A cohort study of childhood hypertrophic cardiomyopathy

摘要: Abstract OBJECTIVES The study analyzed factors, including treatment, affecting disease-related death in patients with hypertrophic cardiomyopathy (HCM) presenting childhood. BACKGROUND Previous smaller studies suggest that mortality is higher HCM childhood compared presentation adulthood, but these have all originated from selected patient populations tertiary referral centers, and reported no significant protection by treatment. METHODS Retrospective comparisons of were done total cohort to three regional centers pediatric cardiology. There 66 (25 Noonan’s syndrome) at age RESULTS Among risk factors for congestive heart failure (p = 0.008), large electrocardiogram voltages (Sokolow-Lyon index p 0.0003), degree septal 0.004) left ventricular 0.028) hypertrophy expressed as percent 95th centile value. only treatment significantly reduced the on multifactorial analysis variance was high-dose beta-adrenoceptor antagonist therapy (propranolol 5 23 mg/kg/day or equivalent; 0.0001). Nineteen out 40 managed conventionally (no 0.8 4 mg/kg propranolol, verapamil) died, median survival 15.8 years, deaths among 26 beta-blockers 0.0004); proportions 10 years 0.65 (95% confidence interval 0.49–0.80) 1.0, respectively 0.0015). Survival time shows better beta-blocker group “no specific therapy” 0.0009) conventional-dose 0.002). Hazard ratio suggests produces a 5–10- fold reduction death. CONCLUSIONS High-dose improves HCM.