@Article{chd.2023.028829,
AUTHOR = {Charis Tan , Diana Zannino, Carley Clendenning, Sophie Offen, Thomas L. Gentles, Julian Ayer, David Tanous, Vishva Wijesekera, Leeanne Grigg, David Celermajer, Mark McGuire , Yves d’Udekem, Rachael Cordina},
TITLE = {Ventricular Arrhythmia in the Fontan Circulation: Prevalence, Risk Factors and Clinical Implications},
JOURNAL = {Structural and Congenital Heart Disease},
VOLUME = {18},
YEAR = {2023},
NUMBER = {5},
PAGES = {507--523},
URL = {http://www.techscience.com/schd/v18n5/54532},
ISSN = {3071-1738},
ABSTRACT = {<b>Objective:</b> 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. <b>Methods:</b> 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. <b>Results:</b>
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 (<i>p</i> = 0.03) and older age
Fontan operation (<i>p</i> < 0.001) were associated with VA. Earlier Fontan era (<i>p</i> < 0.003), atriopulmonary Fontan (<i>p</i> <
0.001), pre-Fontan atrioventricular valve repair (<i>p</i> = 0.013) pre- or post-Fontan atrial arrhythmia (<i>p</i> = 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),
<i>p</i> = 0.02). <b>Conclusions:</b> 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.},
DOI = {10.32604/chd.2023.028829}
}