic throughout much of the procedure with mean systemic pressures maintained between 65 and 70 mmHg. Although her estimated blood loss was 1,000 mL, she did not acutely decompensate during delivery, and ECPR was not initiated. She was transferred to the ICU postoperatively on pressor support including vasopressin at 0.06 units/min, phenylephrine at 50 mcg/min, and epinephrine at 3 mcg/min.

She remained on inhaled nitric oxide, a vasopressin infusion, and a treprostinil infusion at 8 ng/kg/min on postoperative day one. She was weaned off vasoactive support by postoperative day two. Treprostinil was continued, and she was started on goal directed therapy with tadalafil, furosemide, and spironolactone. She had persistent hypoxemia, and she was discharged on postoperative day 11 with supplemental oxygen (2–4 L/min via nasal cannula). She was readmitted six weeks after discharge with a pulmonary hypertensive crisis, ventricular fibrillation arrest, and acute hypoxic respiratory failure. Her urine drug screen resulted positive for amphetamine and cannabinoids. She unfortunately passed away 9 months later due to an out of hospital cardiac arrest.

TTE findings are summarized in Table 2. Images of the TTE findings are shown in Figure 1 and Figure 2.

For most patients with CHD with left-to-right shunt, PAH is a late stage finding and portends a poor prognosis. Pregnancy in patients with PAH is associated with high risk of maternal and neonatal mortality. These patients are at an elevated risk of pulmonary hypertensive crisis, pulmonary embolism, and cerebrovascular accident.

Pregnancy in individuals with Eisenmenger syndrome and PAH is associated with peripartum mortality and poor fetal outcomes. Individuals with Eisenmenger syndrome and pulmonary hypertension should be counseled about the risk of pregnancy. Care of the pregnant individual with CHD warrants multidisciplinary coordination between obstetricians, obstetric and cardiac anesthesiologists, cardiology, cardiothoracic surgery, and neonatologists. ECPR has emerged as an important approach to support parturients with significant cardiac disease through delivery.

This case represents a unique comprehensive approach to the management of pregnant patients with severe pulmonary hypertension and Eisenmenger syndrome with careful medical management and ECPR standby. The team balanced the extremely high surgical and anesthetic risk of her suprasystemic pulmonary pressures, intermittent right-to-left shunting, and absence of chronic pulmonary vasodilator therapy against the risks of ECPR itself, including bleeding, anticoagulation, and circuit related complications. 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 ECPR together capture the critical elements of management in this complex setting.