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


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.

SUBMITTER: Frontera A 

PROVIDER: S-EPMC6512137 | biostudies-literature | 2019 May

REPOSITORIES: biostudies-literature

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

Frontera Antonio A   Vlachos Konstantinos K   Kitamura Takeshi T   Mahida Saagar S   Pillois Xavier X   Fahy Gerard G   Marquie Christelle C   Cappato Riccardo R   Stuart Graham G   Defaye Pascal P   Kaski Juan Pablo JP   Ector Joris J   Maltret Alice A   Scanu Patrice P   Pasquie Jean-Luc JL   Deisenhofer Isabelle I   Blankoff Ivan I   Scherr Daniel D   Manninger Martin M   Aizawa Yoshifusa Y   Koutbi Linda L   Denis Arnaud A   Pambrun Thomas T   Ritter Philippe P   Sacher Frederic F   Hocini Meleze M   Maury Philippe P   Jaïs Pierre P   Bordachar Pierre P   Haïssaguerre Michel M   Derval Nicolas N  

Journal of the American Heart Association 20190501 9


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, elec  ...[more]

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