- Email: [email protected]
Caesarean section for twin pregnancy in a parturient with Brugada syndrome
J. Bramall et al.
181
Caesarean section for twin pregnancy in a parturient with Brugada syndrome J. Bramall, A. Combeer, J. Springett, R. Wendler Department of Anaesthesia, St. George’s Hospital, London, UK ABSTRACT Brugada syndrome is a common cause of sudden cardiac death. We report the anaesthetic management of a woman with Brugada syndrome for elective caesarean section of twins. There are few reports of this disease in pregnancy and we believe this is the first of elective caesarean section in a parturient with the syndrome. The characteristic electrocardiographic changes of the syndrome are linked to sodium channel blockers including local anaesthetics such as bupivacaine. The use of bupivacaine for intrathecal central neuraxial blockade as well as other drugs commonly used in obstetric anaesthesia is discussed. c 2010 Elsevier Ltd. All rights reserved.
Keywords: Caesarean section; Spinal anaesthesia; Brugada syndrome
Introduction Brugada syndrome is an inherited cardiac disease causing ventricular tachyarrythmias. It was first described in 19921 and it is recognised as a common cause of sudden cardiac death. Diagnosis is made on the basis of distinctive electrocardiogram (ECG) changes: right bundle branch block and characteristic ST elevation in leads V1–3 in a structurally normal heart. Some patients have a normal ECG at rest and changes are only apparent on provocation with drugs, in particular sodium channel blockers.2 We report a case of elective caesarean section for twins in a woman with Brugada syndrome.
Case report A 40-year-old nulliparous woman with Brugada syndrome and dichorionic diamniotic twins conceived by in vitro fertilization, presented for elective caesarean delivery. Her previous medical history included a molar pregnancy. She had been diagnosed with Brugada syndrome one year previously after her sister died in her sleep aged 42 years of age. She and her close family were subsequently investigated. A computerised tomography (CT) scan of her head and resting ECG were normal, but an exercise ECG revealed some subtle changes that raised the possibility of Brugada syndrome. An ajmaline provocation test showed typical covert ST segment elevation and incomplete right bundle branch block. There were no abnormalities on echocardiography. Although asymptomatic, she had a history of syncopal episodes with loss of consciousness some years previously. These were thought at the time to be of vasovagal origin but in Accepted October 2010 Correspondence to: J. Bramall, Department of Anaesthesia, St George’s Hospital, London SW17 0QT, UK. E-mail address: [email protected]
retrospect may have been related to Brugada syndrome. After extensive discussion an implantable cardiac defibrillator (ICD) had been inserted in the left pre-pectoral pocket. Insertion was uncomplicated and the device had been tested. Her past medical history was otherwise unremarkable and she had received general anaesthesia complicated only by postoperative nausea and vomiting. She was on no regular medication and had no known drug allergies. The patient was seen together with obstetricians and cardiologists in our High-Risk Obstetric Anaesthesia Clinic. After reviewing the available literature on Brugada syndrome, she was booked for elective caesarean section under spinal anaesthesia at 37 weeks of gestation The cardiology team advised placing a magnet over the ICD during the procedure to allow the use of diathermy and provided a list of medications that might provoke ECG changes or arrhythmia. The list included sodium channel blocking drugs (including bupivacaine), with advice to avoid systemic absorption and to monitor the ECG; proprofol, alpha-adrenergic agonists, beta blockers, calcium channel blockers, and ergot alkaloids. Premedication with oral ranitidine and metoclopramide was given. After securing intravenous access with a 16-gauge cannula, an infusion of Hartmann’s solution was commenced. Non-invasive blood pressure, ECG and oxygen saturation monitoring was established. A 25-gauge pencil-point needle was inserted in the L3–4 interspace with the patient in the sitting position using a strict aseptic technique. Following free flow of clear cerebrospinal fluid, 0.5% hyperbaric bupivacaine 13.5 mg and diamorphine 400 lg were injected, after which she was positioned supine with left-lateral tilt. A bilateral block to cold to T4 was achieved; light touch sensation was above T10 and there was a good motor block. She received boluses of phenylephrine 50 lg to
182 maintain blood pressure within normal limits; a total of 600 lg was given during the procedure. External defibrillator paddles were placed on her chest and her ICD was deactivated with a ring magnet. Routine ECG monitoring throughout the procedure showed no changes or arrhythmias. Diathermy was not used during the procedure, but could have been used since the defibrillator was deactivated. Healthy male and female infants were delivered uneventfully. After delivery of the second twin a 5-IU slow intravenous bolus of oxytocin was given. There was a brief period of uterine atony which was treated with a 40-IU oxytocin infusion over 4 h. Estimated blood loss was higher than average at 1500 mL. Routine antibiotic prophylaxis of 1.2 g co-amoxiclav was administered after delivery. The magnet was removed before transfer to the recovery ward. She was given rectal diclofenac and prescribed regular paracetamol and diclofenac for the postoperative period. Low-molecular-weight heparin was given 4 h after the spinal injection. She was admitted to the obstetric high-dependency unit for 12 h postpartum. The postoperative period was otherwise unremarkable. An ECG performed on the first post-operative day was unchanged, and she was returned to the care of the cardiology team for further follow-up.
