Long QT syndrome - Symptoms, Causes, Images, and Treatment Options

Diseases

Highlights & Basics

Key Highlights

Long QT syndrome (LQTS) is characterized by a prolonged QT interval on ECG, which may be congenital or acquired.
In congenital LQTS, genetic mutations affect ion channels important in myocardial repolarization.
Acquired LQTS may occur secondary to ingestion of QT interval-prolonging drugs, electrolyte imbalances, or bradyarrhythmias.
Patients with LQTS are at increased risk of syncope, ventricular arrhythmias (including torsades de pointes), and sudden cardiac death.
Unless there is an identifiable reversible cause, treatment primarily involves lifestyle modification and beta-blocker therapy with the implantation of a cardioverter-defibrillator in selected cases.

ECG findings in type 1 long QT syndrome

From the collection of Dr James P. Daubert

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History & Exam
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Definition

Epidemiology

Etiology

Pathophysiology

Images

ECG findings in type 1 long QT syndrome
ECG findings in type 2 long QT syndrome
ECG findings in type 3 long QT syndrome
ECG showing QT prolongation (QTc = 519 ms)
ECG showing a corrected QTc interval of 760 ms
ECG findings in type 1 long QT syndrome
ECG findings in type 2 long QT syndrome
ECG findings in type 3 long QT syndrome
Rhythm strips showing torsades de pointes

Citations

Key Articles

Zeppenfeld K, Tfelt-Hansen J, de Riva M, et al. 2022 ESC guidelines for the management of patients with ventricular arrhythmias and the prevention of sudden cardiac death. Eur Heart J. 2022 Oct 21;43(40):3997-4126.[Abstract][Full Text]
Priori SG, Wilde AA, Horie M, et al. HRS/EHRA/APHRS expert consensus statement on the diagnosis and management of patients with inherited primary arrhythmia syndromes. Heart Rhythm. 2013 Dec;10(12):1932-63.[Abstract][Full Text]
Wilde AAM, Semsarian C, Márquez MF, et al. European Heart Rhythm Association (EHRA)/Heart Rhythm Society (HRS)/Asia Pacific Heart Rhythm Society (APHRS)/Latin American Heart Rhythm Society (LAHRS) expert consensus statement on the state of genetic testing for cardiac diseases. Europace. 2022 Sep 1;24(8):1307-67.[Abstract][Full Text]
Roden DM. Clinical practice. Long-QT syndrome. N Engl J Med. 2008 Jan 10;358(2):169-76.[Abstract]
Al-Khatib SM, Stevenson WG, Ackerman MJ, et al. 2017 AHA/ACC/HRS guideline 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 on clinical practice guidelines and the Heart Rhythm Society. Circulation. 2018 Sep 25;138(13):e272-391.[Abstract][Full Text]
Sauer AJ, Moss AJ, McNitt S, et al. Long QT syndrome in adults. J Am Coll Cardiol. 2007 Jan 23;49(3):329-37.[Abstract]
Schwartz PJ, Moss AJ, Vincent GM, et al. Diagnostic criteria for the long QT syndrome. An update. Circulation. 1993 Aug;88(2):782-4.[Abstract]
Schwartz PJ, Crotti L. QTc behavior during exercise and genetic testing for the long-QT syndrome. Circulation. 2011 Nov 15;124(20):2181-4.[Abstract][Full Text]

