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Obstetric Anesthesia Management in a Patient with Holt-Oram Syndrome
Abstract Number: 74
Abstract Type: Case Report/Case Series
Holt-Oram Syndrome (HOS) is a rare genetic condition characterized by upper extremity malformations, congenital heart malformations, and/or cardiac conduction disease (1). This is the first case report that describes the anesthetic management of a parturient with HOS who underwent both a cesarean section and a vaginal birth after cesarean delivery (VBAC). A 20-year-old primigravida with history of HOS presented at 37 weeks of gestation with oligohydramnios. Her diagnosis was based on a left upper extremity abnormality, corrected atrial and ventricular septal defects, atrial arrhythmias, and sinus node dysfunction. Cesarean delivery was planned secondary to a breech presentation. Recent cardiac work-up demonstrated that she had a 1st degree atrioventricular block, normal left ventricle size, left ventricle ejection fracture of 44%, and mild right atrial enlargement. In the operating room, a right radial arterial line was placed in addition to standard monitors. An epidural catheter was placed in the left lateral position at the L2/L3 interspace using an 18-gauge Tuohy needle and loss of resistance technique. Two 5 ml boluses of 2% Lidocaine were given via the catheter during the 1 hour cesarean section to establish adequate surgical analgesia. Epidural Duramorph in a 3 mg dose was given for postoperative analgesia. She had no intra-operative hypotension or cardiac dysrhythmias. She was monitored in the Surgical Intensive Care Unit (SICU) for one day. On postoperative day 2, premature atrial contractions were noted on her telemetry and an electrocardiogram demonstrated a junctional rhythm, for which no treatment was recommended.
Two years later, she returned to deliver at 38 weeks gestation and desired a VBAC. She was normotensive and in sinus bradycardia. In the SICU, a right radial arterial line was placed in addition to standard monitors. In the sitting position, an epidural catheter was placed and dosed to provide labor analgesia. A continuous infusion was then run at 12 ml/hr of Fentanyl 4mcg/Bupivacaine 0.0625% throughout the delivery, which lasted approximately 10 hours. She had excellent analgesia throughout. She was monitored for one day in the SICU and discharged home.
HOS is a disease with variable expression with regards to both limb deformities and cardiac disease (2). As a result, anesthetic management should be individualized. This case demonstrates the successful use of epidural anesthesia for both a cesarean section and a VBAC in a patient with HOS. Invasive blood pressure monitoring and intensive care unit delivery ensure safe management of such a patient with moderate left ventricular function and conduction abnormalities.
1 McDermott, DA et al. (2005) Pediatr Res 58: 981-6.
2 Basson, CT et al. (1994) N Engl J 330:885-91.