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Frontera, A; Vlachos, K; Kitamura, T; Mahida, S; Pillois, X; Fahy, G; Marquie, C; Cappato, R; Stuart, G; Defaye, P; Kaski, JP; Ector, J; Maltret, A; Scanu, P; Pasquie, JL; Deisenhofer, I; Blankoff, I; Scherr, D; Manninger, M; Aizawa, Y; Koutbi, L; Denis, A; Pambrun, T; Ritter, P; Sacher, F; Hocini, M; Maury, P; Jaïs, P; Bordachar, P; Haïssaguerre, M; Derval, N.
Long-Term Follow-Up of Idiopathic Ventricular Fibrillation in a Pediatric Population: Clinical Characteristics, Management, and Complications.
J Am Heart Assoc. 2019; 8(9): e011172-e011172.
Doi: 10.1161/JAHA.118.011172
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- Co-Autor*innen der Med Uni Graz
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Manninger-Wünscher Martin
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Scherr Daniel
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- Abstract:
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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.
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idiopathic
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defibrillator
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ventricular fibrillation
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complications
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ventricular tachycardia
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syncope