Discussion Brugada syndrome is inherited in an autosomal dominant pattern with incomplete penetrance. Arrhythmias have been observed at any age but commonly occur in the fourth decade. The incidence is estimated at 5 per 10 000. It is endemic in South East Asia where it has a male predominance (male–female ratio 8:1),2 and is thought to be caused by changes in the alpha subunit of the sodium channel leading to an altered pattern of depolarisation. Mutations have been identified in the SCN5A gene that encodes for the alpha subunit of the human cardiac sodium channel.3 Three types of ECG pattern are described in Brugada syndrome (Fig. 1). Type I is characterised by prominent coved ST elevation followed by a negative T wave. Type II also has ST elevation followed by a positive or biphasic T wave that results in a ‘saddle back’ appearance. Type III is a right precordial ST elevation of coved or saddleback type, or both.2 Ajmaline is a class I anti-arrhythmic drug used to demonstrate the characteristic ECG changes of Brugada syndrome. It is a potent sodium channel blocker with a short half-life, and has been used for the treatment for Wolff-Parkinson-White syndrome and monomorphic ventricular tachycardia. There are problems with the availability of ajmaline due to its limited applications.5 Individuals with Brugada syndrome at high risk of ventricular arrhythmias are treated with an ICD, which is the only therapy known to be effective. Drug treatment
Brugada syndrome and caesarean delivery
Type I
Type II
Type III
V1
V1
V1
V2
V2
V2
V3
V3
V3
Fig. 1 ECG pattern in Brugada syndrome. Type I ST segment elevation is either spontaneously present or induced with a Ajmaline/Flecainide test which is considered diagnostic.2 Type II and III may lead to suspicion but drug challenge is required for diagnosis.4
with beta-blockers and amiodarone does not protect against arrhythmias and, although quinidine has some effect at normalising ST segment changes and may have a role as adjunctive therapy, it has no effect on reducing sudden cardiac death. Low-dose isoprenaline has been used in the treatment of arrhythmic storms.2,6 Several case reports document general anaesthesia in Brugada syndrome,7–13 although this is the first description of an elective caesarean section in a patient with the syndrome. Caesarean section in patients with other causes of polymorphic ventricular tachycardia such as polymorphic catecholamine-sensitive tachycardia14 and Long QT syndrome has been described.15–17 These are distinct from Brugada syndrome, and their management differs in that it is important to avoid increases in sympathetic tone and catecholamine surges which can precipitate tachyarrhythmias; both are managed with beta blockers. Three reports describe the use of regional anaesthesia14,16,17 during which efforts were made to avoid sudden loud noises that might precipitate arrhythmias. One describes the use of general anaesthesia with a remifentanil infusion.15 In contrast, arrhythmias in Brugada syndrome are thought to be caused by excess parasympathetic tone, which explains why most events occur at rest or at night.6 A recent case report documented persistent ventricular fibrillation in a pregnant woman with Brugada syndrome.18 She had stopped taking amiodarone and verapamil during pregnancy and had been admitted with progressively more frequent episodes of ventricular
J. Bramall et al. fibrillation which triggered her ICD. She became hypoxaemic requiring tracheal intubation and mechanical ventilation. An emergency caesarean section was performed and she commenced extra corporeal membrane oxygenation (ECMO) as a temporising measure until drug levels could be re-established. Amiodarone and verapamil were restarted and over the following three days the frequency of ventricular fibrillation decreased and she was weaned from ECMO support. Mother and baby were discharged uneventfully. Peripartum febrile illness such as mastitis has also been reported to provoke ECG changes typical of Brugada syndrome.19 Type I ECG changes disappeared when the fever resolved. Our main concern was the safety of bupivacaine in Brugada syndrome. Sodium channel blockade is used to provoke the characteristic ECG changes of the syndrome and there was concern that bupivacaine might cause the same changes if plasma levels were sufficient. There are case reports of epidural or paravertebral bupivacaine inducing Brugada-like ECG changes with some leading to life-threatening arrhythmias.7,8,20 We felt that plasma levels of bupivacaine would determine the effects on the heart and that the total dose of bupivacaine in a spinal anaesthetic is considerably less than that for an epidural. A study in rats showed higher plasma concentrations of lidocaine and bupivacaine after epidural compared with intrathecal administration.21 