Association of congenital, diffuse electrical disease in children with normal heart: Sick sinus syndrome, intraventricular conduction block, and monomorphic ventricular tachycardia

Journal of Cardiovascular Electrophysiology

Volumn 19, Issue 5, 2008, Pages 550-555

  Iturralde Torres, Pedro ^a   Nava Townsend, Santiago ^a   Gómez Flores, Jorge ^a   Medeiros Domingo, Argelia ^a   Colín Lizalde, Luis ^a   Hermosillo, Antonio G ^a   Victoria, Diana ^a   Márquez, Manlio F ^a  

^a INSTITUTO NACIONAL DE CARDIOLOGÍA IGNACIO CHÁVEZ   (Mexico)

Author keywords

Bundle branch block; Cardiac conduction disorder; Channelopathies; Children; Congenital arrhythmias; Intraventricular block; Long QT syndrome; Monomorphic ventricular tachycardia; Sinus node disease

Indexed keywords

AMIODARONE; LIDOCAINE;

ARTICLE; ARTIFICIAL HEART PACEMAKER; ATRIOVENTRICULAR BLOCK; BRADYCARDIA; BRUGADA SYNDROME; CASE REPORT; CATECHOLAMINERGIC POLYMORPHIC VENTRICULAR TACHYCARDIA; CHANNELOPATHY; CLINICAL FEATURE; DISEASE ASSOCIATION; DISEASE SEVERITY; ECHOCARDIOGRAPHY; ELECTROCARDIOGRAPHY; ELECTROPHYSIOLOGY; HEART MUSCLE CONDUCTION DISTURBANCE; HEART RIGHT BUNDLE BRANCH BLOCK; HOLTER MONITORING; HUMAN; LONG QT SYNDROME; MALE; MONOMORPHIC VENTRICULAR TACHYCARDIA; PATHOPHYSIOLOGY; PHENOTYPE; PRESCHOOL CHILD; PRIORITY JOURNAL; PROGRESSIVE CARDIAC CONDUCTION DEFECT; RADIOFREQUENCY ABLATION; SCHOOL CHILD; SHORT QT SYNDROME; SICK SINUS SYNDROME; SINUS RHYTHM; SYMPTOMATOLOGY;

ATRIOVENTRICULAR BLOCK; CHILD; FEMALE; HUMANS; INFANT; MALE; SICK SINUS SYNDROME; TACHYCARDIA, VENTRICULAR;

EID: 43049084771     PISSN: 10453873     EISSN: 15408167     Source Type: Journal    
DOI: 10.1111/j.1540-8167.2007.01006.x     Document Type: Article

References (28)

