© 2004 by European Society of Cardiology
CASE REPORT
Electrical storm in Brugada syndrome successfully treated using isoprenaline
aFederation of Cardiology, University Hospital Rangueil 31059 Toulouse Cedex 09, France; bClinique Pasteur Toulouse, France; cCardiovascular Institute, Hospital Clinic Barcelona, Spain
Manuscript submitted 17 August 2003. Accepted after revision 9 November 2003.
*Corresponding author. Tel.: +33-5-61-32-20-94; fax: +33-5-61-32-22-46. E-mail address: mauryjphil{at}hotmail.com (P. Maury).
 |
Abstract |
|---|
 Top Abstract Case reportDiscussionReferences |
|---|
A case of an electrical storm occurring in a patient implanted with a cardioverter-defibrillator for Brugada syndrome is reported. Recurrent ventricular fibrillation was initiated by short-coupled isolated monomorphic ventricular premature beats probably originating from the right ventricular outflow tract, associated with a manifest electrocardiographic pattern of Brugada syndrome. Infusion of atropine accelerated the heart rate but did not prevent ventricular fibrillation, however, low doses of isoprenaline quickly obviated any recurrence of ventricular fibrillation. This was associated with the disappearance of the short-coupled premature beats together with a normalization of the electrocardiographic pattern. Possible mechanisms are discussed according to the accepted pathophysiological hypothesis.
Key Words: electrical storm, Brugada syndrome, isoprenaline
|
Case report |
|---|
|
TopAbstractCase reportDiscussionReferences |
|---|
A 36-year-old man was referred to our institution for recurrent episodes of ventricular fibrillation leading to repeated syncope and shocks from an implanted cardioverter-defibrillator. The patient had been implanted 4 years ago because of an initially asymptomatic Brugada syndrome with inducible ventricular fibrillation. One year ago, he had presented with several episodes of ventricular fibrillation, which were successfully treated by the device and were possibly induced by a transient increase in vagal tone in relation to an episode of gastroenteritis. The patient remained free of new events until few days before admission, when he presented an episode of bronchitis without fever. After several shocks the patient was admitted to the intensive care unit, and continued to present incessant ventricular fibrillation (11 episodes in 1 h). Serum level of potassium was in the normal range (3.8 mEq/l).
Ventricular fibrillation was constantly induced by monomorphic ventricular premature beats probably arising from the right ventricular outflow tract with a very short coupling interval (280 ms). This happened during an accelerated sinus rhythm due to increased sympathetic tone secondary to the stress induced by the shocks (Figs. 1 and 2). Frequent similar ventricular premature beats with a long coupling interval (560 ms) were also present, which had been already noted for many years, during monitoring at our institution, but had never induced a sustained arrhythmia. A manifest electrocardiographic pattern of Brugada syndrome was present during this electrical storm (Fig. 1).
|
|
Because of the possible vagal influence on the arrhythmias in Brugada syndrome, 1 mg atropine was intravenously administered but was totally ineffective in preventing recurrent ventricular fibrillation despite further transient significant increase in the cardiac rate. Low doses (0.15 µg/min) of isoprenaline were then infused. After a few minutes, cardiac rate slightly increased and ST elevation decreased (Fig. 3). Long-coupled monomorphic premature ventricular beats persisted during the next few hours and days, but short-coupled premature ventricular beats were never seen again and ventricular fibrillation did not recur. The patient was referred to a highly specialized centre for ablation of the ventricular premature beats as constant trigger of ventricular fibrillation [1]
.
|
|
Discussion |
|---|
|
TopAbstractCase reportDiscussionReferences |
|---|
Although the prevalence of malignant ventricular arrhythmias can be high in Brugada syndrome [2]
, electrical storm is a very rare phenomenon and has seldom been reported [3–5]. In patients with Brugada syndrome, isoprenaline infusion normalizes the electrocardiographic pattern and prevents ventricular fibrillation induction during electrophysiological study [3,6]. It might, therefore, represent valuable therapy in case of an electrical storm in Brugada syndrome [3,4,6]. The Brugada electrocardiographic pattern and the ventricular excitability or vulnerability are believed to be dependent on sympathetic imbalance and on cardiac rate. Worsening of the electrophysiological conditions has been described when vagal tone increases and/or heart rate decreases [7–10]. Cardiac rate acceleration or vagal tone withdrawal could not explain the spectacular effect of isoprenaline in our patient because of the initial failure of atropine and of the increased heart rate.