We were unable to find any reports of intrathecal bupivacaine provoking ECG changes in patients with Brugada syndrome. Alpha-adrenergic agonists were also of concern. Phenylephrine has been used, albeit unsuccessfully, in an attempt to provoke Brugada syndrome by stimulating the baroreflex causing vagal stimulation,22 but it is also the vasopressor of choice at our institution for obstetric anaesthesia. Miyazaki et al.23 noted that ‘‘selective alpha-adrenoceptor stimulation by intravenous norepinephrine in the presence of propranolol or by intravenous methoxamine consistently augmented, whereas alpha-adrenoceptor blockade reduced, ST segment elevation in three patients’’. However, in these cases alpha agonism was not performed during neuraxial blockade, when systemic vascular resistance is reduced. There is an evidence-based internet resource that provides a list of drugs which should be avoided in Brugada syndrome.24 Included is ergovrine (ergometrine), which has been used to stimulate coronary spasm in patients with Brugada syndrome leading to ventricular fibrillation.25 Its use as a uterotonic may be precluded in this condition. We were unable to find any published data on the safety of oxytocin or other uterotonics in Brugada syndrome. Oxytocin is the routine uterotonic agent at our institution, and was used without adverse effect. Routine non-invasive monitoring was used during surgery and anaesthesia. The use of invasive arterial blood pressure monitoring for this case was considered
183 but was not used as she had been completely asymptomatic and her ICD had never fired. In conclusion we performed a caesarean section for a parturient with Brugada syndrome with a twin pregnancy using intrathecal bupivacaine for central neuraxial blockade and the cautious use of phenylephrine as a vasoconstrictor.
References 1. Brugada P, Brugada J. Right bundle branch block, persistent ST segment elevation and sudden cardiac death: a distinct clinical and electrocardiographic syndrome. A multicenter report. J Am Coll Cardiol 1992;20:1391–6. 2. Antzelevitch C, Brugada P, Borggrefe M et al. Brugada syndrome: report of the second consensus conference. Endorsed by the Heart Rhythm Society and the European Heart Rhythm Association. Circulation 2005;111:659–70. 3. Antzelevitch C, Brugada P, Brugada J et al. Brugada syndrome: a decade of progress. Circ Res 2002;91:1114. 4. Napolitano C, Priori S. Brugada syndrome. Orphanet J Rare Dis 2006;1:35. 5. Brugada J, Brugada P, Brugada R. The ajmaline challenge in Brugada syndrome: a useful tool or misleading information? Eur Heart J 2003;24:1085–6. 6. Rossenbacker T, Priori S. The Brugada syndrome. Curr Opin Cardiol 2007;22:163–70. 7. Phillips N, Priestley M, Denniss A, Uther J. Brugada-type electrocardiographic pattern induced by epidural bupivacaine. Anesth Analg 2003;97:264–7. 8. Fujiwara Y, Shibata Y, Kurokawa S, Satou Y, Komatsu T. Ventricular tachycardia in a patient with Brugada syndrome during general anesthesia combined with thoracic paravertebral block. Anesth Analg 2006;102:1590–1. 9. Edge C, Blackman D, Gupta K, Sainsbury M. General anaesthesia in a patient with Brugada syndrome. Br J Anaesth 2002;89:788–91. 10. Hayashida H, Miyauchi Y. Anaesthetic management in patients with high-risk Brugada syndrome. Br J Anaesth 2006;97:118–9. 11. Vaccarella A, Vitale P, Presti C. General anaesthesia in a patient affected by Brugada syndrome. Minerva Anestesiol 2008;74:149–52. 12. Santambrogio L, Mencherini S, Fuardo M, Caramella F, Braschi A. The surgical patient with Brugada syndrome: a four-case clinical experience. Anesth Analg 2005;100:1263–6. 13. Inamura M, Okamoto H, Kuroiwa M, Hoka S. General anesthesia for patients with Brugada syndrome. A report of six cases. Can J Anesth 2005;52:409–12. 14. Chan T, Dob D. The anaesthetic management of a parturient with polymorphic catecholamine-sensitive ventricular tachycardia. Int J Obstet Anesth 2002;11:122–4. 15. Johnston A, Hall J, Levy D. Anaesthesia with remifentanil and rocuronium for caesarean section in a patient with long-QT syndrome and an automatic implantable cardioverter-defibrillator. Int J Obstet Anesth 2000;9:133–6. 16. Behl S, Wauchob T. Long QT syndrome: anaesthetic management at delivery. Int J Obstet Anesth 2005;14:347–50. 17. Hodgkinson P, McAtamney D. Combined spinal-epidural anaesthesia for caesarean section in a patient with long QT syndrome. Int J Obstet Anesth 2008;17:78–80. 18. Pagel P, Lilly R, Nicolosi A. Use of ECMO to temporize circulatory instability during severe Brugada electrical storm. Ann Thorac Surg 2010;88:982–3. 19. Ambardekar A, Lewkowiez L, Krantz M. Mastitis unmasks Brugada syndrome. Int J Cardiol 2009;132:e94–6. 20. Vernooy K, Sicouri S, Dumaine R et al. Genetic and biophysical basis for bupivacaine-induced ST segment elevation and VT/VF.