1. Schott JJ, Alshinawi C, Kyndt F, Probst V, Hoorntje TM, Hulsbeek M, Wilde AA, Escande D, Mannens MM, Le Marec H : Cardiac conduction defects associated with mutations in SCN5A. Nat Genet 1999 23 : 20 21.
2. Viswanathan PC, Balser JR : Inherited sodium channelopathies: A continuum of channel dysfunction. Trends Cardiovasc Med 2004 14 : 28 35.
3. Corrado D, Basso C, Thiene G : Is it time to include ion channel diseases among cardiomyopathies? J Electrocardiol 2005 38 : 81 87.
4. Priori SG : Inherited arrhythmogenic disease; the complexity beyond monogenic disorders. Circ Res 2004 94 : 140 145.
5. Ackerman MJ : Molecular basis of congenital and acquired long QT syndromes. J Electrocardiol 2004 37 : 1 6.
6. 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 1396.
7. Tan HL, Bink-Boelkens MTE, Bezzina CR, Viswanathan PC, Beaufort-Krol GC, van Tintelen PJ, van den Berg MP, Wilde AA, Balser JR : A sodium-channel mutation causes isolated cardiac conduction disease. Nature 2001 409 : 1043 1047.
8. Benson DW, Wang DW, Dyment M, Knilans TK, Fish FA, Strieper MJ, Rhodes TH, George AL : Congenital sick sinus syndrome caused by recessive mutations in the cardiac sodium channel gene (SCN5A). J Clin Invest 2003 112 : 1019 1028.
9. Grant AO, Carboni MP, Neplioueva, Starmer CF, Memmi M, Napolitano C, Priori S : Long QT syndrome, Brugada syndrome, and conduction system disease are linked to a single sodium channel mutation. J Clin Invest 2002 110 : 1201 1209.
10. Remme CA, Verkerk AO, Nuyens D, van Ginneken AC, van Brunschot S, Belterman C, Wilders R, van Roon MA, Tan HL, Wilde AA, Carmeliet P, de Bakker J, Veldkamp MW, Bezzina CR : Overlap syndrome of cardiac sodium channel disease in mice carrying the equivalent mutation of human SCN5A-1795insD. Circulation 2006 114 : 2584 2594.
11. Chang CC, Acharfi S, Wu MH, Chiang FR, Wang JK, Sung TC, Chahine M : A novel SCN5A mutation manifests as a malignant form of long QT syndrome with perinatal onset of tachycardia/bradycardia. Cardiovasc Res 2004 64 : 268 278.
12. Smits JP, Koopmann TT, Wilders R, Veldkamp MW, Opthof T, Bhuiyan ZA, Mannens MM, Balser JR, Tan HL, Bezzina CR, Wilde AA : A mutation in the human cardiac sodium channel (E161K) contributes to sick sinus syndrome, conduction disease, and Brugada syndrome in two families. J Mol Cell Cardiol 2005 38 : 965 968.
13. Makiyama T, Asao M, Tsuji K, Doi T, Ohno S, Takenaka K, Kobori A, Ninomiya T, Yoshida H, Takano M, Makita N, Tanagisawa F, Higashi Y, Takeyama Y, Kita T, Horie M : High risk for bradyarrhythmic complications in patients with Brugada syndrome caused by SCN5A gene mutations. J Am Coll Cardiol 2005 46 : 2100 2106.
14. Rossenbacker T, Carroll SJ, Liu H, Kuiperi C, de Ravel TJ, Devriendt K, Carmeliet P, Kass RS, Heidbuchel H : Novel pore mutation in SCN5A manifests as a spectrum of phenotypes ranging from atrial flutter, conduction disease, and Brugada syndrome to sudden cardiac death. Heart Rhythm 2004 1 : 610 615.
15. Smits JP, Eckardt L, Probst V, Bezzina CR, Schott JJ, Remme CA, Haverkamp W, Breithardt G, Escande D, Schulze-Bahr E, LeMarec H, Wilde AA : Genotype-phenotype relationship in Brugada syndrome: Electrocardiographic features differentiate SCN5A-related patients from non-SCN5A-related patients. J Am Coll Cardiol 2002 40 : 350 356.
16. Ruipérez Abizanda JA, Campos Peris JV, Garcia Garcia J, Jaen Armand E, Ruiz Ros JA, Salas Nieto J, Rodriguez Ruiz P, Marco Quiles J, Martinez Jérez M, Pico Aracil F : Estudio clínico de un nuevo fármaco antiarritmico: Propafenona. Resultados a corto y largo plazo. Arch Inst Cardiol Mex 1981 51 : 371 376.
17. Scanu P, Grollier G, Guilleman D, Iselin M, Bustany P, Hurpe JM, Potier JC : Malignant ventricular tachycardia during propafenone treatment in a child with junctional automatic tachycardia: Effectiveness of intravenous molar sodium lactate. Pacing Clin Electrophysiol 1991 14 : 783 786.
18. Kang TS, Yoon YW, Park S, Hong BK, Kim D, Kwon HM, Kim HS : A case of acute ventricular capture threshold rise associated with flecainide acetate. Yonsei Med J 2006 47 : 152 154.
19. Bertomeu-Gonzalez V, Ruiz-Granell R, Garcia-Civera R, Morell-Cabedo S, Ferrero A : Syncopal monomorphic ventricular tachycardia with pleomorphism, sensitive to antitachycardia pacing in a patient with Brugada syndrome. Europace 2006 12 : 1048 1050.
20. Probst V, Denjoy I, Meregalli PG, Amirault JC, Sacher F, Mansourati J, Babuty D, Villain E, Victor J, Schott JJ, Lupoglazoff JM, Mabo P, Veltmann C, Jesel L, Chevalier P, Clur SA, Haissaguerre M, Wolpert C, Le Marec H, Wilde AA : Clinical aspects and prognosis of Brugada syndrome in children. Circulation 2007 115 : 2042 2048.
21. Viskin S, Alla SR, Barron HV, Heller K, Saxon L, Kitzis I, Hare GF, Wong MJ, Lesh MD, Scheinman MM : Mode of onset of torsade de pointes in congenital long QT syndrome. J Am Coll Cardiol 1996 28 : 1262 1268.
22. Noda T, Shimizu W, Satomi K, Suyama K, Kurita T, Aihara N, Kamakura S : Classification and mechanism of Torsade de Pointes initiation in patients with congenital long QT syndrome. Eur Heart J 2004 23 : 2149 2154.
23. Tan HL, Bardai A, Shimizu W, Moss AJ, Schulze-Bahr E, Noda T, Wilde A : Genotype-specific onset of arrhythmias in congenital long-QT syndrome: Possible therapy implications. Circulation 2006 114 : 2096 2103.
24. Garson A Jr., Dick M 2nd., Fournier A, Gillette PC, Hamilton R, Kugler JD, van Hare GF 3rd., Vetter V, Vick GW : The long QT syndrome in children. An international study of 287 patients. Circulation 1993 6 : 1866 1872.
25. Wolf C, Berul C : Inherited conduction system abnormalities-one group of diseases, many genes. J Cardiovasc Electrophysiol 2006 17 : 446 455.
26. Moskowitz IPG, Kim JB, Moore ML, Wolf CM, Peterson MA, Shendure J, Nobrega MA, Yokota Y, Berul C, Izumo S, Seidman JG, Seideman CE : A molecular pathway including idf, tbx5, and nkx2-5 required for cardiac conduction system development. Cell 2007 129 : 1365 1376.
27. Rifai L, Maazouzi W, Sefiani A : Novel point mutation in the NEX2-5 in a Moroccan family with atrioventricular conduction disturbance and an atrial septal defect in the oval fossa. Cardiol Young 2007 17 : 107 109.
28. Mohler PJ, Le Scouarnec S, Denjoy I, Lowe JS, Guicheney P, Caron L, Driskell IM, Schott JJ, Norris K, Leenhardt A, Kim RB, Escande D, Roden DM : Defining the cellular phenotype of "ankyrin-B syndrome" variants: Human ANK2 associated with clinical phenotypes display a spectrum of activities in cardiomyocytes. Circulation 2007 115 : 432 441.