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

Autor: Jack F. Price, Bryan C. Cannon, John L. Jefferies, William J. Dreyer, Joseph W. Rossano, Sarah K. Clunie, Corey M. Gates, Jeffrey J. Kim, Jamie A. Decker, Jeffrey A. Towbin, E. O'Brian Smith, Susan W. Denfield
Rok vydání: 2009
Předmět:
Male
medicine.medical_specialty
pediatrics
Adolescent
medicine.medical_treatment
Cardiomyopathy
Kaplan-Meier Estimate
Left ventricular hypertrophy
Sudden death
sudden cardiac death
Sudden cardiac death
Cohort Studies
implantable cardioverter-defibrillator
Risk Factors
Internal medicine
medicine
Humans
cardiovascular diseases
Risk factor
Child
Exercise
Retrospective Studies
Heart Failure
business.industry
Age Factors
Hypertrophic cardiomyopathy
Cardiomyopathy
Hypertrophic
medicine.disease
Implantable cardioverter-defibrillator
Survival Analysis
Texas
Defibrillators
Implantable
Transplantation
Death
Sudden
Cardiac
Disease Progression
cardiovascular system
Cardiology
Female
Hypertrophy
Left Ventricular
ventricular tachycardia
Hypotension
hypertrophy
Cardiology and Cardiovascular Medicine
business
cardiomyopathy
Algorithms
Zdroj: Journal of the American College of Cardiology. 54:250-254
ISSN: 0735-1097
DOI: 10.1016/j.jacc.2009.03.051
Popis: ObjectivesThis 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 pediatric HCM.BackgroundCardiac death in HCM occurs suddenly (SCD) or may be nonsudden (non-SCD). Little data exists on non-SCD in children. Risk factors for SCD in adult HCM are characterized and consensus management strategies detailed. Their application to children is uncertain and treatment strategies vary.MethodsA retrospective cohort study of children with HCM was performed. Primary end points were cardiac death and transplantation. Frequency and outcomes of known adult risk factors were assessed. Outcomes analysis was performed using Kaplan-Meier curves and Cox regression analysis.ResultsNinety-six patients were included. The average age at diagnosis was 10.6 ± 5.4 years, and mean follow-up was 6.4 ± 5.2 years. Primary end points occurred in 11 patients over the 20-year follow-up (11%), 4 underwent cardiac transplant and 7 died (3 suddenly). Extreme left ventricular hypertrophy (z-score: >6) and an abnormal blood pressure response to exercise were predictive of non-SCD (p < 0.02 and p < 0.03, respectively). Kaplan-Meier survival analysis predicts an 82% survival over a 20-year period.ConclusionsIn children with isolated HCM managed primarily with exercise restriction and medication, cardiac death occurred infrequently. Non-SCD or transplant was at least as common as SCD. Extreme left ventricular hypertrophy and blunted blood pressure response to exercise were associated with an increased risk of non-SCD.
Databáze: OpenAIRE
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