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Efficacy of Intentional Permanent Atrial Pacing in the Long-Term Management of Congenital Long QT Syndrome.

Abstract：

BACKGROUND:Unfortunately, some patients with long QT syndrome (LQTS) experience breakthrough cardiac events (BCEs) despite maximal therapy. Small studies have shown that refractory LQTS cases may benefit from intentional permanent atrial pacing (IPAP). 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 patients diagnosed with LQTS 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, sustained 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 the LQT2 cohort (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. This article is protected by copyright. All rights reserved.

journal_name

J Cardiovasc Electrophysiol

journal_title

Journal of cardiovascular electrophysiology

authors

Kowlgi GN,Giudicessi JR,Barake W,Bos JM,Ackerman MJ

doi

10.1111/jce.14920

subject

Has Abstract

pub_date

2021-01-29 00:00:00

eissn

1045-3873

issn

1540-8167

pub_type

杂志文章

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Efficacy of Intentional Permanent Atrial Pacing in the Long-Term Management of Congenital Long QT Syndrome.