Managing long QT syndrome patients, cooking, and common sense.

IF 1.7 4区 医学 Q3 CARDIAC & CARDIOVASCULAR SYSTEMS European Heart Journal Supplements Pub Date : 2025-02-19 eCollection Date: 2025-02-01 DOI:10.1093/eurheartjsupp/suae085
Peter J Schwartz, Federica Dagradi, Fulvio L F Giovenzana, Paolo Cerea
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引用次数: 0

Abstract

This essay stems from a controversial recommendation present in the 2022 European Guidelines which indicated the appropriateness of considering an implantable cardioverter defibrillator (ICD) implant even for still asymptomatic long QT syndrome (LQTS) patients deemed to be at high risk by the 1-2-3 LQTS score based on QTc and genotype calculated prior to the institution of therapy. As 15 years ago, we also had proposed, but never used, a risk score called M-FACT to identify patients at high risk of an appropriate ICD shock, we felt the responsibility of assessing what would have happened to our patients if we had rigorously used that score. We performed a study recently published in the European Heart Journal which brought to general attention two concepts important for clinical management. One is that all LQTS patients should be seen at least once a year for a reassessment of arrhythmic risk based on standard electrocardiogram, 12-lead 24 h Holter recording and an exercise stress test. The other is that, based on these yearly visits, we perform 'therapy optimization' by adding to the standard β-blocker therapy either left cardiac sympathetic denervation or mexiletine or an ICD implant. On almost 1000 LQTS patients, all genotyped, this dynamic approach was accompanied by not a single death, few events, and out of 142 patients who should have received an ICD based on the score, only 22 did and only 3 had an ICD shock. These data and concepts call for a reconsideration of the recommendation made by the guidelines.

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来源期刊
European Heart Journal Supplements
European Heart Journal Supplements 医学-心血管系统
CiteScore
3.00
自引率
0.00%
发文量
575
审稿时长
12 months
期刊介绍: The European Heart Journal Supplements (EHJs) is a long standing member of the ESC Journal Family that serves as a publication medium for supplemental issues of the flagship European Heart Journal. Traditionally EHJs published a broad range of articles from symposia to special issues on specific topics of interest. The Editor-in-Chief, Professor Roberto Ferrari, together with his team of eminent Associate Editors: Professor Francisco Fernández-Avilés, Professors Jeroen Bax, Michael Böhm, Frank Ruschitzka, and Thomas Lüscher from the European Heart Journal, has implemented a change of focus for the journal. This entirely refreshed version of the European Heart Journal Supplements now bears the subtitle the Heart of the Matter to give recognition to the focus the journal now has. The EHJs – the Heart of the Matter intends to offer a dedicated, scientific space for the ESC, Institutions, National and Affiliate Societies, Associations, Working Groups and Councils to disseminate their important successes globally.
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