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Eisenmenger Syndrome: A Recent Success
Abstract Number: F-53
Abstract Type: Case Report/Case Series
Pregnancy mortality in patients with Eisenmenger syndrome (ES) is reported to be 30-50%.1 Patients with ES are cautioned to avoid pregnancy, and termination is often suggested should the patient become pregnant.1 Regardless of the risks that pregnancy carries, Eisenmenger patients may sometimes choose to continue a pregnancy, posing great challenge to the anesthesiologist.
A 33 year-old G1P0 with an unrepaired ASD with pulmonary pressures equal to systemic pressures resulting in ES and severely decreased RV function presented at 26 weeks gestation for management of the remainder of the pregnancy. Pulmonary hypertension (pHTN) was managed with O2, sildenafil PO, epoprostenol IV, and iloprost nebulizer.
At 33 weeks gestation, the patient developed progressively worsening hypoxia and thromboyctopenia (platelets 94K decreased to 58K) due to a pHTN crisis. Plans were made for urgent CD. PTT/PT/INR were normal. Rotational thromboelaastometry (ROTEM) analysis was performed to confirm that despite the thrombocytopenia, clotting ability was only moderately inhibited. Inhaled NO was initiated via high-flow nasal oxygen. A central line was placed. She was taken to a CT OR. Dobutamine was initiated (5 mcg/kg/min). Vasopressin and phenylephrine were titrated to maintain SBP>110 (monitored via arterial line) which resulted in oxygen saturation >97%. We performed a CSE (spinal: bupivacaine 2mg, fentanyl 10mcg). Epidural was dosed slowly to a T6 level, while catheters were placed to facilitate femoral VA ECMO access should it be needed. Uneventful CD was performed (1465g infant - Apgars 8,9). Epidural catheter was maintained for postoperative pain control.
On POD3 platelets reached a nadir of 33K. Due to concern for continued pHTN crisis, the critical care team desired to initiate systemic anticoagulation. ROTEM analysis was performed with results similar to the day of delivery. The epidural catheter was removed. Therapeutic anticoagulation was initiated 6 hours later. Pulmonary vasodilators, inotropy and vasopressors were continued through the first postoperative week.
Outcomes of parturients with ES are poor. It is not known whether vaginal delivery or CD is safer, nor is the optimal anesthetic for CD known1. This patient required CD for the indication of pHTN crisis, remote from delivery. Despite thrombocytopenia, neuraxial anesthesia was utilized as it was felt that the hemodynamic changes associated with induction of GETA could result in cardiopulmonary collapse. With the initiation of inotropy and epidural blockade (decrease in SVR), the patient’s oxygen saturation improved, perhaps indicating a need for decreased preload. ES carries a risk of thrombus formation thus systemic anticoagulation was crucial during the pHTN crisis2. ROTEM analysis can guide clinicians at the extreme limits of safe neuraxial manipulation.
1. Eur Heart J. 2000 Jan;21(2):104-15.
2. J Am Coll Cardiol. 2007 Aug 14;50(7):634-42.