TY - JOUR
T1 - Intentional nontherapy in long QT syndrome
AU - MacIntyre, Ciorsti J.
AU - Rohatgi, Ram K.
AU - Sugrue, Alan M.
AU - Bos, J. Martijn
AU - Ackerman, Michael J.
N1 - Funding Information:
This study was supported by The Mayo Clinic Windland Smith Rice Comprehensive Sudden Cardiac Death Program. Dr MacIntyre has received honoraria or speaker fees from Abbott and Medtronic . Dr Ackerman is a consultant for Audentes Therapeutics, Biotronik, Boston Scientific, Daiichi Sankyo, Gilead Sciences, Invitae, Medtronic, MyoKardia, and St. Jude Medical. Dr Ackerman and Mayo Clinic have a licensing agreement with AliveCor. None of these entities participated in this study in any way. All other authors have reported that they have no conflicts relevant to the contents of this paper to disclose.
Funding Information:
This study was supported by The Mayo Clinic Windland Smith Rice Comprehensive Sudden Cardiac Death Program. Dr MacIntyre has received honoraria or speaker fees from Abbott and Medtronic. Dr Ackerman is a consultant for Audentes Therapeutics, Biotronik, Boston Scientific, Daiichi Sankyo, Gilead Sciences, Invitae, Medtronic, MyoKardia, and St. Jude Medical. Dr Ackerman and Mayo Clinic have a licensing agreement with AliveCor. None of these entities participated in this study in any way. All other authors have reported that they have no conflicts relevant to the contents of this paper to disclose.
Publisher Copyright:
© 2020
PY - 2020/7
Y1 - 2020/7
N2 - 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.
AB - 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.
KW - Genetics
KW - Ion channels
KW - Long QT syndrome
KW - Sudden cardiac arrest
KW - Sudden cardiac death
UR - http://www.scopus.com/inward/record.url?scp=85086569655&partnerID=8YFLogxK
U2 - 10.1016/j.hrthm.2020.02.017
DO - 10.1016/j.hrthm.2020.02.017
M3 - Article
C2 - 32105774
AN - SCOPUS:85086569655
SN - 1547-5271
VL - 17
SP - 1147
EP - 1150
JO - Heart Rhythm
JF - Heart Rhythm
IS - 7
ER -