184
Cesarean delivery with Fontan circulation and psuedocholinesterase deficiency
Anesthesia unmasked Brugada syndrome. Heart Rhythm 2006;3:1074–8. 21. Berman M, Lipka L. Relative sodium current block by bupivacaine and lidocaine in neonatal rat myocytes. Anesth Analg 1994;79:350–6. 22. Probst V, Mabo P, Sacher F et al. Effect of baroreflex stimulation using phenylephrine injection on ST segment elevation and ventricular arrhythmia-inducibility in Brugada syndrome patients. Europace 2009;11:382–4. 23. Miyazaki T, Mitamura H, Miyoshi S et al. Autonomic and antiarrhythmic drug modulation of ST segment elevation in
patients with Brugada syndrome. J Am Coll Cardiol 1996;27: 1061–70. 24. Postema P, Wolpert C, Amin A et al. Drugs and Brugada syndrome patients: Review of the literature, recommendations, and an up-to-date website (www.brugadadrugs.org). Heart Rhythm 2009;6:1335–41. 25. Noda T, Shimizu W, Taguchi A et al. ST-segment elevation and ventricular fibrillation without coronary spasm by intracoronary injection of acetylcholine and/or ergonovine maleate in patients with Brugada syndrome. J Am Coll Cardiol 2002;40: 1841–7.
0959-289X/$ - see front matter c 2010 Elsevier Ltd. All rights reserved. doi:10.1016/j.ijoa.2010.10.006
Urgent cesarean delivery and prolonged ventilatory support in a parturient with Fontan circulation and undiagnosed pseudocholinesterase deficiency K.J. Grim,a K.W. Arendt,a A.K. Jacob,a C.H. Rose,b M.T. Keegana a b
Department of Anesthesiology, Mayo Clinic, Rochester, MN, USA Department of Obstetrics and Gynecology, Mayo Clinic, Rochester MN, USA
ABSTRACT A parturient with Fontan circulation required general anesthesia for urgent cesarean delivery and subsequent prolonged postoperative ventilation for newly-diagnosed pseudocholinesterase deficiency. Anesthetic management necessitated a thorough understanding of the hemodynamic principles of the Fontan circulation and physiologic adaptations during surgical delivery and recovery in the intensive care unit. c 2010 Elsevier Ltd. All rights reserved.
Keywords: Fontan: circulation, procedure; Pseudocholinesterase deficiency; Pregnancy; Positive pressure mechanical ventilation; Negative pressure mechanical ventilation; Cesarean delivery; Tricuspid atresia
Introduction Parturients with congenital heart disease pose significant anesthetic challenges in the peripartum period. Pseudocholinesterase deficiency, which occurs in 1:3200 parturients, also complicates anesthetic management, especially if the condition is undiagnosed before induction of general anesthesia. This combination of conditions in a parturient presents unique challenges to the anesthesiologist and critical care physician in both ventilatory management and hemodynamic control. We describe a parturient with tricuspid atresia and Fontan circulation who underwent general anesthesia for urgent cesarean delivery and required prolonged postoperative Accepted November 2010 Correspondence to: Katherine Arendt, Department of Anesthesiology, Mayo Clinic College of Medicine, 200 First Street SW, Rochester, MN 55905, USA. E-mail address: [email protected]
mechanical ventilation for undiagnosed pseudocholinesterase deficiency. This case has been published by our obstetric colleagues,1 but in this report we discuss the implications of anesthesia and mechanical ventilation.
Case report A 26-year-old 63 kg G3P0 parturient at 33 + 6 weeks of gestation presented for urgent cesarean delivery. Her cardiac history was significant for congenital tricuspid atresia with hypoplastic right heart, for which she underwent surgical palliation with Fontan circulation at age seven years. Her functional cardiac status before pregnancy was New York Heart Association (NYHA) class I, and she was able to participate in moderate regular exercise. Her past medical history was otherwise significant for scoliosis and an elective blepharoplasty, at an outside institution, for which she was unable to recall the type of anesthesia. She denied any family history of anesthetic complications.
