Congenital long QT syndrome: Diagnosis and management in pediatric patients

Current Treatment Options in Cardiovascular Medicine

Volumn 8, Issue 5, 2006, Pages 387-395

  Bar Cohen, Yaniv ^a   Silka, Michael J ^b  

^a CHILDREN S HOSPITAL LOS ANGELES   (United States)

^b NONE

Author keywords

[No Author keywords available]

Indexed keywords

ANTIARRHYTHMIC AGENT; ATENOLOL; BETA ADRENERGIC RECEPTOR BLOCKING AGENT; BETA ADRENERGIC RECEPTOR STIMULATING AGENT; CIMETIDINE; METOPROLOL; MEXILETINE; NADOLOL; PROPRANOLOL; QUINIDINE;

ATAXIA; BLURRED VISION; BRADYCARDIA; BRONCHOSPASM; CHILD; CONFUSION; COST EFFECTIVENESS ANALYSIS; DEFIBRILLATOR; DEPRESSION; DISEASE CLASSIFICATION; DRUG BLOOD LEVEL; DRUG CHOICE; DRUG CONTRAINDICATION; DRUG COST; DRUG HALF LIFE; ECG ABNORMALITY; FAMILY HISTORY; GENETIC SCREENING; HEART BLOCK; HEART DENERVATION; HEART DISEASE; HEART PACING; HEART PROARRHYTHMIA; HEART REPOLARIZATION; HIGH RISK PATIENT; HUMAN; HYPOGLYCEMIA; INCIDENCE; LONG QT SYNDROME; NAUSEA; PATIENT CARE; QT INTERVAL; RESPIRATORY DISTRESS; REVIEW; RISK FACTOR; SINUS BRADYCARDIA; SUDDEN DEATH; SYNCOPE; TREMOR; WHEEZING;

EID: 33749039447     PISSN: 10928464     EISSN: None     Source Type: Journal    
DOI: 10.1007/s11936-006-0043-5     Document Type: Review

References (38)

