Intentional nontherapy in long QT syndrome

Ciorsti J. MacIntyre, Ram K. Rohatgi, Alan M. Sugrue, J. Martijn Bos, Michael J. Ackerman

Research output: Contribution to journalArticle

Abstract

Background: International guidelines advise universal beta-blocker therapy as either a class I (symptomatic or QTc ≥470 ms) or class II (asymptomatic and QTc <470 ms) recommendation for treatment of long QT syndrome (LQTS). Objective: The purpose of this study was to evaluate the outcomes of a highly selected cohort of patients with LQTS managed with an observation-only (intentional nontreatment) strategy. Methods: The cohort was derived from a comprehensive retrospective registry of patients with LQTS. Clinical phenotype and genotype data were collected via review of electronic health records. Results: Among 661 patients with LQTS, 55 (8.3%) asymptomatic patients (53% female; 16 age <18 years) were managed with intentional nontherapy. Only preventative measures were advised. Mean age at diagnosis was 37.8 ± 21.2 years. Mean QTc was 448 ± 30 ms. None of the patients experienced an LQTS-triggered cardiac event over mean follow-up of 7.5 ± 4.3 years. Compared to the larger treated cohort, this intentionally untreated cohort was less symptomatic, was older at diagnosis, and had lower resting QTc values (P <.0001). Conclusion: After careful clinical evaluation, risk assessment, and institution of precautionary measures, an observation-only strategy may be considered in a highly selected group of LQTS patients with a clinical profile that includes asymptomatic status, older age at diagnosis, and QTc <470 ms, with excellent outcomes and better quality of life than LQTS patients treated with beta-blocker. LQTS patients with this low-risk profile should not receive a prophylactic implantable cardioverter–defibrillator.

Original languageEnglish (US)
Pages (from-to)1147-1150
Number of pages4
JournalHeart rhythm
Volume17
Issue number7
DOIs
StatePublished - Jul 2020

Keywords

  • Genetics
  • Ion channels
  • Long QT syndrome
  • Sudden cardiac arrest
  • Sudden cardiac death

ASJC Scopus subject areas

  • Cardiology and Cardiovascular Medicine
  • Physiology (medical)

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    MacIntyre, C. J., Rohatgi, R. K., Sugrue, A. M., Bos, J. M., & Ackerman, M. J. (2020). Intentional nontherapy in long QT syndrome. Heart rhythm, 17(7), 1147-1150. https://doi.org/10.1016/j.hrthm.2020.02.017