The action potential shape between epicardial and endocardial layers of the right ventricular outflow tract is heterogeneous in humans. Epicardial cells display a more prominent phase 1 ("spike and dome morphology" of the action potential) due to the local preponderance of Ito [11,12]. According to the theory underlying the Brugada syndrome, in case of a mutation in the gene SCN5A encoding for the cardiac sodium channel, a reduction of Na current, due for example to faster inactivation of INa, would make outward currents (Ito) overwhelm inward currents (INa and ICaL). This would be responsible for the deeper notch and loss of the action potential dome in epicardial cells. This would, in turn, lead to both an electrocardiographic pattern of Brugada syndrome (J wave and right precordial ST elevation, due to an important transmural voltage gradient) and local short-coupled premature beats (phase 2 reentry) [12]. The occurrence of polymorphic ventricular arrhythmias could be favoured by the heterogeneicity in refractoriness between the layers and even between adjacent sites in the epicardium, leading to reentry [11].
ß-Adrenergic stimulation with isoprenaline increases ICaL and should restore the dome of epicardial action potentials, reducing the degree of heterogeneicity between layers [11,12]. This can be sufficient to decrease the degree of ST elevation [9] and could have aborted the genesis of premature beats in our case. An accelerated heart rate (decreasing Ito) [8] and/or the withdrawal of vagal influence (decreasing IKAch and increasing ICaL) [8,11] were apparently not sufficient in this case. Finally, an arrhythmic storm occurring despite apparent major physiological adrenergic stimulation, as initially documented in our patient, can be somewhat paradoxical due to the above hypothesis. The associated 
-adrenergic stimulation, leading to ST elevation in Brugada syndrome [10], could have overwhelmed the beneficial effects of ß-adrenergic stimulation.
|
References |
|---|
|
TopAbstractCase reportDiscussionReferences |
|---|
[1] Haïssaguerre M., Extramiana F., Hocini M., et al. Mapping and ablation of ventricular fibrillation associated with long-QT and Brugada syndromes. Circulation 2003; 108: 925–928.
[2] Brugada P. and Brugada J. Right bundle branch block, persistent ST segment elevation and sudden cardiac death: a distinct clinical and electrocardiographic syndrome. J Am Coll Cardiol 1992; 20: 1391–1396.[Abstract]
[3] Shimizu W. and Kamakura S. Catecholamines in children with congenital long QT syndrome and Brugada syndrome. J Electrocardiol 2001; 34:Suppl 173–175.[Medline]
[4] Suzuki H., Torigoe K., Numata O., Yazaki S. Infant case with a malignant form of Brugada syndrome. J Cardiovasc Electrophysiol 2000; 11: 1277–1280.[CrossRef][Web of Science][Medline]
[5] Chalvidan T., Deharo J.C., Dieuzaide P., Defaye P., Djiane P. Near fatal electrical storm in a patient equipped with an implantable cardioverter defibrillator for Brugada syndrome. Pacing Clin Electrophysiol 2000; 23: 410–412.[CrossRef][Medline]
[6] Tanaka H., Kinoshita O., Uchikawa S., et al. Successful prevention of recurrent ventricular fibrillation by intravenous isoproterenol in a patient with Brugada syndrome. Pacing Clin Electrophysiol 2001; 24: 1293–1294.[CrossRef][Medline]
[7] Antzelevitch C., Brugada P., Brugada J., Brugada R., Nedemanee K., Towbin J. Pathogenesis and role of the autonomic nervous system. In Camm A.J. (Ed.). Clinical approaches to tachyarrhythmias (vol. 10: the Brugada syndrome) 1999; New York Futura Publishing Co., Inc pp. 33–38 45–7.
[8] Miyazaki T., Mitamura A.H., Miyoshi S., Soejima K., Aizawa Y., Ogawa S. Autonomic and antiarrhythmic modulation of ST segment elevation in patients with Brugada syndrome. J Am Coll Cardiol 1996; 27: 1061–1070.[Abstract]
[9] Kasanuki H., Ohnishi S., Ohtura M., et al. Idiopathic ventricular fibrillation induced with vagal activity in patients without obvious heart disease. Circulation 1997; 95: 2277–2285.
[10] Miyazaki T., Mitamura H., Miyoshi S., Soejima K., Ogawa S. Autonomic and antiarrhythmic drug modulation of ST segment elevation in patients with Brugada syndrome. J Am Coll Cardiol 1996; 27: 1061–1070.[Abstract]
[11] Antzelevitch C. Ion and channels and ventricular arrhythmias: cellular and ionic mechanisms underlying the Brugada syndrome. Curr Opin Cardiol 1999; 14: 274–279.[CrossRef][Web of Science][Medline]
[12] Alings M. and Wilde A. "Brugada" syndrome: clinical data and suggested pathophysiological mechanisms. Circulation 1999; 99: 666–673.
CiteULike 
Connotea 
Del.icio.us What's this?
