Korean Circ J.  2016 Nov;46(6):747-752. 10.4070/kcj.2016.46.6.747.

Management of Patients with Long QT Syndrome

Affiliations
  • 1Department of Internal Medicine, Kyungpook National Univeristy Hospital, Daegu, Korea. choyk@knu.ac.kr

Abstract

Long QT syndrome (LQTS) is a rare cardiac channelopathy associated with syncope and sudden death due to torsades de pointes and ventricular fibrillation. Syncope and sudden death are frequently associated with physical and emotional stress. Management of patients with LQTS consists of life-style modification, β-blockers, left cardiac sympathetic denervation (LCSD), and implantable cardioverter-defibrillator (ICD) implantation. Prohibition of competitive exercise and avoidance of QT-prolonging drugs are important issues in life-style modification. Although β-blockers are the primary treatment modality for patients with LQTS, these drugs are not completely effective in some patients. Lifelong ICD implantation in young and active patients is associated with significant complications. LCSD is a relatively simple and highly effective surgical procedure. However, LCSD is rarely used.

Keyword

Long QT syndrome; Adrenergic beta-blocker; Sympathectomy; Defibrillators, implantable

MeSH Terms

Channelopathies
Death, Sudden
Defibrillators, Implantable
Humans
Long QT Syndrome*
Stress, Psychological
Sympathectomy
Syncope
Torsades de Pointes
Ventricular Fibrillation

Figure

  • Fig. 1 Development of torsades de pointes in a young lady with long QT syndrome.

  • Fig. 2 Left cardiac sympathetic denervation is performed as a videoassisted thoracoscopic surgery.


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