Abstract
In recent years a number of genetic heart diseases have been recognized that can be complicated by malignant arrhythmias leading to sudden death [1]. These genetic diseases can be divided into two groups: (1) channelopathies (congenital dysfunction of cellular ion-channels without macroscopic heart disease) and (2) genetic cardiomyopathies.
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References
Sarkozy A, Brugada P (2005) Sudden cardiac death and inherited arrhythmia syndromes. J Cardiovasc Electrophysiol 16:S8–S20
Jervell A, Lange-Nielsen F (1957) Congenital deaf mutism, functional heart disease with prolongation of the QT interval and sudden death. Am Heart J 54:59–68
Romano C, Gemme G, Pongiglione R (1963) Aritmie cardiache rare dell’età pediatrica, II: accessi sincopali per fibrillazione ventricolare parossistica. Clin Pediatr (Bologna) 45:656–683
Schwartz PJ (1985) Idiopathic long QT syndrome: progress and questions. Am Heart J 109:399–411
Marks ML, Trippel DL, Keating MT (1995) Long QT syndrome associated with syndactyly identified in females. Am J Cardiol 76:744–745
Duggal P, Vesely MR, Wattanasirichaigoon D et al (1998) Mutation of the gene for IsK associated with both Jervell and Lang-Nielsen and Romano-Ward forms of long QT syndromes. Circulation 97:142–146
Locati EH, Zareba W, Moss AJ et al (1998) Age-and sex-related differences in clinical manifestations in patients with congenital long-QT syndrome: findings from the International LQTS Registry. Circulation 97:2237–2244
Priori SG, Schwartz PJ, Napolitano C et al (2003) Risk stratification in the long-QT syndrome. N Engl J Med 348:1866–1874
Napolitano C, Bloise R, Priori S (2006) Long QT syndrome and short QT syndrome: how to make correct diagnosis and what about eligibility for sport activity. J Cardiovasc Med 7:250–256
Gaita F, Giustetto C, Bianchi F et al (2003) Short QT syndrome. A familial cause of sudden death. Circulation 108:965–970
Brugada R, Hong K, Dumaine R et al (2004) Sudden death associated with short-QT syndrome linked to mutations in HERG. Circulation 109:30–35
Brugada P, Brugada J (1992) Right bundle branch block, persistent ST segment elevation and sudden cardiac death: a distinct clinical and electrocardiographic syndrome. J Am Coll Cardiol 20:1391–1396
Brugada R, Brugada J, Antzelevitch C et al (2000) Sodium channel blockers identify risk for sudden death in patients with ST-segment elevation and right bundle branch block but structurally normal hearts. Circulation 101:510–515
Brugada P, Brugada R, Mont L et al (2003) Natural history of Brugada syndrome: the prognostic value of programmed electrical stimulation of the heart. J Cardiovas Electrophysiol 14:455–457
Priori SG, Napolitano C, Gasparini M et al (2002) Natural history of Brugada syndrome. Insight for risk stratification and management. Circulation 105:1342–1347
Antzelevitch C, Brugada P, Borggrefe M et al (2006) Brugada syndrome. Report of the second consensus conference. Circulation 111:659–670
Delise P, Marras E, Bocchino M (2006) Brugada-like electrocardiogram pattern: how to stratify the risk for sudden cardiac death. Is sport activity contraindicated? J Cardiovasc Med 7:239–245
Coumel P, Fidelle J, Lucet V et al (1978) Catecholaminergic-induced severe ventricular arrhythmias with Adams-Stokes syndrome in children: report of four cases. Br Heart J 40:28–37
Leenhardt A, Lucet V, Denjoy I et al (1995) Catecholaminergic polymorphic ventricular tachycardia in children. A 7-year follow-up of 21 patients. Circulation 91:1512–1519
Gasparini M, Priori S, Mantica M et al (2002) Programmed electrical stimulation in Brugada syndrome: how reproducible are the results? J Cardiovasc Electrophysiol 13:880–887
Kanda M, Shimizu W, Matsuo K et al (2002) Electrophysiologic characteristics and implications of induced ventricular fibrillation in symptomatic patients with Brugada syndrome. J Am Coll Cardiol 39:1799–1805
Eckardt L, Probst V, Smits JP et al (2005) Long-term prognosis of individuals with right precordial ST-segment elevation Brugada syndrome. Circulation 111:257–263
Brugada J, Brugada R, Brugada P (2003) Determinants of sudden death in individuals with the electrocardiographic pattern of Brugada syndrome and no previous cardiac arrest. Circulation 108:3092–3096
ACC/AHA/ESC (2006) 2006 guidelines for management of patients with ventricular arrhythmias and the prevention of sudden cardiac death — Executive summary. Eur Heart J 27:2099–2140
Belhassen B, Viskin S, Fish R et al (1999) Effects of electrophysiologic-guided therapy with Class IA antiarrhyhtmic drugs on the long term outcome of patients with idiopathic ventricular fibrillation with or without the Brugada syndrome. J Cardiovasc Electrophysiol 10:1301–1312
Hermida JS, Denjoy I, Clerc J et al (2004) Hydroquinidine therapy in Brugada Syndrome. J Am Coll Cardiol 43:1853–1860
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Delise, P. (2007). Prevention of Sudden Death in Patients with Genetic Arrhythmias. In: Gulizia, M.M. (eds) Current News in Cardiology. Springer, Milano. https://doi.org/10.1007/978-88-470-0636-2_33
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DOI: https://doi.org/10.1007/978-88-470-0636-2_33
Publisher Name: Springer, Milano
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