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Medication

Medication Summary

No treatment addresses the cause of long QT syndrome (LQTS). Antiadrenergic therapeutic measures (eg, use of beta-blockers, left cervicothoracic stellectomy) and device therapy (eg, use of pacemakers, implantable cardioverter-defibrillators [ICD]s) aim to decrease the risk and lethality of cardiac events.

As previously mentioned, the protective effect of beta-blockers is related to their adrenergic blockade, which diminishes the risk of cardiac arrhythmias. They may also reduce the QT interval in some patients. Beta-blockers used in patients with LQTS include the following:

Nadolol (preferred)
Propranolol
Metoprolol
Atenolol

Nadolol is the preferred beta blocker to be used at a dose of 1-1.5 mg/kg/day (once a day for patients older than 12 years and divided twice a day for younger patients).^[3]

Class Summary

Antiadrenergic therapy effectively protects most patients with long QT syndrome (LQTS). Beta-blockers, especially propranolol, are the drugs most frequently used in patients with LQTS. Inform patients and their family members that beta-blockers should be continued indefinitely. Interruption in beta-blocker therapy may increase the risk of cardiac events.

Propranolol (Inderal LA, Inderal XL, InnoPran XL, Hemangeol)

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Propranolol decreases the effect of sympathetic stimulation on the heart. It decreases conduction through the atrioventricular (AV) node and has negative chronotropic and inotropic effects. Consult a cardiologist because dosing varies and is individualized in patients with LQTS. Patients with asthma should use cardioselective beta-blockers. Patients with LQTS who cannot take beta-blockers may require an ICD as first-line therapy.

Nadolol (Corgard)

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Nadolol is frequently prescribed because of its long-term effect. This agent decreases the effect of sympathetic stimulation on the heart. Nadolol decreases conduction through the AV node and has negative chronotropic and inotropic effects. Consult a cardiologist because dosing varies and is individualized in patients with LQTS. Patients with asthma should use cardioselective beta-blockers. Patients with LQTS who cannot take beta-blockers may require an ICD as first-line therapy.

Metoprolol (Lopressor, Toprol XL)

Metoprolol is a selective beta1-adrenergic receptor blocker that decreases the automaticity of contractions. During intravenous (IV) administration, carefully monitor blood pressure, heart rate, and ECG. Consult a cardiologist because dosing varies and is individualized in patients with LQTS. Patients with LQTS who cannot take beta-blockers may require an ICD as first-line therapy.

Atenolol (Tenormin)

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Atenolol selectively blocks beta1-receptors with little or no effect on beta2 types. Consult a cardiologist because dosing varies and is individualized in patients with LQTS. Patients with LQTS who cannot take beta-blockers may require an ICD as first-line therapy.

Questions

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Media Gallery

Marked prolongation of QT interval in a 15-year-old male adolescent with long QT syndrome (LQTS) (R-R = 1.00 s, QT interval = 0.56 s, QT interval corrected for heart rate [QTc] = 0.56 s). Abnormal morphology of repolarization can be observed in almost every lead (ie, peaked T waves, bowing ST segment). Bradycardia is a common feature in patients with LQTS.
Genetically confirmed long QT syndrome (LQTS) with borderline values of QT corrected for heart rate (QTc) duration (R-R = 0.68 s, QT interval = 0.36 s, QT interval corrected for heart rate [QTc] = 0.44 s) in a 12-year-old girl. Note the abnormal morphology of the T wave (notches) in leads V2-V4.
ECG of a 13-year-old female who had a syncopal event while running to a school bus. She awoke after a few seconds, and her subsequent clinical course was uneventful.

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Table 1. Genetic Background of Inherited Forms of LQTS (Romano-Ward Syndrome: LQT1-6, Anderson Syndrome: LQT7, Timothy Syndrome: LQT8, and Jervell and Lang-Nielsen Syndrome: JLN1-2)

Type of LQTS	Chromosomal Locus	Mutated Gene	Ion Current Affected
LQT1	11p15.5	KVLQT1 or KCNQ1 (heterozygotes)	Potassium (I_Ks)
LQT2	7q35-36	HERG, KCNH2	Potassium (I_Kr)
LQT3	3p21-24	SCN5A	Sodium (I_Na)
LQT4	4q25-27	ANK2, ANKB	Sodium, potassium and calcium
LQT5	21q22.1-22.2	KCNE1 (heterozygotes)	Potassium (I_Ks)
LQT6	21q22.1-22.2	MiRP1, KNCE2	Potassium (I_Kr)
LQT7 (Anderson syndrome)	17q23.1-q24.2	KCNJ2	Potassium (I_K1)
LQT8 (Timothy syndrome)	12q13.3	CACNA1C	Calcium (I_Ca-Lalpha)
LQT9	3p25.3	CAV3	Sodium (I_Na)
LQT10	11q23.3	SCN4B	Sodium (I_Na)
LQT11	7q21-q22	AKAP9	Potassium (I_Ks)
LQT12		SNTAI	Sodium (I_Na)
JLN1	11p15.5	KVLQT1 or KCNQ1 (homozygotes)	Potassium (I_Ks)
JLN2	21q22.1-22.2	KCNE1 (homozygotes)	Potassium (I_Ks)

Table 2. Diagnostic Criteria for LQTS

Criterion	Points
Electrocardiogram findings ^*
QTc, ms^†	>480	3
	460-469	2
	450-459 in male patient	1
Torsade de pointes^‡		2
T-wave alternans		1
Notched T wave in 3 leads		1
Low heart rate for age^§		0.5
Clinical history
Syncope^║	With stress	2
	Without stress	1
Congenital deafness		0.5
Family history ^¶
A. Family members with definite LQTS^#		1
B. Unexplained sudden cardiac death at age <30 years in an immediate family member		0.5
LQTS = long QT syndrome. ^*In the absence of medications or disorders known to affect these electrocardiographic features. ^†QTc calculated by Bazett's formula. ^‡Mutually exclusive. ^§Resting heart rate below the second percentile for age. ^\|\|Mutually exclusive. ^¶The same family member cannot be counted in both A and B. ^#Definite LQTS is defined by an LQTS score above 3 (≥4).

