Contemporary Outcomes in Patients With Long QT Syndrome

Ram K. Rohatgi, Alan Sugrue, J. Martijn Bos, Bryan C. Cannon, Samuel J Asirvatham, Christopher Moir, Heidi J. Owen, Katy M. Bos, Teresa Kruisselbrink, Michael John Ackerman

Research output: Contribution to journalArticle

21 Citations (Scopus)

Abstract

BACKGROUND: Long QT syndrome (LQTS) is a potentially lethal cardiac channelopathy with a 1% to 5% annual risk of LQTS-triggered syncope, aborted cardiac arrest, or sudden cardiac death.

OBJECTIVES: This study sought to evaluate LQTS outcomes from a single center in the contemporary era.

METHODS: The authors conducted a retrospective study comprising the 606 patients with LQTS (LQT1 in 47%, LQT2 in 34%, and LQT3 in 9%) who were evaluated in Mayo Clinic's Genetic Heart Rhythm Clinic from January 1999 to December 2015. Breakthrough cardiac events (BCEs) were defined as LQTS-attributable syncope or seizures, aborted cardiac arrest, appropriate ventricular fibrillation-terminating implantable cardioverter-defibrillator shocks, and sudden cardiac death.

RESULTS: There were 166 (27%) patients who were symptomatic prior to their first Mayo Clinic evaluation. Median age at first symptom was 12 years. Treatment strategies included no active therapy in 47 (8%) patients, beta-blockers alone in 350 (58%) patients, implantable cardioverter-defibrillators alone in 25 (4%) patients, left cardiac sympathetic denervation alone in 18 (3%) patients, and combination therapy in 166 (27%) patients. Over a median follow-up of 6.7 (IQR: 3.9 to 9.8) years, 556 (92%) patients have not experienced an LQTS-triggered BCE. Only 8 of 440 (2%) previously asymptomatic patients have experienced a single BCE. In contrast, 42 of 166 (25%) previously symptomatic patients have experienced ≥1 BCE. Among the 30 patients with ≥2 BCEs, 2 patients have died and 3 LQT3 patients underwent cardiac transplantation.

CONCLUSIONS: Although outcomes have improved markedly, further optimization of treatment strategies is still needed given that 1 in 4 previously symptomatic patients experienced at least 1 subsequent, albeit nonlethal, LQTS-triggered cardiac event.

Original languageEnglish (US)
Pages (from-to)453-462
Number of pages10
JournalJournal of the American College of Cardiology
Volume70
Issue number4
DOIs
StatePublished - Jul 25 2017

Fingerprint

Long QT Syndrome
Implantable Defibrillators
Sudden Cardiac Death
Syncope
Heart Arrest
Channelopathies
Sympathectomy
Ventricular Fibrillation
Heart Transplantation
Therapeutics
Shock
Seizures
Retrospective Studies

Keywords

  • breakthrough cardiac events
  • genetics
  • long QT syndrome
  • LQTS
  • outcomes

ASJC Scopus subject areas

  • Cardiology and Cardiovascular Medicine

Cite this

Contemporary Outcomes in Patients With Long QT Syndrome. / Rohatgi, Ram K.; Sugrue, Alan; Bos, J. Martijn; Cannon, Bryan C.; Asirvatham, Samuel J; Moir, Christopher; Owen, Heidi J.; Bos, Katy M.; Kruisselbrink, Teresa; Ackerman, Michael John.

In: Journal of the American College of Cardiology, Vol. 70, No. 4, 25.07.2017, p. 453-462.

Research output: Contribution to journalArticle

Rohatgi, RK, Sugrue, A, Bos, JM, Cannon, BC, Asirvatham, SJ, Moir, C, Owen, HJ, Bos, KM, Kruisselbrink, T & Ackerman, MJ 2017, 'Contemporary Outcomes in Patients With Long QT Syndrome', Journal of the American College of Cardiology, vol. 70, no. 4, pp. 453-462. https://doi.org/10.1016/j.jacc.2017.05.046
Rohatgi, Ram K. ; Sugrue, Alan ; Bos, J. Martijn ; Cannon, Bryan C. ; Asirvatham, Samuel J ; Moir, Christopher ; Owen, Heidi J. ; Bos, Katy M. ; Kruisselbrink, Teresa ; Ackerman, Michael John. / Contemporary Outcomes in Patients With Long QT Syndrome. In: Journal of the American College of Cardiology. 2017 ; Vol. 70, No. 4. pp. 453-462.
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AU - Cannon, Bryan C.

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AU - Moir, Christopher

AU - Owen, Heidi J.

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N2 - BACKGROUND: Long QT syndrome (LQTS) is a potentially lethal cardiac channelopathy with a 1% to 5% annual risk of LQTS-triggered syncope, aborted cardiac arrest, or sudden cardiac death.OBJECTIVES: This study sought to evaluate LQTS outcomes from a single center in the contemporary era.METHODS: The authors conducted a retrospective study comprising the 606 patients with LQTS (LQT1 in 47%, LQT2 in 34%, and LQT3 in 9%) who were evaluated in Mayo Clinic's Genetic Heart Rhythm Clinic from January 1999 to December 2015. Breakthrough cardiac events (BCEs) were defined as LQTS-attributable syncope or seizures, aborted cardiac arrest, appropriate ventricular fibrillation-terminating implantable cardioverter-defibrillator shocks, and sudden cardiac death.RESULTS: There were 166 (27%) patients who were symptomatic prior to their first Mayo Clinic evaluation. Median age at first symptom was 12 years. Treatment strategies included no active therapy in 47 (8%) patients, beta-blockers alone in 350 (58%) patients, implantable cardioverter-defibrillators alone in 25 (4%) patients, left cardiac sympathetic denervation alone in 18 (3%) patients, and combination therapy in 166 (27%) patients. Over a median follow-up of 6.7 (IQR: 3.9 to 9.8) years, 556 (92%) patients have not experienced an LQTS-triggered BCE. Only 8 of 440 (2%) previously asymptomatic patients have experienced a single BCE. In contrast, 42 of 166 (25%) previously symptomatic patients have experienced ≥1 BCE. Among the 30 patients with ≥2 BCEs, 2 patients have died and 3 LQT3 patients underwent cardiac transplantation.CONCLUSIONS: Although outcomes have improved markedly, further optimization of treatment strategies is still needed given that 1 in 4 previously symptomatic patients experienced at least 1 subsequent, albeit nonlethal, LQTS-triggered cardiac event.

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