181
Caesarean section for twin pregnancy in a parturient with Brugada syndrome J. Bramall, A. Combeer, J. Springett, R. Wendler Department of Anaesthesia, St. George’s Hospital, London, UK ABSTRACT Brugada syndrome is a common cause of sudden cardiac death. We report the anaesthetic management of a woman with Brugada syndrome for elective caesarean section of twins. There are few reports of this disease in pregnancy and we believe this is the first of elective caesarean section in a parturient with the syndrome. The characteristic electrocardiographic changes of the syndrome are linked to sodium channel blockers including local anaesthetics such as bupivacaine. The use of bupivacaine for intrathecal central neuraxial blockade as well as other drugs commonly used in obstetric anaesthesia is discussed. c 2010 Elsevier Ltd. All rights reserved.
Keywords: Caesarean section; Spinal anaesthesia; Brugada syndrome
Introduction Brugada syndrome is an inherited cardiac disease causing ventricular tachyarrythmias. It was first described in 19921 and it is recognised as a common cause of sudden cardiac death. Diagnosis is made on the basis of distinctive electrocardiogram (ECG) changes: right bundle branch block and characteristic ST elevation in leads V1–3 in a structurally normal heart. Some patients have a normal ECG at rest and changes are only apparent on provocation with drugs, in particular sodium channel blockers.2 We report a case of elective caesarean section for twins in a woman with Brugada syndrome.
Case report A 40-year-old nulliparous woman with Brugada syndrome and dichorionic diamniotic twins conceived by in vitro fertilization, presented for elective caesarean delivery. Her previous medical history included a molar pregnancy. She had been diagnosed with Brugada syndrome one year previously after her sister died in her sleep aged 42 years of age. She and her close family were subsequently investigated. A computerised tomography (CT) scan of her head and resting ECG were normal, but an exercise ECG revealed some subtle changes that raised the possibility of Brugada syndrome. An ajmaline provocation test showed typical covert ST segment elevation and incomplete right bundle branch block. There were no abnormalities on echocardiography. Although asymptomatic, she had a history of syncopal episodes with loss of consciousness some years previously. These were thought at the time to be of vasovagal origin but in Accepted October 2010 Correspondence to: J. Bramall, Department of Anaesthesia, St George’s Hospital, London SW17 0QT, UK. E-mail address: [email protected]
retrospect may have been related to Brugada syndrome. After extensive discussion an implantable cardiac defibrillator (ICD) had been inserted in the left pre-pectoral pocket. Insertion was uncomplicated and the device had been tested. Her past medical history was otherwise unremarkable and she had received general anaesthesia complicated only by postoperative nausea and vomiting. She was on no regular medication and had no known drug allergies. The patient was seen together with obstetricians and cardiologists in our High-Risk Obstetric Anaesthesia Clinic. After reviewing the available literature on Brugada syndrome, she was booked for elective caesarean section under spinal anaesthesia at 37 weeks of gestation The cardiology team advised placing a magnet over the ICD during the procedure to allow the use of diathermy and provided a list of medications that might provoke ECG changes or arrhythmia. The list included sodium channel blocking drugs (including bupivacaine), with advice to avoid systemic absorption and to monitor the ECG; proprofol, alpha-adrenergic agonists, beta blockers, calcium channel blockers, and ergot alkaloids. Premedication with oral ranitidine and metoclopramide was given. After securing intravenous access with a 16-gauge cannula, an infusion of Hartmann’s solution was commenced. Non-invasive blood pressure, ECG and oxygen saturation monitoring was established. A 25-gauge pencil-point needle was inserted in the L3–4 interspace with the patient in the sitting position using a strict aseptic technique. Following free flow of clear cerebrospinal fluid, 0.5% hyperbaric bupivacaine 13.5 mg and diamorphine 400 lg were injected, after which she was positioned supine with left-lateral tilt. A bilateral block to cold to T4 was achieved; light touch sensation was above T10 and there was a good motor block. She received boluses of phenylephrine 50 lg to
182 maintain blood pressure within normal limits; a total of 600 lg was given during the procedure. External defibrillator paddles were placed on her chest and her ICD was deactivated with a ring magnet. Routine ECG monitoring throughout the procedure showed no changes or arrhythmias. Diathermy was not used during the procedure, but could have been used since the defibrillator was deactivated. Healthy male and female infants were delivered uneventfully. After delivery of the second twin a 5-IU slow intravenous bolus of oxytocin was given. There was a brief period of uterine atony which was treated with a 40-IU oxytocin infusion over 4 h. Estimated blood loss was higher than average at 1500 mL. Routine antibiotic prophylaxis of 1.2 g co-amoxiclav was administered after delivery. The magnet was removed before transfer to the recovery ward. She was given rectal diclofenac and prescribed regular paracetamol and diclofenac for the postoperative period. Low-molecular-weight heparin was given 4 h after the spinal injection. She was admitted to the obstetric high-dependency unit for 12 h postpartum. The postoperative period was otherwise unremarkable. An ECG performed on the first post-operative day was unchanged, and she was returned to the care of the cardiology team for further follow-up.
