ArticleEuropean heart journal2024
Long QT syndrome: importance of reassessing arrhythmic risk after treatment initiation.
Article in European heart journal, 2024. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 10 papers.
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Who cites it
10 citing papers in PubMed.
- From Molecules to Machines: An Integrative Framework Linking Molecular Pathogenesis, Multi-Factorial Risk, Risk Stratification, Clinical Management, and Artificial Intelligence in QT Prolongation and Sudden Cardiac Death.Clinical cardiology · 2026Review
- Implantable cardioverter defibrillators for long QT syndrome and catecholaminergic polymorphic ventricular tachycardia? (Not so fast, Louis).Europace : European pacing, arrhythmias, and cardiac electrophysiology : journal of the working groups on cardiac pacing, arrhythmias, and cardiac cellular electrophysiology of the European Society of Cardiology · 2025Article
- Software-based analysis of T-wave morphology: identifying the electrocardiogram signature of high-risk long QT syndrome.Europace : European pacing, arrhythmias, and cardiac electrophysiology : journal of the working groups on cardiac pacing, arrhythmias, and cardiac cellular electrophysiology of the European Society of Cardiology · 2025Article
- TP-fusion at peak exercise: a novel marker for the recognition of unsuspected long QT syndrome patients.Europace : European pacing, arrhythmias, and cardiac electrophysiology : journal of the working groups on cardiac pacing, arrhythmias, and cardiac cellular electrophysiology of the European Society of Cardiology · 2025Article
- Congenital Long QT Syndrome: A Focus on Risk Stratification and Management.Reviews in cardiovascular medicine · 2025Review
- Sacubitril Does Not Exert Proarrhythmic Effects in Combination with Different Antiarrhythmic Drugs.Pharmaceuticals (Basel, Switzerland) · 2025Article
- Managing long QT syndrome patients, cooking, and common sense.European heart journal supplements : journal of the European Society of Cardiology · 2025Article
- Anti-Ro/SSA antibodies in adult arrhythmias: pathogenesis, clinical implications, and therapeutic strategies.Frontiers in immunology · 2025Review
- Different beta-blockers for preventing arrhythmic events in patients with long QT syndrome: a network meta-analysis.Frontiers in medicine · 2025Article
- Coronary artery disease and the risk of life-threatening cardiac events after age 40 in long QT syndrome.Frontiers in cardiovascular medicine · 2024Article
Corrections and comments
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Authors and funding
10 authors.
Funding
Abstract
BACKGROUND AND
aimsRisk scores are proposed for genetic arrhythmias. Having proposed in 2010 one such score (M-FACT) for the long QT syndrome (LQTS), this study aims to test whether adherence to its suggestions would be appropriate.
methodsLQT1/2/3 and genotype-negative patients without aborted cardiac arrest (ACA) before diagnosis or cardiac events (CEs) below age 1 were included in the study, focusing on an M-FACT score ≥2 (intermediate/high risk), either at presentation (static) or during follow-up (dynamic), previously associated with 40% risk of implantable cardioverter defibrillator (ICD) shocks within 4 years.
resultsOverall, 946 patients (26 ± 19 years at diagnosis, 51% female) were included. Beta-blocker (βB) therapy in 94% of them reduced the rate of those with a QTc ≥500 ms from 18% to 12% (P < .001). During 7 ± 6 years of follow-up, none died; 4% had CEs, including 0.4% with ACA. A static M-FACT ≥2 was present in 110 patients, of whom 106 received βBs. In 49/106 patients with persistent dynamic M-FACT ≥2, further therapeutic optimization (left cardiac sympathetic denervation in 55%, mexiletine in 31%, and ICD at 27%) resulted in just 7 (14%) patients with CEs (no ACA), with no CEs in the remaining 57. Additionally, 32 patients developed a dynamic M-FACT ≥2 but, after therapeutic optimization, only 3 (9%) had CEs. According to an M-FACT score ≥2, a total of 142 patients should have received an ICD, but only 22/142 (15%) were implanted, with shocks reported in 3.
conclusionsBeta-blockers often shorten QTc, thus changing risk scores and ICD indications for primary prevention. Yearly risk reassessment with therapy optimization leads to fewer ICD implants (3%) without increasing life-threatening events.
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