medigraphic.com

2000, Number 3

<< Back Next >>

Arch Cardiol Mex 2000; 70 (3)

ST segment elevation right bundle branch block and sudden death: Brugada´s syndrome

Asensio LE, Álvarez MB, Lozano DE, Farías VA, Brugada TR, Brugada TP, Brugada TJ

Language: Spanish
References: 49
Page: 301-311
PDF size: 461.06 Kb.


ABSTRACT

Brugada’s syndrome is one of the main causes of sudden death in young adults without a structural heart disease. This is an electrical cardiac illness secondary to a mutation of SCN5A gene of chromosome 3 that has a dominant autosomic transmission pattern. This mutation implies the dysfunction of the sodium channel that increases the Ito, loosing the dome of the epicardiac action potential phase two. An “all or none” repolarization pattern ensues and gives rise to a phase two reentry. This kind of reentry is responsible for the initiation and perpetuation of malignant ventricular arrhythmias among these patients. The clinical characteristics of the syndrome are the right bundle branch block, ST segment elevation from V1 to V3 leads and sudden death or syncope. In some patients, a pharmacological test must be done with ajmaline or procainamide to unmask the electrocardiographic changes. At present, the only effective treatment is the implantable cardioverter defibrillator (ICD). This device has the capability to reduce mortality from 40% annually to 0% at ten years. Pharmacological treatment is not useful.


REFERENCES

  1. Brugada P, Brugada J: Right bunble branch block, persistent ST segment elevation and sudden cardiac death: A distinct clinical and electrocardiographic syndrome. J Am Coll Cardiol 1992; 20: 1391-6.

  2. Altemose G, Buxton A: Idiopathic ventricular tachycardia. Annu Rev Med 1999; 50: 159-77.

  3. Antzelevich C, Brugada P, Brugada J, Brugada R, Nademanee K, Towbin J: The Brugada Syndrome. Futura Publishing Co. Clinical approaches to tachyarrhythmias Vol. 10, 1999.

  4. Tohyou Y, Nakazawa K, Ozawa A: A survey in the incidence of right bundle branch block with ST segment elevation among normal population. Jpn J Electrocardiol 1995; 15: 223-6.

  5. Namiki T, Ogura T, Kubawara Y: Five-year mortality and clinical characteristics of adult subjects with right bundle branch block and ST elevation. Circulation 1995; 93: 334.

  6. Hata Y, Chiba N, Hotta K: Incidence and clinical significance of right bundle branch block and ST segment elevation in V1-V3 6-to 10-year old school children in Japan. Circulation 1997; 20: 2310.

  7. Lafaye A, Desachy A, Roustan J, Lagrange P, Vignon P, François B: Une cause rare de mort subite de I’adulte jeune a “coeur normal”: Le syndrome de Brugada. Presse Med 13 1999; 28(10): 527-30.

  8. Asenjo R, Madariaga R, Morris R, Montagna R, Nuñez J, Ortiz M, et al: Muerte súbita por fibrilación ventricular recuperada: ¿Síndrome de Brugada? Rev Med Chil 1998; 126(7): 814-21.

  9. Antzelevitch C: Ion channels and ventricular arrhythmias: cellular and ionic mechanisms underlyg the Brugada syndrome. Curr Opin Cardiol 1999; 14(3): 274-9.

  10. Corrado D, Buja G, Basso C, Nava A, Thiene G: What is the Brugada syndrome? Cardiol Rev 1999; 7(4): 191-5.

  11. Brugada P: El síndrome de Brugada. Conferencia en el 10th Madrid Arrtythmia Meeting 1999.

  12. Gussak I, Antzelevitch C, Bjerregaard P, Towbin JA, Chaitman BR: The Brugada syndrome: clinical, electrophysiological and genetic aspects. J Am Coll Cardiol 1999; 33(1): 5-15.

  13. Zygmunt AC: The calcium-activated conductace Ito2, in canine ventricle is a chloride current. Biophys J 1993; 64: A389 (Abstract).