1. Moss AJ: Long QT syndrome. JAMA 2003, 289:2041-2044.
2. Shah M, Akar FG, Tomaselli GF: Molecular basis of arrhythmias. Circulation 2005, 112:2517-2529.
3. Roden DM: Drug-induced prolongation of the QT interval. N Engl J Med 2004, 350:1013-1022.
4. Prioro SG, Schwartz PJ, Napolitano C, et al.: Risk stratification in the long-QT syndrome. N Engl J Med 2003, 348:1866-1874.
5. Goel AK, Berger S, Pelech A, Dhala A: Implantable cardioverter defibrillator therapy in children with long QT syndrome. Pediatr Cardiol 2004, 25:370-378.
6. Moss AJ, Robinson JL, Gessman L, et al.: Comparison of clinical and genetic variables of cardiac events associated with loud noise versus swimming among subjects with the long QT syndrome. Am J Cardiol 1999, 84:876-879.
7. Zareba W, Moss AJ, Schwartz PJ, et al.: Influence of genotype on the clinical course of the long-QT syndrome. N Engl J Med 1998, 339:960-965.
8. Splawski I, Shen J, Timothy KW, et al: Spectrum of mutations in long-QT syndrome genes KVLQT1, HERG, SCN5A, KCNE1, and KCNE. Circulation 2000, 102:1178-1185.
9. Schwartz PJ, Moss AJ, Vincent GM, Crampton RS: Diagnostic criteria for the long QT syndrome. An update. Circulation 1993, 88:782-784.
10. Zareba W, Moss, AJ: Long QT syndrome in children. J Electrocardiol 2001, 34(suppl): 167-171.
11. Malfatto G, Beria G, Sala S, et al.: Quantitative analysis of T wave abnormalities and their prognostic implications in the idiopathic long QT syndrome. J Am Coll Cardiol 1994, 23:296-301.
12. Zhang L, Timothy KW, Vincent GM, et al.: Spectrum of ST-T-wave patterns and repolarization parameters in congenital long-QT syndrome: ECG findings identify genotypes. Circulation 2000, 102:2849-2855.
13. Seslar SP, Zimetbaum P, Berul CI, Josephson ME: Diagnosis of congenital long QT syndrome. 2006 UpTo-Date. Available at http://www.utdol.com. Accessed February 16, 2006.
14. Nemec J, Ackerman MJ, Tester DJ, et al.: Catecholamine-provoked microvoltage T wave alternans in genotyped long QT syndrome. Pacing Clin Eiectrophysiol 2003, 26: 1660-1667.
15. Napolitano C, Priori SG, Schwartz PJ: Significance of QT dispersion in the long QT syndrome. Prog Cardiovasc Dis 2000, 42:345-350.
16. van den Berg MP, Haaksma J, Veeger NJ, Wilde AA: Diurnal variation of ventricular repolarization in a large family with LQT3-Brugada syndrome characterized by nocturnal sudden death. Heart Rhythm 2006, 3:290-295.
17. Garson A Jr, Dick M 2nd, Fournier A, et al.: The long QT syndrome in children. An international study of 287 patients. Circulation 1993, 87:1866-1872.
18. Christiansen JL, Guccione P, Garson A Jr: Difference in QT interval measurement on ambulatory ECG compared with standard ECG. Pacing Clin Electrophysiol 1996, 19:1296-1303.
19. Dillenburg RF, Hamilton RM: Is exercise testing useful in identifying congenital long QT syndrome? Am J Cardiol 2002, 89:233-236.
20. Takenaka K, Ai T, Shimizu W, et al.: Exercise stress test amplifies genotype-phenotype correlation in the LQT1 and LQT2 forms of the long-QT syndrome. Circulation 2003, 107:838-844.
21. Tester DJ, Will ML, Haglund CM, Ackerman MJ: Effect of clinical phenotype on yield of long QT syndrome genetic testing. J Am Coll Cardiol 2006, 47:764-768.
22. Moss AJ, Zareba W, Hall WJ, et al.: Effectiveness and limitations of beta-blocker therapy in congenital long-QT syndrome. Circulation 2000, 101:616-623.
23. Priori SG, Napolitano C, Schwartz PJ, et al.: Association of long QT syndrome loci and cardiac events among patients treated with beta-blockers. JAMA 2004, 292:1341-1344.
24. Khan IA, Gowda RM: Novel therapeutics for treatment of long-QT syndrome and torsade de pointes. Int J Cardiol 2004, 95:1-6.
25. Zareba W, Moss AJ, Daubert JP, et al.: Implantable cardioverter defibrillator in high-risk long QT syndrome patients. J Cardiovasc Electrophysiol 2003, 14:337-341.
26. Stephenson EA, Batra AS, Knilans TK, et al.: A multicenter experience with novel implantable cardioverter defibrillator configurations in the pediatric and congenital heart disease population. J Cardiovasc Electrophysiol 2006, 17:41-46.
27. Stephenson EA, Cecchin F, Walsh EP, Berul CI: Utility of routine follow-up defibrillator threshold testing in congenital heart disease and pediatric populations. J Cardiovasc Electrophysiol 2005, 16:69-73.
28. DeMaso DR, Lauretti A, Spieth L, et al.: Psychosocial factors and quality of life in children and adolescents with implantable cardioverter-defibrillators. Am J Cardiol 2004, 93:582-587.
29. Schwartz PJ: QT prolongation, sudden death, and sympathetic imbalance: the pendulum swings. J Cardiovasc Electrophysiol 2001, 12:1074-1077.
30. Schwartz PJ, Priori SG, Cerrone M, et al.: Left cardiac sympathetic denervation in the management of high-risk patients affected by the long-QT syndrome. Circulation 2004, 109:1826-1833.
31. Moss AJ, Zareba W: Long QT syndrome: therapeutic considerations. In Cardiac Electrophysiology: From Cell to Bedside, edn 4. Edited by Zipes DP, Jalife J. Philadelphia: WB Saunders; 2004:660-667.
32. Li C, Hu D, Shang L, et al.: Surgical left cardiac sympathetic denervation for long QT syndrome: effects on QT interval and heart rate. Heart Vessels 2005, 20:137-141.
33. Wang L, Feng G: Left cardiac sympathetic denervation as the first-line therapy for congenital long QT syndrome. Med Hypotheses 2004, 63:438-441.
34. Viskin S, Glikson M, Fish R, et al.: Rate smoothing with cardiac pacing for preventing torsade de pointes. Am J Cardiol 2000, 86:111K-115K.
35. Dorostkar PC, Eldar M, Belhassen B, Scheinman MM: Long-term follow-up of patients with long-QT syndrome treated with beta-blockers and continuous pacing. Circulation 1999, 100:2431-2436.
36. Gregoratos G, Abrams J, Epstein AE, et al.: ACC/AHA/NASPE 2002 guideline update for implantation of cardiac pacemakers and antiarrhythmia devices: summary artide. A report of the American College of Cardiology/ American Heart Association Task Force on Practice Guidelines (ACC/AHA/ NASPE Committee to Update the 1998 Pacemaker Guidelines). Circulation 2002, 106:2145-2161.
37. Pinski SL, Eguia LE, Trohman RG: What is the minimal pacing rate that prevents torsades de pointes? Insights from patients with permanent pacemakers. Pacing Clin Electrophysiol 2002, 25:1612-1615.
38. Etheridge SP, Compton SJ, Tristani-Firouzi M, Mason JW: A new oral therapy for long QT syndrome: long-term potassium improves repolarization in patients with HERG mutations. J Am Coll Cardiol 2003, 42:1777-1782.