Risk Factors and Mode of Death in Isolated Hypertrophic Cardiomyopathy in Children

作者: Jamie A. Decker , Joseph W. Rossano , E. O'Brian Smith , Bryan Cannon , Sarah K. Clunie

DOI: 10.1016/J.JACC.2009.03.051

关键词:

摘要: Objectives This study was designed to review outcomes of pediatric isolated hypertrophic cardiomyopathy (HCM) managed uniformly at a single institution and assess whether reported adult risk factors for sudden death are predictive in HCM. Background Cardiac HCM occurs suddenly (SCD) or may be nonsudden (non-SCD). Little data exists on non-SCD children. Risk SCD characterized consensus management strategies detailed. Their application children is uncertain treatment vary. Methods A retrospective cohort with performed. Primary end points were cardiac transplantation. Frequency known assessed. Outcomes analysis performed using Kaplan-Meier curves Cox regression analysis. Results Ninety-six patients included. The average age diagnosis 10.6 ± 5.4 years, mean follow-up 6.4 5.2 years. occurred 11 over the 20-year (11%), 4 underwent transplant 7 died (3 suddenly). Extreme left ventricular hypertrophy (z-score: >6) an abnormal blood pressure response exercise (p Conclusions In primarily restriction medication, infrequently. Non-SCD least as common SCD. blunted associated increased non-SCD.

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