This article has been cited by other articles:
|
|
 |
|
  K. Kyriazis, E. Bahlmann, H. van der Schalk, and K.-H. Kuck Electrical storm in Brugada syndrome successfully treated with orciprenaline; effect of low-dose quinidine on the electrocardiogram Europace, May 1, 2009; 11(5): 665 - 666. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 M. Haissaguerre, F. Sacher, A. Nogami, N. Komiya, A. Bernard, V. Probst, S. Yli-Mayry, P. Defaye, Y. Aizawa, R. Frank, et al. Characteristics of recurrent ventricular fibrillation associated with inferolateral early repolarization role of drug therapy. J. Am. Coll. Cardiol., February 17, 2009; 53(7): 612 - 619. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
T. Wichter What role for autonomic dysfunction in Brugada Syndrome? Pathophysiological and prognostic implications Europace, July 1, 2008; 10(7): 782 - 783. [Full Text] [PDF]
|
|
|
|
|
|
P.-S. Chen and S. G. Priori The Brugada Syndrome J. Am. Coll. Cardiol., March 25, 2008; 51(12): 1176 - 1180. [Full Text] [PDF]
|
|
|
|
|
|
Developed in Collaboration With the European Heart, D. P. Zipes, A. J. Camm, M. Borggrefe, A. E. Buxton, B. Chaitman, M. Fromer, G. Gregoratos, G. Klein, A. J. Moss, et al. ACC/AHA/ESC 2006 Guidelines for Management of Patients With Ventricular Arrhythmias and the Prevention of Sudden Cardiac Death--Executive Summary: A Report of the American College of Cardiology/American Heart Association Task Force and the European Society of Cardiology Committee for Practice Guidelines (Writing Committee to Develop Guidelines for Management of Patients With Ventricular Arrhythmias and the Prevention of Sudden Cardiac Death) J. Am. Coll. Cardiol., September 5, 2006; 48(5): 1064 - 1108. [Full Text] [PDF]
|
|
|
|
|
|
Developed in Collaboration With the European Heart, D. P. Zipes, A. J. Camm, M. Borggrefe, A. E. Buxton, B. Chaitman, M. Fromer, G. Gregoratos, G. Klein, A. J. Moss, et al. ACC/AHA/ESC 2006 Guidelines for Management of Patients With Ventricular Arrhythmias and the Prevention of Sudden Cardiac Death: A Report of the American College of Cardiology/American Heart Association Task Force and the European Society of Cardiology Committee for Practice Guidelines (Writing Committee to Develop Guidelines for Management of Patients With Ventricular Arrhythmias and the Prevention of Sudden Cardiac Death) J. Am. Coll. Cardiol., September 5, 2006; 48(5): e247 - e346. [Full Text] [PDF]
|
|
|
|
 |
|
 D. P. Zipes, D. P. Zipes, A. J. Camm, M. Borggrefe, A. E. Buxton, B. Chaitman, M. Fromer, G. Gregoratos, G. Klein, A. J. Moss, et al. ACC/AHA/ESC 2006 guidelines for management of patients with ventricular arrhythmias and the prevention of sudden cardiac death--executive summary: A report of the American College of Cardiology/American Heart Association Task Force and the European Society of Cardiology Committee for Practice Guidelines (Writing Committee to Develop Guidelines for Management of Patients with Ventricular Arrhythmias and the Prevention of Sudden Cardiac Death) Developed in collaboration with the European Heart Rhythm Association and the Heart Rhythm Society. Eur. Heart J., September 1, 2006; 27(17): 2099 - 2140. [Full Text] [PDF]
|
|
|
|
|
|
Writing Committee Members, D. P. Zipes, A. J. Camm, M. Borggrefe, A. E. Buxton, B. Chaitman, M. Fromer, G. Gregoratos, G. Klein, A. J. Moss, et al. ACC/AHA/ESC 2006 guidelines for management of patients with ventricular arrhythmias and the prevention of sudden cardiac death: A report of the American College of Cardiology/American Heart Association Task Force and the European Society of Cardiology Committee for Practice Guidelines (Writing Committee to Develop Guidelines for Management of Patients With Ventricular Arrhythmias and the Prevention of Sudden Cardiac Death) Developed in collaboration with the European Heart Rhythm Association and the Heart Rhythm Society Europace, September 1, 2006; 8(9): 746 - 837. [Full Text] [PDF]
|
|
|
|
|
|
A. Watanabe, K. Fukushima Kusano, H. Morita, D. Miura, W. Sumida, S. Hiramatsu, K. Banba, N. Nishii, S. Nagase, K. Nakamura, et al. Low-dose isoproterenol for repetitive ventricular arrhythmia in patients with Brugada syndrome Eur. Heart J., July 1, 2006; 27(13): 1579 - 1583. [Abstract] [Full Text] [PDF]
|
|
| ||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||