Discussion Brugada syndrome is inherited in an autosomal dominant pattern with incomplete penetrance. Arrhythmias have been observed at any age but commonly occur in the fourth decade. The incidence is estimated at 5 per 10 000. It is endemic in South East Asia where it has a male predominance (male–female ratio 8:1),2 and is thought to be caused by changes in the alpha subunit of the sodium channel leading to an altered pattern of depolarisation. Mutations have been identified in the SCN5A gene that encodes for the alpha subunit of the human cardiac sodium channel.3 Three types of ECG pattern are described in Brugada syndrome (Fig. 1). Type I is characterised by prominent coved ST elevation followed by a negative T wave. Type II also has ST elevation followed by a positive or biphasic T wave that results in a ‘saddle back’ appearance. Type III is a right precordial ST elevation of coved or saddleback type, or both.2 Ajmaline is a class I anti-arrhythmic drug used to demonstrate the characteristic ECG changes of Brugada syndrome. It is a potent sodium channel blocker with a short half-life, and has been used for the treatment for Wolff-Parkinson-White syndrome and monomorphic ventricular tachycardia. There are problems with the availability of ajmaline due to its limited applications.5 Individuals with Brugada syndrome at high risk of ventricular arrhythmias are treated with an ICD, which is the only therapy known to be effective. Drug treatment
Brugada syndrome and caesarean delivery
Type I
Type II
Type III
V1
V1
V1
V2
V2
V2
V3
V3
V3
Fig. 1 ECG pattern in Brugada syndrome. Type I ST segment elevation is either spontaneously present or induced with a Ajmaline/Flecainide test which is considered diagnostic.2 Type II and III may lead to suspicion but drug challenge is required for diagnosis.4
with beta-blockers and amiodarone does not protect against arrhythmias and, although quinidine has some effect at normalising ST segment changes and may have a role as adjunctive therapy, it has no effect on reducing sudden cardiac death. Low-dose isoprenaline has been used in the treatment of arrhythmic storms.2,6 Several case reports document general anaesthesia in Brugada syndrome,7–13 although this is the first description of an elective caesarean section in a patient with the syndrome. Caesarean section in patients with other causes of polymorphic ventricular tachycardia such as polymorphic catecholamine-sensitive tachycardia14 and Long QT syndrome has been described.15–17 These are distinct from Brugada syndrome, and their management differs in that it is important to avoid increases in sympathetic tone and catecholamine surges which can precipitate tachyarrhythmias; both are managed with beta blockers. Three reports describe the use of regional anaesthesia14,16,17 during which efforts were made to avoid sudden loud noises that might precipitate arrhythmias. One describes the use of general anaesthesia with a remifentanil infusion.15 In contrast, arrhythmias in Brugada syndrome are thought to be caused by excess parasympathetic tone, which explains why most events occur at rest or at night.6 A recent case report documented persistent ventricular fibrillation in a pregnant woman with Brugada syndrome.18 She had stopped taking amiodarone and verapamil during pregnancy and had been admitted with progressively more frequent episodes of ventricular
J. Bramall et al. fibrillation which triggered her ICD. She became hypoxaemic requiring tracheal intubation and mechanical ventilation. An emergency caesarean section was performed and she commenced extra corporeal membrane oxygenation (ECMO) as a temporising measure until drug levels could be re-established. Amiodarone and verapamil were restarted and over the following three days the frequency of ventricular fibrillation decreased and she was weaned from ECMO support. Mother and baby were discharged uneventfully. Peripartum febrile illness such as mastitis has also been reported to provoke ECG changes typical of Brugada syndrome.19 Type I ECG changes disappeared when the fever resolved. Our main concern was the safety of bupivacaine in Brugada syndrome. Sodium channel blockade is used to provoke the characteristic ECG changes of the syndrome and there was concern that bupivacaine might cause the same changes if plasma levels were sufficient. There are case reports of epidural or paravertebral bupivacaine inducing Brugada-like ECG changes with some leading to life-threatening arrhythmias.7,8,20 We felt that plasma levels of bupivacaine would determine the effects on the heart and that the total dose of bupivacaine in a spinal anaesthetic is considerably less than that for an epidural. A study in rats showed higher plasma concentrations of lidocaine and bupivacaine after epidural compared with intrathecal administration.21 