Table 3. Definition of QTc Based on Age- and Sex-Specific Criteria

Group	Prolonged QTc, sec	Borderline QTc, sec	Reference Range, sec
Children and adolescents (<15 years)	>0.46	0.44-0.46	<0.44
Men	>0.45	0.43-0.45	<0.43
Women	>0.46	0.45-0.46	<0.45

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Author

Ali A Sovari, MD, FACP, FACC Attending Physician, Cardiac Electrophysiologist, Cedars Sinai Medical Center and St John's Regional Medical Center

Ali A Sovari, MD, FACP, FACC is a member of the following medical societies: American College of Cardiology, American College of Physicians, American Physician Scientists Association, American Physiological Society, Biophysical Society, Heart Rhythm Society, Society for Cardiovascular Magnetic Resonance

Disclosure: Nothing to disclose.

Coauthor(s)

Abraham G Kocheril, MD, FACC, FACP, FHRS Professor of Medicine, University of Illinois College of Medicine

Abraham G Kocheril, MD, FACC, FACP, FHRS is a member of the following medical societies: American College of Cardiology, Central Society for Clinical and Translational Research, Heart Failure Society of America, Cardiac Electrophysiology Society, American College of Physicians, American Heart Association, American Medical Association, Illinois State Medical Society

Disclosure: Nothing to disclose.

Ramin Assadi, MD Assistant Professor of Medicine, Division of Interventional Cardiology, University of California, Los Angeles, David Geffen School of Medicine

Ramin Assadi, MD is a member of the following medical societies: American College of Cardiology, American College of Physicians, American Medical Association, Society for Cardiovascular Angiography and Interventions

Disclosure: Nothing to disclose.

Arnold S Baas, MD, FACC, FACP Professor of Medicine, Division of Cardiology, Fellowship Director for Advanced Heart Failure and Transplant Cardiology, Ahmanson UCLA Cardiomyopathy Center, Mechanical Circulatory Support, and Heart Transplant Program, University of California, Los Angeles, David Geffen School of Medicine; Attending Physician, Ronald Reagan UCLA Medical Center

Arnold S Baas, MD, FACC, FACP is a member of the following medical societies: American College of Cardiology, American College of Physicians, American Society of Echocardiography, International Society for Heart and Lung Transplantation

Disclosure: Nothing to disclose.

Chief Editor

Mikhael F El-Chami, MD Associate Professor, Department of Medicine, Division of Cardiology, Section of Electrophysiology, Emory University School of Medicine

Mikhael F El-Chami, MD is a member of the following medical societies: Alpha Omega Alpha, American College of Cardiology, American Heart Association, Heart Rhythm Society

Disclosure: Serve(d) as a director, officer, partner, employee, advisor, consultant or trustee for: Medtronic; Boston Scientific, Biotronik<br/>Received research grants (as part of Multicenter studies) from Medtronic and Boston Scientific.

Acknowledgements

Brian Olshansky, MD Professor of Medicine, Department of Internal Medicine, University of Iowa College of Medicine

Brian Olshansky, MD is a member of the following medical societies: American Autonomic Society, American College of Cardiology, American College of Chest Physicians, American College of Physicians, American College of Sports Medicine, American Federation for Clinical Research, American Heart Association, Cardiac Electrophysiology Society, Heart Rhythm Society, and New York Academy of Sciences

Disclosure: Guidant/Boston Scientific Honoraria Speaking and teaching; Medtronic Honoraria Speaking and teaching; Guidant/Boston Scientific Consulting fee Consulting; Novartis Honoraria Speaking and teaching; Novartis Consulting fee Consulting

Justin D Pearlman, MD, ME, PhD, FACC, MA Chief, Division of Cardiology, Director of Cardiology Consultative Service, Director of Cardiology Clinic Service, Director of Cardiology Non-Invasive Laboratory, Director of Cardiology Quality Program KMC, Dartmouth-Hitchcock Medical Center, Dartmouth Medical School

Justin D Pearlman, MD, ME, PhD, FACC, MA is a member of the following medical societies: American College of Cardiology, American College of Physicians, American Federation for Medical Research, International Society for Magnetic Resonance in Medicine, and Radiological Society of North America

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Salary Employment

Long QT Syndrome Medication

Medication Summary

Beta-Adrenergic Blocking Agents

Class Summary

Propranolol (Inderal LA, Inderal XL, InnoPran XL, Hemangeol)

Propranolol (Inderal LA, Inderal XL, InnoPran XL, Hemangeol)

Nadolol (Corgard)

Nadolol (Corgard)

Metoprolol (Lopressor, Toprol XL)

Atenolol (Tenormin)

Atenolol (Tenormin)

Long QT Syndrome Medication

Medication Summary

Beta-Adrenergic Blocking Agents

Class Summary

Propranolol (Inderal LA, Inderal XL, InnoPran XL, Hemangeol)

Propranolol (Inderal LA, Inderal XL, InnoPran XL, Hemangeol)

Nadolol (Corgard)

Nadolol (Corgard)

Metoprolol (Lopressor, Toprol XL)

Atenolol (Tenormin)

Atenolol (Tenormin)

References

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