  14. Chen Q, Kirsch G, Zhang D: Genetic basis and molecular mechanisms for idiopathic ventricular fibrillation. Nature 1998; 392: 293-6.

  15. Di Diego J, Zun Q, Antzelevitch C: Ito and action potential notch are smaller in left Vs. rigth canine ventricular epicardium. Am J Physiol 1996; 271: H548-61.

  16. Yan GX, Antzelevitch C: Cellular basis for the electrocardiographic J wave. Circulation 1996; 93: 372-9.

  17. Antzelevitch C, Sicouri S, Lukas A, Di Diego J, Nesterenko V, Liu D, et al: Clinical implications of electrical heterogeneity in the heart: The electrophysiology and pharmacology of epicardial, M and endocardial cells. En Podrid PJ, Kowey PR Eds. Cardiac arrhythmia: Mechanism and management. Baltimore, MD, Williams and Wilkins 1995: 88-107.

  18. Lukas A, Antzelevitch C: Phase 2 reentry as a mechanism of initiation of circus movement reentry in canine epicardium exposed to simulated ischemia. The antiarrhythmic effects of AP-4. Cardiovasc Res 1996; 32: 593-603.

  19. Eckardt L, Kirchof P, Johna R, Breithard G, Borgreffe M, Haverkamp W: Transient local changes in right ventricular monophasic action potentials due to ajmaline in a patient with Brugada syndrome. J Cardiovasc Electrophysiol 1999; 10(7): 1010-5.

  20. Lukas A, Antzelevitch C: Differences in the electrophysiologic response of canine ventricular epicardium and endocardium to ischemia: Role of the transient outward current. Circulation 1993; 88: 2903-15.

  21. Suzuki J, Tsubone H, Sugano S: Characteristics of ventricular activation and recovery patterns in the rat. J Vet Med Sci 1992; 54: 711-16.

  22. Janse M, Wilde A: Molecular mechanisms of arrhythmias. Rev Port Cardiol 1998; 17(Supl 2): 41-6.

  23. Litovsky SH, Antzelevitch C: Diferences in the electrophysiologic response of canine ventricular subendocardium and subepicardium to acetylcholine and isoproterenol: A direct effect of acetylcholine in ventricular myocardium. Circ Res 1990; 67: 615-27.

  24. Yan G, Antzelevitch C: Cellular basis for idiopathic VT/VF syndrome. Circulation 1996; 94: 625 (abstract).

  25. Nademanee K, Veerakul G, Nimmannit S, Chaowakul V, Bhuripanyo K, Likittanasombat K, et al: Arrhythmogenic marker for the sudden unexplained death syndrome in Thai men. Circulation 1997; 96: 2595-2600.

  26. Viskin S, Lesh M, Eldar M, Fish R, Setbon I, Laniado S, et al: Mode of onset of malignant ventricular arrhythmias in idiopathic ventricular fibrillation. J Cardiovasc Electrophysiol 1997; 8: 1115-20.

  27. Miyazaki T, Mitamura 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: 1061-70.

  28. Agostini D, Scanu P, Loiselet P, Babatasi G, Darlas Y, Grollier G, et al: Iodine 123 Metaiodobenzilguanidine SPECT of regional cardiac denervation in Brugada syndrome. J Nucl Med 1998; 39(7): 1129-32.

  29. Nomura M, Nada T, Endo J, Kondo Y, Yukinaka M, Saito K, et al: Brugada syndrome associated with an autonomic disorder. Heart 1998; 80(2): 194-6.

  30. Matsuo K, Kurita T, Inagaki M, Kakishita M, Aihara N, Shimizu W, et al: The circadian pattem of the development of ventricular fibrillation in patients with Brugada syndrome. Eur Heart J 1999; 20(6): 465-70.

  31. Brugada J, Brugada P: Further characterization of the syndrome of right bundle branch block, ST segment elevation and sudden cardiac death. J Cardiovasc Electrophysiol 1997; 8: 325-331.