We were unable to find any reports of intrathecal bupivacaine provoking ECG changes in patients with Brugada syndrome. Alpha-adrenergic agonists were also of concern. Phenylephrine has been used, albeit unsuccessfully, in an attempt to provoke Brugada syndrome by stimulating the baroreflex causing vagal stimulation,22 but it is also the vasopressor of choice at our institution for obstetric anaesthesia. Miyazaki et al.23 noted that ‘‘selective alpha-adrenoceptor stimulation by intravenous norepinephrine in the presence of propranolol or by intravenous methoxamine consistently augmented, whereas alpha-adrenoceptor blockade reduced, ST segment elevation in three patients’’. However, in these cases alpha agonism was not performed during neuraxial blockade, when systemic vascular resistance is reduced. There is an evidence-based internet resource that provides a list of drugs which should be avoided in Brugada syndrome.24 Included is ergovrine (ergometrine), which has been used to stimulate coronary spasm in patients with Brugada syndrome leading to ventricular fibrillation.25 Its use as a uterotonic may be precluded in this condition. We were unable to find any published data on the safety of oxytocin or other uterotonics in Brugada syndrome. Oxytocin is the routine uterotonic agent at our institution, and was used without adverse effect. Routine non-invasive monitoring was used during surgery and anaesthesia. The use of invasive arterial blood pressure monitoring for this case was considered
183 but was not used as she had been completely asymptomatic and her ICD had never fired. In conclusion we performed a caesarean section for a parturient with Brugada syndrome with a twin pregnancy using intrathecal bupivacaine for central neuraxial blockade and the cautious use of phenylephrine as a vasoconstrictor.
References 1. Brugada P, Brugada J. Right bundle branch block, persistent ST segment elevation and sudden cardiac death: a distinct clinical and electrocardiographic syndrome. A multicenter report. J Am Coll Cardiol 1992;20:1391–6. 2. Antzelevitch C, Brugada P, Borggrefe M et al. Brugada syndrome: report of the second consensus conference. Endorsed by the Heart Rhythm Society and the European Heart Rhythm Association. Circulation 2005;111:659–70. 3. Antzelevitch C, Brugada P, Brugada J et al. Brugada syndrome: a decade of progress. Circ Res 2002;91:1114. 4. Napolitano C, Priori S. Brugada syndrome. Orphanet J Rare Dis 2006;1:35. 5. Brugada J, Brugada P, Brugada R. The ajmaline challenge in Brugada syndrome: a useful tool or misleading information? Eur Heart J 2003;24:1085–6. 6. Rossenbacker T, Priori S. The Brugada syndrome. Curr Opin Cardiol 2007;22:163–70. 7. Phillips N, Priestley M, Denniss A, Uther J. Brugada-type electrocardiographic pattern induced by epidural bupivacaine. Anesth Analg 2003;97:264–7. 8. Fujiwara Y, Shibata Y, Kurokawa S, Satou Y, Komatsu T. Ventricular tachycardia in a patient with Brugada syndrome during general anesthesia combined with thoracic paravertebral block. Anesth Analg 2006;102:1590–1. 9. Edge C, Blackman D, Gupta K, Sainsbury M. General anaesthesia in a patient with Brugada syndrome. Br J Anaesth 2002;89:788–91. 10. Hayashida H, Miyauchi Y. Anaesthetic management in patients with high-risk Brugada syndrome. Br J Anaesth 2006;97:118–9. 11. Vaccarella A, Vitale P, Presti C. General anaesthesia in a patient affected by Brugada syndrome. Minerva Anestesiol 2008;74:149–52. 12. Santambrogio L, Mencherini S, Fuardo M, Caramella F, Braschi A. The surgical patient with Brugada syndrome: a four-case clinical experience. Anesth Analg 2005;100:1263–6. 13. Inamura M, Okamoto H, Kuroiwa M, Hoka S. General anesthesia for patients with Brugada syndrome. A report of six cases. Can J Anesth 2005;52:409–12. 14. Chan T, Dob D. The anaesthetic management of a parturient with polymorphic catecholamine-sensitive ventricular tachycardia. Int J Obstet Anesth 2002;11:122–4. 15. Johnston A, Hall J, Levy D. Anaesthesia with remifentanil and rocuronium for caesarean section in a patient with long-QT syndrome and an automatic implantable cardioverter-defibrillator. Int J Obstet Anesth 2000;9:133–6. 16. Behl S, Wauchob T. Long QT syndrome: anaesthetic management at delivery. Int J Obstet Anesth 2005;14:347–50. 17. Hodgkinson P, McAtamney D. Combined spinal-epidural anaesthesia for caesarean section in a patient with long QT syndrome. Int J Obstet Anesth 2008;17:78–80. 18. Pagel P, Lilly R, Nicolosi A. Use of ECMO to temporize circulatory instability during severe Brugada electrical storm. Ann Thorac Surg 2010;88:982–3. 19. Ambardekar A, Lewkowiez L, Krantz M. Mastitis unmasks Brugada syndrome. Int J Cardiol 2009;132:e94–6. 20. Vernooy K, Sicouri S, Dumaine R et al. Genetic and biophysical basis for bupivacaine-induced ST segment elevation and VT/VF.
