Open Access

ARTICLE

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

Charis Tan^1,2 , Diana Zannino³, Carley Clendenning³, Sophie Offen⁴, Thomas L. Gentles⁵, Julian Ayer⁶, David Tanous⁷, Vishva Wijesekera⁸, Leeanne Grigg⁹, David Celermajer^2,4,10, Mark McGuire^2,4 , Yves d’Udekem^3,11,12, Rachael Cordina^2,4,10,*

1 Department of Cardiothoracic Surgery, Royal Prince Alfred Hospital, Camperdown, Australia
2 Sydney Medical School, University of Sydney, Camperdown, Australia
3 Clinical Epidemiology and Biostatistics Unit, Murdoch Children’s Research Institute, Parkville, Australia
4 Department of Cardiology, Royal Prince Alfred Hospital, Camperdown, Australia
5 Department of Paediatric Cardiology, Starship Hospital, Auckland, New Zealand
6 Department of Paediatric Cardiology, The Children’s Hospital at Westmead, Westmead, Australia
7 Department of Cardiology, Westmead Hospital, Westmead, Australia
8 Adult Congenital Heart Disease Unit, The Prince Charles Hospital, Brisbane, Australia
9 Department of Cardiology, The Royal Melbourne Hospital, Melbourne, Australia
10 Heart Research Institute, Camperdown, Australia
11 Department of Cardiac Surgery, The Royal Children’s Hospital Melbourne, Parkville, Australia
12 Department of Paediatrics, The University of Melbourne, Parkville, Australia

* Corresponding Author: Rachael Cordina. Email:

Congenital Heart Disease 2023, 18(5), 507-523. https://doi.org/10.32604/chd.2023.028829

Received 10 January 2023; Accepted 15 May 2023; Issue published 10 November 2023

Abstract

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 lacking. We 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 outcomes. Methods: A retrospective cohort study was performed. Inclusion criteria were documented non-sustained ventricular tachycardia, sustained ventricular tachycardia, ventricular fibrillation, resuscitated cardiac arrest or SCD > 30 days post-Fontan completion. Results: 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) years. On univariable analysis, hypoplastic left heart syndrome (p = 0.03) and older age Fontan operation (p < 0.001) were associated with VA. Earlier 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 SCD. Patients 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. Routine VA screening in this cohort is essential. More data are needed to aid risk stratification.

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