caa a22 4500 445825340 CHVBK 20180317145257.0 cr unu---uuuuu 170323e20111201xx s 000 0 eng 10.1007/s00246-011-9967-y doi (NATIONALLICENCE)springer-10.1007/s00246-011-9967-y Long-Term Follow-Up of Children and Adolescents Diagnosed with Hypertrophic Cardiomyopathy: Risk Factors for Adverse Arrhythmic Events [Elektronische Daten] [Jeffrey Moak, Eric Leifer, Dorothy Tripodi, Saidi Mohiddin, Lameh Fananapazir] Our aim was to identify prognostic factors for an arrhythmic event (AE) in children with hypertrophic cardiomyopathy (HCM) without a previous AE. One hundred thirty-one nonconsecutive patients (≤20years) with HCM but no previous AE were evaluated at the NIH Clinical Center from 1980 to 2001. At a median follow-up of 6.4years, 22 patients experienced an AE [sudden death (SD) (n=12), resuscitated cardiac arrest (n=3), clinical sustained ventricular tachycardia (VT) (n=2), and implantable cardiac defibrillator discharge (n=5)], resulting in a 2% annual AE rate. Baseline factors that were most predictive in univariate risk analysis included ventricular septal thickness (ST) (P=0.01), VT induction by programmed ventricular stimulation (PVS) (P=0.01), age (P=0.05), and presyncope/syncope (P=0.05). In multivariate analysis, ST, age, presyncope/syncope, and PVS were not independently predictive of risk for an AE. However, the 5-year event rates for AE was 15% (95% CI: 5-23%) if ST≥20mm, 19% (95% CI: 6-31%) when age≥13years and ST≥20mm were combined together, and 23% (95% CI: 3-39%) when PVS and ST≥20mm were combined together. Of the various risk factors that were consideredin our pediatric HCM cohort, ST and inducible VT were the most significant univariate predictors of risk for an AE. More traditional risk factors identified in older patients (family history of SD, VT on Holter, and exercise-induced hypotension) were not predictive of an AE in patients age under 21years. Springer Science+Business Media, LLC, 2011 Hypertrophic cardiomyopathy nationallicence Arrhythmia event nationallicence Children nationallicence Moak Jeffrey Department of Cardiology, Children's National Medical Center, Washington, DC, USA aut Leifer Eric Office of Biostatistics Research, National Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, MD, USA aut Tripodi Dorothy Cardiovascular Branch, National Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, MD, USA aut Mohiddin Saidi Cardiovascular Branch, National Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, MD, USA aut Fananapazir Lameh Cardiovascular Branch, National Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, MD, USA aut Pediatric Cardiology Springer-Verlag 32/8(2011-12-01), 1096-1105 0172-0643 32:8<1096 2011 32 246 https://doi.org/10.1007/s00246-011-9967-y text/html Onlinezugriff via DOI 1 research-article jats NATIONALLICENCE 856 40 https://doi.org/10.1007/s00246-011-9967-y text/html Onlinezugriff via DOI NATIONALLICENCE 700 1- Moak Jeffrey Department of Cardiology, Children's National Medical Center, Washington, DC, USA aut NATIONALLICENCE 700 1- Leifer Eric Office of Biostatistics Research, National Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, MD, USA aut NATIONALLICENCE 700 1- Tripodi Dorothy Cardiovascular Branch, National Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, MD, USA aut NATIONALLICENCE 700 1- Mohiddin Saidi Cardiovascular Branch, National Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, MD, USA aut NATIONALLICENCE 700 1- Fananapazir Lameh Cardiovascular Branch, National Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, MD, USA aut NATIONALLICENCE 773 0- Pediatric Cardiology Springer-Verlag 32/8(2011-12-01), 1096-1105 0172-0643 32:8<1096 2011 32 246 Metadata rights reserved Springer special CC-BY-NC licence nationallicence BK010053 XK010053 XK010000 NATIONALLICENCE NATIONALLICENCE NL-springer