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Anesthesia unmasked Brugada syndrome. Heart Rhythm 2006;3:1074–8. 21. Berman M, Lipka L. Relative sodium current block by bupivacaine and lidocaine in neonatal rat myocytes. Anesth Analg 1994;79:350–6. 22. Probst V, Mabo P, Sacher F et al. Effect of baroreflex stimulation using phenylephrine injection on ST segment elevation and ventricular arrhythmia-inducibility in Brugada syndrome patients. Europace 2009;11:382–4. 23. Miyazaki T, Mitamura H, Miyoshi S et al. Autonomic and antiarrhythmic drug modulation of ST segment elevation in
patients with Brugada syndrome. J Am Coll Cardiol 1996;27: 1061–70. 24. Postema P, Wolpert C, Amin A et al. Drugs and Brugada syndrome patients: Review of the literature, recommendations, and an up-to-date website (www.brugadadrugs.org). Heart Rhythm 2009;6:1335–41. 25. Noda T, Shimizu W, Taguchi A et al. ST-segment elevation and ventricular fibrillation without coronary spasm by intracoronary injection of acetylcholine and/or ergonovine maleate in patients with Brugada syndrome. J Am Coll Cardiol 2002;40: 1841–7.
0959-289X/$ - see front matter c 2010 Elsevier Ltd. All rights reserved. doi:10.1016/j.ijoa.2010.10.006
Urgent cesarean delivery and prolonged ventilatory support in a parturient with Fontan circulation and undiagnosed pseudocholinesterase deficiency K.J. Grim,a K.W. Arendt,a A.K. Jacob,a C.H. Rose,b M.T. Keegana a b
Department of Anesthesiology, Mayo Clinic, Rochester, MN, USA Department of Obstetrics and Gynecology, Mayo Clinic, Rochester MN, USA
ABSTRACT A parturient with Fontan circulation required general anesthesia for urgent cesarean delivery and subsequent prolonged postoperative ventilation for newly-diagnosed pseudocholinesterase deficiency. Anesthetic management necessitated a thorough understanding of the hemodynamic principles of the Fontan circulation and physiologic adaptations during surgical delivery and recovery in the intensive care unit. c 2010 Elsevier Ltd. All rights reserved.
Keywords: Fontan: circulation, procedure; Pseudocholinesterase deficiency; Pregnancy; Positive pressure mechanical ventilation; Negative pressure mechanical ventilation; Cesarean delivery; Tricuspid atresia
Introduction Parturients with congenital heart disease pose significant anesthetic challenges in the peripartum period. Pseudocholinesterase deficiency, which occurs in 1:3200 parturients, also complicates anesthetic management, especially if the condition is undiagnosed before induction of general anesthesia. This combination of conditions in a parturient presents unique challenges to the anesthesiologist and critical care physician in both ventilatory management and hemodynamic control. We describe a parturient with tricuspid atresia and Fontan circulation who underwent general anesthesia for urgent cesarean delivery and required prolonged postoperative Accepted November 2010 Correspondence to: Katherine Arendt, Department of Anesthesiology, Mayo Clinic College of Medicine, 200 First Street SW, Rochester, MN 55905, USA. E-mail address: [email protected]
mechanical ventilation for undiagnosed pseudocholinesterase deficiency. This case has been published by our obstetric colleagues,1 but in this report we discuss the implications of anesthesia and mechanical ventilation.
Case report A 26-year-old 63 kg G3P0 parturient at 33 + 6 weeks of gestation presented for urgent cesarean delivery. Her cardiac history was significant for congenital tricuspid atresia with hypoplastic right heart, for which she underwent surgical palliation with Fontan circulation at age seven years. Her functional cardiac status before pregnancy was New York Heart Association (NYHA) class I, and she was able to participate in moderate regular exercise. Her past medical history was otherwise significant for scoliosis and an elective blepharoplasty, at an outside institution, for which she was unable to recall the type of anesthesia. She denied any family history of anesthetic complications.