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Ventricular Arrhythmia in the Fontan Circulation:Prevalence,Risk Factors and Clinical Implications

Congenital Heart Disease 2023年第5期18卷 507-523页

作者： Charis Tan Diana Zannino Carley Clendenning sophie offen Thomas LGentles Julian Ayer David Tanous Vishva Wijesekera Leeanne Grigg David Celermajer Mark McGuire Yves d’Udekem Rachael Cordina Department of Cardiothoracic Surgery Royal Prince Alfred HospitalCamperdownAustralia Sydney Medical School University of SydneyCamperdownAustralia Clinical Epidemiology and Biostatistics Unit Murdoch Children’s Research InstituteParkvilleAustralia Department of Cardiology Royal Prince Alfred HospitalCamperdownAustralia Department of Paediatric Cardiology Starship HospitalAucklandNew Zealand Department of Paediatric Cardiology The Children’s Hospital at WestmeadWestmeadAustralia Department of Cardiology Westmead HospitalWestmeadAustralia Adult Congenital Heart Disease Unit The Prince Charles HospitalBrisbaneAustralia Department of Cardiology The Royal Melbourne HospitalMelbourneAustralia Heart Research Institute CamperdownAustralia Department of Cardiac Surgery The Royal Children’s Hospital MelbourneParkvilleAustralia Department of Paediatrics The University of MelbourneParkvilleAustralia

Objective:Sudden cardiac death(SCD)and malignant ventricular arrhythmia(VA)are increasingly recognized as important issues for people living with a Fontan circulation,but data are *** sought to characterize the cohort who had sudden cardiac death,most likely related to VA and/or documented VA in the Australia and New Zealand Fontan Registry including risk factors and clinical ***:A retrospective cohort study was *** criteria were documented non-sustained ventricular tachycardia,sustained ventricular tachycardia,ventricular fibrillation,resuscitated cardiac arrest or SCD>30 days post-Fontan ***:Of 1611 patients,20(1.2%)had VA;14(1.0%)had VA without SCD and 6(<1%)had SCD(6%of all deaths recorded in Registry;5 of those had documented VA at the time of arrest and 1 was presumed to be VA-associated).The median age at first VA was 20.5(14–32)years,10(50%)were females,and the median age at Fontan operation was 8(4–17)*** univariable analysis,hypoplastic left heart syndrome(p=0.03)and older age Fontan operation(p<0.001)were associated with *** Fontan era(p<0.003),atriopulmonary Fontan(p<0.001),pre-Fontan atrioventricular valve repair(p=0.013)pre-or post-Fontan atrial arrhythmia(p=0.010)were associated with *** with VA had a 3 times higher risk of death or heart transplant(HR 3.27(1.19,8.98),p=0.02).Conclusions:A proportion of people living with a Fontan circulation have malignant *** VA screening in this cohort is *** data are needed to aid risk stratification.

关键词： Fontan congenital cardiac arrhythmia sudden cardiac death ventricular tachyarrhythmia

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