eMedicine Specialties > Cardiology > Arrhythmias

Long QT Syndrome: Follow-up

Author: Ali A Sovari, MD, Clinical and Research Fellow in Cardiovascular Medicine, Section of Cardiology, University of Illinois at Chicago
Coauthor(s): Abraham G Kocheril, MD, FACC, FACP, Professor of Medicine, Director of Clinical Electrophysiology, University of Illinois at Chicago; Ramin Assadi, MD, Fellow, Department of Cardiology, Loma Linda University; Arnold S Baas, MD, FACC, FACP, Assistant Professor of Medicine, Division of Cardiology, University of California, Los Angeles School of Medicine; Attending Physician, UCLA Santa Monica Hospital and UCLA Westwood Hospital; Wojciech Zareba, MD, PhD, FACC, Associate Director of Heart Research, Associate Professor, Department of Medicine, Division of Cardiology, University of Rochester Medical Center; Spencer Rosero, MD, Assistant Professor, Department of Medicine, University of Rochester School of Medicine
Contributor Information and Disclosures

Updated: Jun 18, 2009

Follow-up

Further Inpatient Care

  • Patients with long QT syndrome (LQTS) are frequently hospitalized in a monitored unit after they have a cardiac event (eg, syncope, cardiac arrest) to enable immediate rescue if cardiac arrhythmias recur.
  • Asymptomatic individuals with LQTS usually do not require hospitalization. However, carefully evaluate them and provide follow-up care in an ambulatory setting.

Further Outpatient Care

A cardiologist or a cardiac electrophysiologist should examine patients with long QT syndrome on a regular basis.

Deterrence/Prevention

  • Antiadrenergic therapy (eg, beta-blockers, stellectomy) aims to prevent future cardiac events.
  • Use implantable cardioverter-defibrillators (ICDs) to prevent sudden cardiac death in patients with long QT syndrome (LQTS) who develop ventricular tachyarrhythmias.
  • Educate family members of patients with LQTS regarding the disorder and the basics of cardiopulmonary resuscitation (CPR). The Sudden Arrhythmia Death Syndromes Foundation (SADS) and Cardiac Arrhythmias Research and Education Foundation (CARE) have support groups for families with LQTS.
  • Educate patients and family members about medications that may induce QT prolongation and that should be avoided in patients with LQTS. ArizonaCERT provides lists of Drugs that Prolong the Qt Interval and/or Induce Torsades de Pointes Ventricular Arrhythmia.
    • Anesthetics or asthma medication - Epinephrine (Adrenaline) for local anesthesia or as an asthma medication
    • Antihistamines
      • Terfenadine (Seldane [recalled from US market]) for allergies
      • Astemizole (Hismanal [recalled from US market]) for allergies
      • Diphenhydramine (Benadryl) for allergies
    • Antibiotics
      • Erythromycin (E-Mycin, EES, EryPed, PCE) for lung, ear, and throat infections
      • Trimethoprim and sulfamethoxazole (Bactrim, Septra) for urinary, ear, and lung infections
      • Pentamidine (Pentam, intravenous) for lung infections
    • Heart medications
      • Quinidine (Quinidine, Quinidex, Duraquin, Quinaglute) for heart rhythm abnormalities
      • Procainamide (Pronestyl) for heart rhythm abnormalities
      • Disopyramide (Norpace) for heart rhythm abnormalities
      • Sotalol (Betapace) for heart rhythm abnormalities
      • Probucol (Lorelco) for high triglycerides, cholesterol
      • Bepridil (Vascor) for chest pain (angina)
      • Dofetilide (Tikosyn) for atrial fibrillation
      • Ibutilide (Corvert) for atrial fibrillation
    • Gastrointestinal medications - Cisapride (Propulsid) for esophageal reflux, acid indigestion
    • Antifungal drugs
      • Ketoconazole (Nizoral) for fungal infections
      • Fluconazole (Diflucan) for fungal infections
      • Itraconazole (Sporanox) for fungal infections
    • Psychotropic drugs
      • Tricyclic antidepressants (Elavil, Norpramin, Vivactil) for depression
      • Phenothiazine derivatives (Compazine, Stelazine, Thorazine, Mellaril, Trilafon) for mental disorders
      • Butyrophenones (Haloperidol) for mental disorders
      • Benzisoxazol (Risperdal) for mental disorders
      • Diphenylbutylperidine (Orap) for mental disorders
    • Medications for potassium loss
      • Indapamide (Lozol) for water loss, edema
      • Other diuretics
      • Medications for vomiting and diarrhea

Complications

  • Sudden cardiac death is the most devastating complication of the long QT syndrome, especially because it frequently occurs in young individuals.
  • Neurologic deficits after aborted cardiac arrest may complicate the clinical course after successful resuscitation.

Prognosis

  • The prognosis for patients with long QT syndrome (LQTS) treated with beta-blockers (and other therapeutic measures if needed) is good overall. Fortunately, episodes of torsade de pointes are usually self-terminating in patients with LQTS; only about 4-5% of cardiac events are fatal.
  • Patients at high risk (ie, those with aborted cardiac arrest or recurrent cardiac events despite beta-blocker therapy) have a markedly increased risk of sudden death. Treat these patients with ICDs. Their prognosis after implantation of cardioverter-defibrillators is good.

