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Long QT Syndrome, Pediatric

Akash R. Patel, MD, CEPS, FHRS Reviewed 10/2018
 


BASICS

DESCRIPTION

Long QT syndrome (LQTS) is characterized by prolongation of the QT interval on the surface electrocardiogram (EKG) that can result in symptomatic ventricular arrhythmias. The symptom...

DIAGNOSIS

HISTORY

  • Notable findings include the following:

    • Palpitations

    • Presyncope

    • Syncope

    • Cardiac arrest

  • These symptoms may be a result of provocative stimuli, especially emotional or physical stress.

  • Concom...

TREATMENT

GENERAL MEASURES

  • Patients are usually treated based on symptoms and the clinical severity of the disease; avoidance of QT-prolonging medications and avoidance of provoking situations

  • Automated...

ONGOING CARE

FOLLOW-UP RECOMMENDATIONS

Patient Monitoring

  • Follow-up outpatient appointments should review new or recurrent symptoms, including palpitations, near syncope or syncope, and the efficacy and...

ADDITIONAL READING

  • Ackerman MJ. Genotype-phenotype relationships in congenital long QT syndrome. J Electrocardiol.  2005;38(Suppl 4):64–68. [View Abstract on OvidInsights]

  • Hedley PL, Jørgensen P, ...

CODES

ICD9

426.82 Long QT syndrome 

ICD10

I45.81 Long QT syndrome 

SNOMED

  • 9651007 Long QT syndrome (disorder)

  • 442917000 Congenital long QT syndrome

  • 442946007 Aquired long QT syndrome

FAQ

  • Q: Should activity be restricted in patients with congenital LQTS?

  • A: Because sudden rises in serum catecholamine levels may precipitate symptoms, it is appropriate to initially restrict competitiv...

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