학술논문
Type 3 long QT syndrome: Is the effectiveness of treatment with beta-blockers population-specific?
Document Type
Academic Journal
Author
Hermida A; CNMR Maladies Cardiaques Héréditaires Rares, APHP, Hôpital Bichat, Paris, France; Service de Rythmologie, Centre Hospitalier Universitaire d'Amiens, Amiens, France.; Gourraud JB; L'institut du Thorax, CNMR Maladies Rythmique Héréditaires ou Rares, Service de Cardiologie et Unité INSERM 1087, Centre Hospitalier Universitaire de Nantes, Nantes, France.; Denjoy I; CNMR Maladies Cardiaques Héréditaires Rares, APHP, Hôpital Bichat, Paris, France.; Fressart V; AP-HP, Service de Biochimie Métabolique, Groupe Hospitalier Pitié-Salpêtrière, Paris, France.; Kyndt F; L'institut du Thorax, CNMR Maladies Rythmique Héréditaires ou Rares, Service de Cardiologie et Unité INSERM 1087, Centre Hospitalier Universitaire de Nantes, Nantes, France.; Maltret A; Service de Cardiopathie Congenitale, GHPSJ Hôpital Marie Lannelongue, Le Plessis Robinson, France.; Khraiche D; AP-HP, Pédiatrie, Hôpital Necker, Paris, France.; Klug D; Service de Cardiologie, Centre Hospitalier Universitaire, Lille, France.; Mabo P; Service de Cardiologie, Centre Hospitalier Universitaire, Rennes, France.; Sacher F; Service de Rythmologie, LIRYC Institute, Bordeaux University Hospital, University of Bordeaux, Bordeaux, France.; Maury P; Service de Cardiologie, Centre Hospitalier Universitaire, Toulouse, France.; Winum P; Service de Cardiologie, Centre Hospitalier Universitaire, Nîmes, France.; Defaye P; Service de Cardiologie, Centre Hospitalier Universitaire, Grenoble, France.; Clerici G; Service de Cardiologie, Centre Hospitalier Universitaire, Saint Pierre, La Réunion, France.; Babuty D; Service de Cardiologie, Centre Hospitalier Universitaire, Tours, France.; Elbez Y; Signifience-Biostatistics, Puteaux, France.; Morgat C; CNMR Maladies Cardiaques Héréditaires Rares, APHP, Hôpital Bichat, Paris, France; Université Paris Cité, Paris, France.; Surget E; CNMR Maladies Cardiaques Héréditaires Rares, APHP, Hôpital Bichat, Paris, France.; Messali A; CNMR Maladies Cardiaques Héréditaires Rares, APHP, Hôpital Bichat, Paris, France.; De Jode P; CNMR Maladies Cardiaques Héréditaires Rares, APHP, Hôpital Bichat, Paris, France.; Clédel A; L'institut du Thorax, CNMR Maladies Rythmique Héréditaires ou Rares, Service de Cardiologie et Unité INSERM 1087, Centre Hospitalier Universitaire de Nantes, Nantes, France.; Minois D; L'institut du Thorax, CNMR Maladies Rythmique Héréditaires ou Rares, Service de Cardiologie et Unité INSERM 1087, Centre Hospitalier Universitaire de Nantes, Nantes, France.; Maison-Blanche P; CNMR Maladies Cardiaques Héréditaires Rares, APHP, Hôpital Bichat, Paris, France.; Bloch A; AP-HP, Service de Biochimie Métabolique, Groupe Hospitalier Pitié-Salpêtrière, Paris, France.; Leenhardt A; CNMR Maladies Cardiaques Héréditaires Rares, APHP, Hôpital Bichat, Paris, France; Université Paris Cité, Paris, France.; Probst V; L'institut du Thorax, CNMR Maladies Rythmique Héréditaires ou Rares, Service de Cardiologie et Unité INSERM 1087, Centre Hospitalier Universitaire de Nantes, Nantes, France.; Extramiana F; CNMR Maladies Cardiaques Héréditaires Rares, APHP, Hôpital Bichat, Paris, France; Université Paris Cité, Paris, France. Electronic address: fabrice.extramiana@aphp.fr.
