Long QT Syndrome Questions & Answers

Updated: Nov 29, 2017
  • Author: Ali A Sovari, MD, FACP, FACC; Chief Editor: Mikhael F El-Chami, MD  more...
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Questions & Answers

Overview

What is long QT syndrome (LQTS)?

When is long QT syndrome (LQTS) typically diagnosed?

What physical findings are characteristic of long QT syndrome (LQTS)?

Which studies are included in the evaluation of suspected long QT syndrome (LQTS)?

What are the aims of treatment for long QT syndrome (LQTS)?

Which medications are used in the treatment of QT syndrome (LQTS)?

Which surgical interventions are used in the treatment of long QT syndrome (LQTS)?

What should be avoided by patients with long QT syndrome (LQTS)?

How is long QT syndrome (LQTS) characterized?

How is the QT interval measured in long QT syndrome (LQTS)?

What is the pathophysiology of long QT syndrome (LQTS)?

What is the pathophysiology of torsade de pointes in long QT syndrome (LQTS)?

What is the role of genetics in the etiology of long QT syndrome (LQTS)?

What is the role of the LQT1 gene in the etiology of long QT syndrome (LQTS)?

What is the role of the LQT2 gene in the etiology of long QT syndrome (LQTS)?

What is the role of the LQT3 gene in the etiology of long QT syndrome (LQTS)?

What is the role of the LQT4 gene in the etiology of long QT syndrome (LQTS)?

What is the role of the LQT5, 6, 7, 8, 9 and 10 genes in the etiology of long QT syndrome (LQTS)?

What is the role of the alpha-1-syntrophin gene in the etiology of long QT syndrome (LQTS)?

Which factors may precipitate an arrhythmic response in long QT syndrome (LQTS)?

What is the pathophysiology of drug-induced QT prolongation relative to long QT syndrome (LQTS)?

What is the prevalence of long QT syndrome (LQTS) in the US?

What is the global prevalence of long QT syndrome (LQTS)?

How does the prevalence of long QT syndrome (LQTS) vary by sex?

How does the prevalence of long QT syndrome (LQTS) vary by age?

What is the prognosis of long QT syndrome (LQTS)?

What is the morbidity and mortality associated with long QT syndrome (LQTS)?

What is included in patient education for long QT syndrome (LQTS)?

Presentation

Which clinical history is characteristic of long QT syndrome (LQTS)?

What are the physical findings characteristic of long QT syndrome (LQTS)?

DDX

Which conditions should be included in the differential diagnoses of long QT syndrome (LQTS)?

Workup

Which tests should be included in the workup of long QT syndrome (LQTS)?

What is the role of imaging studies in the workup of long QT syndrome (LQTS)?

What are the diagnostic criteria for long QT syndrome (LQTS)?

Which electrocardiographic (ECG) findings suggest long QT syndrome (LQTS)?

How is prolongation of the QTc interval defined in the workup of long QT syndrome (LQTS)?

What does a finding of bradycardia and tachycardia indicate in the workup of long QT syndrome (LQTS)?

What does a finding of T-wave alternans indicate in the workup of long QT syndrome (LQTS)?

What is the role of pharmacologic provocation in the workup of long QT syndrome (LQTS)?

What family member testing should be included in the workup of long QT syndrome (LQTS)?

What is the role of genetic testing in the workup of long QT syndrome (LQTS)?

How is a response to standing used in the workup of long QT syndrome (LQTS)?

Treatment

Which agents should be used with caution in patients with long QT syndrome (LQTS)?

Which specialist consultations are needed for the diagnosis and treatment of long QT syndrome (LQTS)?

When is inpatient care indicated for long QT syndrome (LQTS)?

How is asymptomatic long QT syndrome (LQTS) managed?

What is the role of beta-blockers in the treatment of long QT syndrome (LQTS)?

What is the role of pacemakers and implantable cardioverter-defibrillators (ICDs) in the treatment of long QT syndrome (LQTS)?

What is the role of stellectomy in the treatment of long QT syndrome (LQTS)?

What physical activity should be restricted in patients with long QT syndrome (LQTS)?

What is the role of gene-specific therapy for the treatment of long QT syndrome (LQTS)?

What local anesthetics and asthma medications should be avoided by patients with long QT syndrome (LQTS)?

Which antihistamines should be avoided by patients with long QT syndrome (LQTS)?

Which antibiotics should be avoided by patients with long QT syndrome (LQTS)?

Which heart medications should be avoided by patients with long QT syndrome (LQTS)?

Which GI medications should be avoided by patients with long QT syndrome (LQTS)?

Which antifungal drugs should be avoided by patients with long QT syndrome (LQTS)?

Which psychotropic drugs should be avoided by patients with long QT syndrome (LQTS)?

Which potassium loss medications should be avoided by patients with long QT syndrome (LQTS)?

Guidelines

What are the HRS/EHRA/APHRS diagnostic criteria for long QT syndrome (LQTS)?

What are the European Society of Cardiology (ESC) diagnostic guidelines for long QT syndrome (LQTS)?

What are the HRS/EHRA guidelines for genetic testing in long QT syndrome (LQTS)?

What are the ESC treatment guidelines for long QT syndrome (LQTS) and preventions of sudden cardiac death (SCD)?

What are r AHA/ACC guidelines for athletic competition by persons with long QT syndrome (LQTS)?

Medications

What are the treatment goals for long QT syndrome (LQTS)?

What is the efficacy of beta-blockers for the treatment of long QT syndrome (LQTS)?

Which medications in the drug class Beta-Adrenergic Blocking Agents are used in the treatment of Long QT Syndrome?