Other Online Resources

Credible Meds (Arizona CERT): drugs that prolong the QT interval

Referenced Articles

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2. Zeppenfeld K, Tfelt-Hansen J, de Riva M, et al. 2022 ESC guidelines for the management of patients with ventricular arrhythmias and the prevention of sudden cardiac death. Eur Heart J. 2022 Oct 21;43(40):3997-4126.[Abstract][Full Text]
3. Priori SG, Wilde AA, Horie M, et al. HRS/EHRA/APHRS expert consensus statement on the diagnosis and management of patients with inherited primary arrhythmia syndromes. Heart Rhythm. 2013 Dec;10(12):1932-63.[Abstract][Full Text]
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26. El-Sherif N, Turitto G. Electrolyte disorders and arrhythmogenesis. Cardiol J. 2011;18(3):233-45.[Abstract]
27. Al-Khatib SM, Stevenson WG, Ackerman MJ, et al. 2017 AHA/ACC/HRS guideline 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 on clinical practice guidelines and the Heart Rhythm Society. Circulation. 2018 Sep 25;138(13):e272-391.[Abstract][Full Text]
28. Sauer AJ, Moss AJ, McNitt S, et al. Long QT syndrome in adults. J Am Coll Cardiol. 2007 Jan 23;49(3):329-37.[Abstract]
29. Priori SG, Schwartz PJ, Napolitano C, et al. Risk stratification in the long-QT syndrome. N Engl J Med. 2003 May 8;348(19):1866-74.[Abstract][Full Text]
30. Migdalovich D, Moss AJ, Lopes CM, et al. Mutation and gender-specific risk in type 2 long QT syndrome: implications for risk stratification for life-threatening cardiac events in patients with long QT syndrome. Heart Rhythm. 2011 Oct;8(10):1537-43.[Abstract]
31. Goldenberg I, Horr S, Moss AJ, et al. Risk for life-threatening cardiac events in patients with genotype-confirmed long-QT syndrome and normal-range corrected QT intervals. J Am Coll Cardiol. 2011 Jan 4;57(1):51-9.[Abstract]
32. Schwartz PJ, Moss AJ, Vincent GM, et al. Diagnostic criteria for the long QT syndrome. An update. Circulation. 1993 Aug;88(2):782-4.[Abstract]
33. Schwartz PJ, Crotti L. QTc behavior during exercise and genetic testing for the long-QT syndrome. Circulation. 2011 Nov 15;124(20):2181-4.[Abstract][Full Text]
34. Schwartz PJ, Priori SG, Spazzolini C, et al. Genotype-phenotype correlation in the long-QT syndrome: gene-specific triggers for life-threatening arrhythmias. Circulation. 2001 Jan 2;103(1):89-95.[Abstract]
35. Seth R, Moss AJ, McNitt S, et al. Long QT syndrome and pregnancy. J Am Coll Cardiol. 2007 Mar 13;49(10):1092-8.[Abstract]
36. Landstrom AP, Chahal AA, Ackerman MJ, et al. Interpreting incidentally identified variants in genes associated with heritable cardiovascular disease: a scientific statement from the American Heart Association. Circ Genom Precis Med. 2023 Apr;16(2):e000092.[Abstract][Full Text]
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39. Page A, Aktas MK, Soyata T, et al. "QT clock" to improve detection of QT prolongation in long QT syndrome patients. Heart Rhythm. 2016 Jan;13(1):190-98.[Abstract][Full Text]
40. Wilde AAM, Amin AS. Clinical spectrum of SCN5A mutations: long QT syndrome, Brugada syndrome, and cardiomyopathy. JACC Clin Electrophysiol. 2018 May;4(5):569-79.[Abstract][Full Text]
41. Musunuru K, Hershberger RE, Day SM, et al. Genetic testing for inherited cardiovascular diseases: a scientific statement from the American Heart Association. Circ Genom Precis Med. 2020 Aug;13(4):e000067.[Abstract][Full Text]
42. Ackerman MJ, Priori SG, Willems S, et al. HRS/EHRA expert consensus statement on the state of genetic testing for the channelopathies and cardiomyopathies. Heart Rhythm. 2011 Aug;8(8):1308-39.[Abstract]
43. Rohatgi RK, Sugrue A, Bos JM, et al. Contemporary outcomes in patients with long QT syndrome. J Am Coll Cardiol. 2017 Jul 25;70(4):453-62.[Abstract]
44. Vyas H, Hejlik J, Ackerman MJ. Epinephrine QT stress testing in the evaluation of congenital long-QT syndrome: diagnostic accuracy of the paradoxical QT response. Circulation. 2006 Mar 21;113(11):1385-92.[Abstract][Full Text]
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47. Quaglini S, Rognoni C, Spazzolini C, et al. Cost-effectiveness of neonatal ECG screening for the long QT syndrome. Eur Heart J. 2006 Aug;27(15):1824-32.[Abstract][Full Text]
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52. Wilde AA, Moss AJ, Kaufman ES, et al. Clinical aspects of type 3 long-QT syndrome: an international multicenter study. Circulation. 2016 Sep 20;134(12):872-82.[Abstract][Full Text]
53. Hobbs JB, Peterson DR, Moss AJ, et al. Risk of aborted cardiac arrest or sudden cardiac death during adolescence in the long-QT syndrome. JAMA. 2006 Sep 13;296(10):1249-54.[Abstract][Full Text]
54. Mazzanti A, Maragna R, Faragli A, et al. Gene-specific therapy with mexiletine reduces arrhythmic events in patients with long QT syndrome type 3. J Am Coll Cardiol. 2016 Mar 8;67(9):1053-8.[Abstract][Full Text]
55. Bos JM, Crotti L, Rohatgi RK, et al. Mexiletine shortens the QT interval in patients with potassium channel-mediated type 2 long QT syndrome. Circ Arrhythm Electrophysiol. 2019 May;12(5):e007280.[Abstract][Full Text]
56. Zareba W, Moss AJ, Daubert JP, et al. Implantable cardioverter defibrillator in high-risk long QT syndrome patients. J Cardiovasc Electrophysiol. 2003 Apr;14(4):337-41.[Abstract]
57. Garratt CJ, Elliott P, Behr E, et al. Heart Rhythm UK position statement on clinical indications for implantable cardioverter defibrillators in adult patients with familial sudden cardiac death syndromes. Europace. 2010 Aug;12(8):1156-75.[Abstract][Full Text]
58. Biton Y, Rosero S, Moss AJ, et al. Primary prevention with the implantable cardioverter-defibrillator in high-risk long-QT syndrome patients. Europace. 2019 Feb 1;21(2):339-46.[Abstract][Full Text]
59. 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 Apr 20;109(15):1826-33.[Abstract][Full Text]
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