Source
Publisher: Elsevier Country of Publication: United States NLM ID: 101200317 Publication Model: Print-Electronic Cited Medium: Internet ISSN: 1556-3871 (Electronic) Linking ISSN: 15475271 NLM ISO Abbreviation: Heart Rhythm Subsets: MEDLINE
Subject
Language
English
Abstract
Background: The efficacy of beta-blocker treatment in type 3 long QT syndrome (LQT3) remains debated.
Objectives: The purpose of this study was to test the hypothesis that beta-blocker use is associated with cardiac events (CEs) in a French cohort of LQT3 patients.
Methods: All patients with a likely pathogenic/pathogenic variant in the SCN5A gene (linked to LQT3) were included and followed-up. Documented ventricular tachycardia/ventricular fibrillation, torsades de pointes, aborted cardiac arrest, sudden death, and appropriate shocks were considered as severe cardiac events (SCEs). CEs also included syncope.
Results: We included 147 patients from 54 families carrying 23 variants. Six of the patients developed symptoms before the age of 1 year and were analyzed separately. The 141 remaining patients (52.5% male; median age at diagnosis 24.0 years) were followed-up for a median of 11 years. The probabilities of a CE and an SCE from birth to the age of 40 were 20.5% and 9.9%, respectively. QTc prolongation (hazard ratio [HR] 1.12 [1.0-1.2]; P = .005]) and proband status (HR 4.07 [1.9-8.9]; P <.001) were independently associated with the occurrence of CEs. Proband status (HR 8.13 [1.7-38.8]; P = .009) was found to be independently associated with SCEs, whereas QTc prolongation (HR 1.11 [1.0-1.3]; P = .108) did not reach statistical significance. The cumulative probability of the age at first CE/SCE was not lower in patients treated with a beta-blocker.
Conclusion: In agreement with the literature, proband status and lengthened QTc were associated with a higher risk of CEs. Our data do not show a protective effect of beta-blocker treatment.
Competing Interests: Disclosures The authors have no conflicts to disclose.
(Copyright © 2023 Heart Rhythm Society. Published by Elsevier Inc. All rights reserved.)
Objectives: The purpose of this study was to test the hypothesis that beta-blocker use is associated with cardiac events (CEs) in a French cohort of LQT3 patients.
Methods: All patients with a likely pathogenic/pathogenic variant in the SCN5A gene (linked to LQT3) were included and followed-up. Documented ventricular tachycardia/ventricular fibrillation, torsades de pointes, aborted cardiac arrest, sudden death, and appropriate shocks were considered as severe cardiac events (SCEs). CEs also included syncope.
Results: We included 147 patients from 54 families carrying 23 variants. Six of the patients developed symptoms before the age of 1 year and were analyzed separately. The 141 remaining patients (52.5% male; median age at diagnosis 24.0 years) were followed-up for a median of 11 years. The probabilities of a CE and an SCE from birth to the age of 40 were 20.5% and 9.9%, respectively. QTc prolongation (hazard ratio [HR] 1.12 [1.0-1.2]; P = .005]) and proband status (HR 4.07 [1.9-8.9]; P <.001) were independently associated with the occurrence of CEs. Proband status (HR 8.13 [1.7-38.8]; P = .009) was found to be independently associated with SCEs, whereas QTc prolongation (HR 1.11 [1.0-1.3]; P = .108) did not reach statistical significance. The cumulative probability of the age at first CE/SCE was not lower in patients treated with a beta-blocker.
Conclusion: In agreement with the literature, proband status and lengthened QTc were associated with a higher risk of CEs. Our data do not show a protective effect of beta-blocker treatment.
Competing Interests: Disclosures The authors have no conflicts to disclose.
(Copyright © 2023 Heart Rhythm Society. Published by Elsevier Inc. All rights reserved.)