Epidural Anesthesia for Cesarean Section in a Patient with Romano-Ward Syndrome

To the Editor: Patients with long QT syndromes, where recalcitrant and often fatal dysrythmias are precipitated by stress, present the anesthesiologist with a considerable challenge. There are a few reports describing the management of these patients under general anesthesia 1,2. We report the management of a patient with Romano-Ward syndrome who presented for emergency cesarean section, which was performed under epidural anesthesia. A 25-yr-old white woman at 36 wk of gestation with Romano-Ward syndrome presented for emergency cesarean section. She was first diagnosed as having prolonged QT interval syndrome in 1985. Her oldest brother died suddenly at the age of 15 yr. Two other brothers also suffer from the same syndrome. The patient has a known allergy to procainamide and quinidine. She had two previous uneventful general anesthetics for toenail surgery and dilation and curettage in 1980 and 1988, respectively. Her preoperative electrocardiogram (EKG) showed sinus bradycardia with occasional premature ventricular contraction and prolonged QT/QTc interval of 428/420 ms. The patient was receiving atenolol 150 mg twice daily and diltiazem 60 mg four times daily. Her physical examination and preoperative biochemistry and hematology were normal. After admission, the patient was monitored by continuous EKG and fetal monitoring. A decision was made to perform a cesarean section after failure to progress with vaginal delivery. The patient had her usual doses of atenalol and diltiazem at 0700 h. After antacid prophylaxis, she was brought to the operating room at 1030 h. Continuous EKG, noninvasive blood pressure, and pulse oximetry were monitored. Defibrillator pads were placed, and a cardiology team was present in the operating room throughout the procedure. After administration of 1 L of crystalloid, an epidural catheter was placed 3 cm into the epidural space through the L3-4 intervertebral space and a test dose of 2 mL of 2% lidocaine without epinephrine was given. A sensory block up to the T-6 segment was achieved with an additional 12 mL of 2% lidocaine given in 3-mL increments every 3 min. The patient was hemodynamically stable throughout the procedure. The postoperative course was uneventful. She was discharged home three days later still taking atenolol and diltiazem. Romano-Ward syndrome is a very rare autosomal dominant inherited condition with primary prolongation of the QT interval (QTc > 440 ms) and syncopal episodes associated with physical or emotional stress 3,4. In view of its high mortality, dysrythmias must be avoided 3. Regular medication should be continued. The best drugs for therapy of prolonged QT interval syndrome are beta-adrenergic blockers, which decrease mortality from 73% to 6% 4. Calcium channel blockers have also been advocated because of their antidysrhythmic action 5. Left stellate ganglion block may be considered to control acute cardiac dysrhythmias in the absence of a response to drug therapy 6. Regional anesthesia was chosen for our patient to avoid the stress of rapid-sequence induction and intubation. Subarachnoid block may have resulted in a precipitous reduction in blood pressure, which could have triggered a dysrythmia. Epidural anesthesia allows more hemodynamic stability. Epinephrine was not used in the test dose for fear of precipitating dysrhythmias. Regarding our choice of local anesthetic, bupivacaine was avoided because of its cardiotoxicity in the event of an inadvertent intravascular injection. Lidocaine was titrated to achieve a slow and controlled block to minimize the risk of hypotension. In summary, we report the successful management of a patient with Romano-Ward syndrome for cesarean section under epidural anesthesia. Raghuvender Ganta, MD Cathy Roberts, MD Robin J. Elwood, MD Venkata R. Maddineni, MD Department of Anesthesiology, University of Oklahoma College of Medicine, Oklahoma City, OK 73152