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///2018 Abstract Details
2018 Abstract Details2019-08-02T15:57:01-05:00

Anesthetic Management of a Patient with Romano-Ward Syndrome

Abstract Number: T4C-9
Abstract Type: Case Report/Case Series

Amy Willett MD1 ; Paula Trigo-Blanco MD2; Alian Aymen MD3; Antonio Gonzalez MD4

Romano-Ward syndrome (RWS) is the major variant of long QT syndrome, inherited in an autosomal dominant pattern. It is characterized by a prolonged QT interval on the electrocardiogram (ECG), syncope, and potentially sudden death secondary to tachyarrhythmias.

31-year-old white woman Gravida 1 Para 0, who presented to the hospital after spontaneous rupture of membranes at 38 weeks gestational age, requesting an epidural for control of her labor pain. She had history of obesity, asthma, RWS with long Q-T syndrome (LQTS) and seizure episodes, previously with AICD in place, but removed due to deep vein thrombosis (DVT) of her right upper extremity (RUE). Her home medications included atenolol to control her QT prolongation, and subcutaneous heparin. Her preoperative electrocardiogram (EKG) showed sinus rhythm with a prolonged QT/QTc interval of 459/447 ms.

Anesthesia was consulted for epidural placement for labor pain. After administration of 1 liter of crystalloid, an epidural catheter was at L3-4 intervertebral space, a test dose of 3 mL of 2% lidocaine without epinephrine was given. A sensory block up to the T10 segment was achieved with an additional 6 mL of 0.25% bupivacaine given in 2-mL increments every 3 min. The patient was hemodynamically stable throughout the procedure. Approximately 10 hours after the epidural was placed, fetal heart rate late decelerations were noted, so decision was made to proceed with cesarean delivery (CD).

In the operating room continuous EKG, noninvasive blood pressure, pulse oximetry were monitored, and external defibrillator pads placed. Epidural was extended for CD with lidocaine 2% 15 mL plus of 8.4% Sodium Bicarbonate -2 mL. The patient was placed supine with left uterine displacement. T4 level was confirmed, surgery commenced. After delivery, intravenous oxytocin 10 units in Lactated Ringers 500 mL was administered, concurrently with methergine 0.2mg IM and 1000 mcg rectal misoprostol for uterine atony. The patient was transferred to postanesthesia care unit (PACU) for continuous telemetry monitoring. Patient's postoperative course was uneventful, and she was discharged home on postoperative day 4.

Patients with long QT syndrome present a challenge for the anesthesiologist, and special precautions should be taking monitoring these patients and minimizing the use of drugs that might prolong the QT and cause fatal arrhythmias. Neuraxial anesthesia via epidural catheter placement can be utilized with great success in this patient population.

1. Anesth Analg. 1995

2.J Anesth. 2005

SOAP 2018