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. 2019 May 7;8(9):e011172.
doi: 10.1161/JAHA.118.011172.

Long-Term Follow-Up of Idiopathic Ventricular Fibrillation in a Pediatric Population: Clinical Characteristics, Management, and Complications

Antonio Frontera  1   2 , Konstantinos Vlachos  1 , Takeshi Kitamura  1 , Saagar Mahida  3 , Xavier Pillois  1   2 , Gerard Fahy  4 , Christelle Marquie  5 , Riccardo Cappato  6 , Graham Stuart  7 , Pascal Defaye  8 , Juan Pablo Kaski  9   10 , Joris Ector  11 , Alice Maltret  12 , Patrice Scanu  13 , Jean-Luc Pasquie  14 , Isabelle Deisenhofer  15 , Ivan Blankoff  16 , Daniel Scherr  17 , Martin Manninger  17 , Yoshifusa Aizawa  18 , Linda Koutbi  19 , Arnaud Denis  1 , Thomas Pambrun  1 , Philippe Ritter  1 , Frederic Sacher  1 , Meleze Hocini  1   2 , Philippe Maury  20 , Pierre Jaïs  1   2 , Pierre Bordachar  1   2 , Michel Haïssaguerre  1   2 , Nicolas Derval  1
Affiliations

Long-Term Follow-Up of Idiopathic Ventricular Fibrillation in a Pediatric Population: Clinical Characteristics, Management, and Complications

Antonio Frontera et al. J Am Heart Assoc. .

Abstract

Background The natural history and long-term outcome in pediatric patients with idiopathic ventricular fibrillation ( IVF ) are poorly characterized. We sought to define the clinical characteristics and long-term outcomes of a pediatric cohort with an initial diagnosis of IVF . Methods and Results Patients were included from an International Registry of IVF (consisting of 496 patients). Inclusion criteria were: (1) VF with no identifiable cause following comprehensive analysis for ischemic, electrical or structural heart disease and (2) age ≤16 years. These included 54 pediatric IVF cases (age 12.7±3.7 years, 59% male) among whom 28 (52%) had a previous history of syncope (median 2 syncopal episodes [interquartile range 1]). Thirty-six (67%) had VF in situations associated with high adrenergic tone. During a median 109±12 months of follow-up, 31 patients (57%) had recurrence of ventricular arrhythmias, mainly VF . Two patients developed phenotypic expression of an inherited arrhythmia syndrome during follow-up (hypertrophic cardiomyopathy and long QT syndrome, respectively). A total of 15 patients had positive genetic testing for inherited arrhythmia syndromes. Ten patients (18%) experienced device-related complications. Three patients (6%) died, 2 due to VF storm. Conclusions In pediatric patients with IVF , a minority develop a definite clinical phenotype during long-term follow-up. Recurrent VF is common in this patient group.

Keywords: complications; defibrillator; idiopathic; syncope; ventricular fibrillation; ventricular tachycardia.

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Figures

Figure 1
Figure 1
Kaplan–Meier curve demonstrating freedom from ventricular arrhythmias.
Figure 2
Figure 2
Summary of phenotypic evaluation following index episode and at follow‐up (78±11 months). CMR indicates cardiac magnetic resonance; ECHO, echocardiogram; HCM, hypertrophic cardiomyopathy; IVF, idiopathic ventricular fibrillation; VF, ventricular fibrillation.
See this image and copyright information in PMC

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