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Efficacy of Intentional Permanent Atrial Pacing in the Long-Term Management of Congenital Long QT Syndrome
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  • Gurukripa Kowlgi,
  • John Giudicessi,
  • Walid Barake,
  • Johan Bos,
  • Michael Ackerman
Gurukripa Kowlgi
Mayo Clinic
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John Giudicessi
Mayo Clinic
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Walid Barake
Mayo Clinic
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Johan Bos
Mayo Clinic
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Michael Ackerman
Mayo Clinic
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Background: Some long QT syndrome (LQTS) patients experience breakthrough cardiac events (BCEs) despite maximal therapy. Small studies have shown that intentional permanent atrial pacing (IPAP) is beneficial in refractory LQTS. As such, we sought to determine the genotype-specific utilization and efficacy of IPAP in a single-center LQTS registry. Methods and Results: In this retrospective study, electronic medical records from 1,065 LQTS patients were used to identify individuals that received IPAP. Pre- and post-IPAP heart rate, heart rate-corrected QT (QTc) values, annual BCE rate, and IPAP-related complications were compared between genotypes. BCEs were defined as LQTS-associated syncope/seizures, ventricular arrhythmia (VA)-terminating ICD therapies, and sudden cardiac arrest/death. Overall, 52/1065 LQTS patients received adjunctive IPAP therapy [77% female; median age 18.5 (IQR 1-35.5) years; 73% with prior VA]. Over an average IPAP follow-up of 121  82 months, the average heart rate increased from 65.8  20.4 bpm to 78.9  17.1 bpm; (p<0.01) and the average QTc decreased from 533.4  66.6 ms to 488.3  52.4 ms; (p<0.01). The mean BCE rate dropped from 0.88 to 0.19 per patient-year (p=0.01), driven by a marked decrease in LQT2 patients (1.01 BCE/year to 0.02 BCE/year; p=0.003). No serious IPAP-related complications were observed. Conclusion: In high-risk LQTS patients, namely those with recalcitrant LQT2, IPAP appears to be a safe and efficacious adjunct therapy. The beneficial effects of IPAP may stem from attenuating the QTc and circumventing a pause-dependent trigger. Whether IPAP might obviate the need for an ICD in some instances warrants further study.

Peer review status:Published

29 Jun 2020Submitted to Journal of Cardiovascular Electrophysiology
30 Jun 2020Submission Checks Completed
30 Jun 2020Assigned to Editor
01 Jul 2020Reviewer(s) Assigned
18 Jul 2020Review(s) Completed, Editorial Evaluation Pending
20 Jul 2020Editorial Decision: Revise Minor
16 Nov 20201st Revision Received
27 Nov 2020Assigned to Editor
27 Nov 2020Submission Checks Completed
27 Nov 2020Reviewer(s) Assigned
14 Dec 2020Review(s) Completed, Editorial Evaluation Pending
14 Dec 2020Editorial Decision: Revise Minor
21 Dec 20202nd Revision Received
23 Dec 2020Submission Checks Completed
23 Dec 2020Assigned to Editor
23 Dec 2020Reviewer(s) Assigned
30 Dec 2020Review(s) Completed, Editorial Evaluation Pending
02 Jan 2021Editorial Decision: Accept
10 Feb 2021Published in Journal of Cardiovascular Electrophysiology. 10.1111/jce.14920