  32. Bertaglia E, Michieletto M, Spedicato L, Pascotto P: Right bundle branch block, intermittent ST segment elevation and inducible ventricular tachicardia in an asymptomatc patient: An unusual presentation of the Brugada syndrome? G Ital Cardiol 1998; 28(8): 893-8.

  33. Matsuo K, Shimizu W, Kurita T, Inagaki M, Aihara N, Kamakura S: Dynamic changes of 12-lead electrocardiograms in a patient with Brugada syndrome. J Cardiovasc Electrophysiol 1998; 9(5): 513-6.

  34. Alings M, Wilde A: Brugada syndrome. Clinical data and suggested pathophysiological mechanisms. Circulation 1999; 99: 666-73.

  35. Krishnan S, Antzelevitch C: Flecainide-induced arrhythmia in canine ventricular epicardium: Phase 2 reentry? Circulation 1993; 87: 562-72.

  36. Di Diego J, Antzelevitch C: Pinacidil-induced electrical heterogeneity and extrasystolic activity in canine ventricular tissues: Does activation on ATP-regulated potassium current promote phase 2 reentry? Circulation 1993; 88: 1177-89.

  37. Goethals P, Debruyne P, Saffarin M: Drug-induced Brugada syndrome. Acta Cardiol 1998; 53(3): 157-60.

  38. Fujiki A, Usui M, Nasagawa H, Mizumaki K, Hayashi H, Inoue H: ST segment elevation in the right precordial leads induced with class Ic antiarrhythmic drugs: insight into the mechanism of Brugada syndrome. J Cardiovasc Electrophysiol 1999; 10(2): 214-8.

  39. Krishnan S, Josephson M: ST segment elevation induced by class IC antiarrythmic agents: underlying electrophysiologic mechanisms and insights into drug-induced proarrhythmia. J Cardiovasc Electrophysiol 1998; 9(11): 1167-72.

  40. Rampazzo A, Nava A, Danieli G, Buja G, Daliento L, Fasoli G, et al: The gene for arrhythmogenic right ventricular cardiomyopathy maps to chromosome l4q23-Q24. Hum Mol Genet 1994; 3: 959-62.

  41. Rampazzo A, Nava A, Miorin M, Fonderico P, Pope B, Tiso N, et al: ARVD4, a new locus for arrhythmogenic right ventricular cardiomyopathy, maps to chromosome 2 long arm. Genomics 1997; 45(2): 259-63.

  42. Chiale P, Przybylski J, Laino R: Electrocardiographic changes evoked by ajmaline in chronic Chagas’ disease without manifest myocarditis. Am J Cardiol 1982; 49: 14-20.

  43. Brugada P, Brugada J, Brugada R, Geelin P: Use of the prophylactic implantable cardioverter defibrillator for patients with normal hearts. Am J Cardiol, 11 1999; 83(5B): 98D-100D.

  44. Brugada J, Brugada R, Brugada P: Right bundle branch block and ST segment elevation in leads V1 to V2-V3: a marker for sudden death in patients without demonstrable structural heart disease. Circulation 1998; 97(5): 457-60.

  45. Chevalier P, Touboul P: Les fibrillations ventriculaires idiopathiques. Arch Mal Coeur 1999; 91 (spec 1): 29-36.

  46. Chinusi M, Aizawa Y, Ogawa Y, Shiba M, Takahashi K: Discrepant drug action of dysopiramide on ECG abnormalities and induction of ventricular arrhythmias in a patient with Brugada syndrome. J Electrocardiol 1997; 30(2): 133-6.

  47. Jordaens L, Tavernier R, Jaroslaw K, Dimmer C: Ventricular arrhythmias in apparently healthy subjects. PACE 1997; 20: 2692-98.

  48. Brugada P, Brugada J, Brugada R: Ajmaline unmasks apparent right bundle branch block and ST segment elevation in V1 in patients with “idiopathic ventricular fibrilation”. (Abstract) Eur Heart J 1996; 17: 212.

  49. Coumel P: Polymorphous ventricular tachyarrhythmias in the absence of structural heart disease. PACE 1995; 18(pte.1): 633-6.




2020     |     www.medigraphic.com