Patient Education

  • Educate patients regarding the nature of the long QT syndrome and factors that trigger cardiac events. Patients should avoid sudden noises (eg, from an alarm clock), strenuous exercise, water activities, and other arousal factors.
  • Educate patients and family members about the critical importance of systematic treatment with beta-blockers.
  • Advise family members and the patient's teachers at school to undergo training in CPR.

Miscellaneous

Medicolegal Pitfalls

  • Failure to diagnose long QT syndrome (LQTS) when the QTc clearly is prolonged
  • Failure to treat patients with LQTS with beta-blockers, unless contraindicated
  • Failure to educate patients and family members about potential risks associated with strenuous sport activities, interruption of beta-blocker therapy, and drugs that prolong the QT interval.
  • Failure to screen for LQTS in family members of proband (ie, the first family member with the disease)

Special Concerns

Pregnancy is not associated with an increased incidence of cardiac events, whereas the postpartum period is associated with substantially increased risk of cardiac events, especially in the subset of patients with LQT2.

 


More on Long QT Syndrome

Overview: Long QT Syndrome
Differential Diagnoses & Workup: Long QT Syndrome
Treatment & Medication: Long QT Syndrome
Follow-up: Long QT Syndrome
Multimedia: Long QT Syndrome
References
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References

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Further Reading

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Keywords

long QT syndrome, LQTS, congenital long QT syndrome, Romano-Ward syndrome, Jervell and Lange-Nielsen syndrome, JLN syndrome, ventricular tachyarrhythmias, syncope, cardiac arrest, sudden death, Anderson syndrome, Timothy syndrome

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Contributor Information and Disclosures

Author

Ali A Sovari, MD, Clinical and Research Fellow in Cardiovascular Medicine, Section of Cardiology, University of Illinois at Chicago
Ali A Sovari, MD is a member of the following medical societies: American College of Cardiology, American College of Physicians, American Heart Association, and American Medical Association
Disclosure: Nothing to disclose.

Coauthor(s)

Abraham G Kocheril, MD, FACC, FACP, Professor of Medicine, Director of Clinical Electrophysiology, University of Illinois at Chicago
Abraham G Kocheril, MD, FACC, FACP is a member of the following medical societies: American College of Cardiology, American College of Physicians, American Heart Association, American Medical Association, Cardiac Electrophysiology Society, Central Society for Clinical Research, Heart Failure Society of America, and Illinois State Medical Society
Disclosure: Nothing to disclose.

Ramin Assadi, MD, Fellow, Department of Cardiology, Loma Linda University
Ramin Assadi, MD is a member of the following medical societies: American College of Cardiology, American College of Physicians, and American Medical Association
Disclosure: Nothing to disclose.

Arnold S Baas, MD, FACC, FACP, Assistant Professor of Medicine, Division of Cardiology, University of California, Los Angeles School of Medicine; Attending Physician, UCLA Santa Monica Hospital and UCLA Westwood Hospital
Arnold S Baas, MD, FACC, FACP is a member of the following medical societies: American College of Cardiology, American College of Physicians, American Federation for Medical Research, and American Society of Echocardiography
Disclosure: Pfizer Honoraria Speaking and teaching

Wojciech Zareba, MD, PhD, FACC, Associate Director of Heart Research, Associate Professor, Department of Medicine, Division of Cardiology, University of Rochester Medical Center
Wojciech Zareba, MD, PhD, FACC is a member of the following medical societies: American College of Cardiology, American Heart Association, European Society of Cardiology, International Society for Holter and Noninvasive Electrocardiology, International Society of Electrocardiology, and Polish Society of Cardiology
Disclosure: Nothing to disclose.

Spencer Rosero, MD, Assistant Professor, Department of Medicine, University of Rochester School of Medicine
Spencer Rosero, MD is a member of the following medical societies: American College of Cardiology
Disclosure: Nothing to disclose.

Medical Editor

Justin D Pearlman, MD, PhD, ME, MA, Director of Advanced Cardiovascular Imaging, Professor of Medicine, Professor of Radiology, Adjunct Professor, Thayer Bioengineering and Computer Science, Dartmouth-Hitchcock Medical Center
Justin D Pearlman, MD, PhD, ME, 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.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: eMedicine Salary Employment

Managing Editor

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; Reliant Grant/research funds Other; Novartis Honoraria Speaking and teaching; Novartis Consulting fee Consulting

CME Editor

Amer Suleman, MD, Consultant in Electrophysiology and Cardiovascular Medicine, Department of Internal Medicine, Division of Cardiology, Medical City Dallas Hospital
Amer Suleman, MD is a member of the following medical societies: American College of Physicians, American Heart Association, American Institute of Stress, American Society of Hypertension, Federation of American Societies for Experimental Biology, Royal Society of Medicine, and Society of Cardiac Angiography and Interventions
Disclosure: Nothing to disclose.

Chief Editor

Jeffrey N Rottman, MD, Professor of Medicine and Pharmacology, Director, Clinical Cardiac Electrophysiology Fellowship Program, Vanderbilt University School of Medicine; Chief, Department of Cardiology, Nashville Veterans Affairs Medical Center
Jeffrey N Rottman, MD is a member of the following medical societies: American Heart Association and North American Society of Pacing and Electrophysiology (NASPE)
Disclosure: Nothing to disclose.

 
 
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