Predictors of risk for sudden death in childhood hypertrophic cardiomyopathy: the importance of the ECG risk score

• Published in: Open heart 2017, Vol.4 (2), p.e000658-e000658
• Main Authors: Östman-Smith, Ingegerd, Sjöberg, Gunnar, Rydberg, Annika, Larsson, Per, Fernlund, Eva
• Format: Article
• Language: English
• Subjects: adolescent; adult; Adults; Age; amiodarone; Arrhythmias and Sudden Death; atenolol; calcium channel blocking agent; Cardiac and Cardiovascular Systems; Cardiac arrhythmia; Cardiology; Cardiomyopathy; cardiomyopathy hypertrophic; cardiovascular parameters; child; childhood disease; clinical article; clinical feature; Clinical Medicine; cohort analysis; Defibrillators; Detroit wall thickness Z score; diagnostic test accuracy study; disopyramide; echography; electrocardiogram; Electrocardiography; exercise test; family history; Family medical history; female; Heart; heart left ventricle outflow tract obstruction; heart ventricle extrasystole; heart ventricle tachycardia; high risk patient; human; hypertrophic cardiomyopathy; Kardiologi; Klinisk medicin; limb lead QRS amplitude sum; male; Medical and Health Sciences; Medicin och hälsovetenskap; metoprolol; paediatric arrythmias; Pediatrics; Pediatrik; prediction; predictive value; predictor variable; priority journal; propranolol; QRS amplitude; risk; risk factor; Risk factors; risk reduction; sensitivity and specificity; septal thickness; ST segment depression; Studies; sudden cardiac death; sudden death; survival; Swedish citizen
• ISSN: 2053-3624; 2398-595X; 2053-3624
• DOI: 10.1136/openhrt-2017-000658

ObjectiveTo establish which risk factors are predictive for sudden death in hypertrophic cardiomyopathy (HCM) diagnosed in childhood.MethodsA Swedish national cohort of patients with HCM diagnosed 2 years (n=100), and features significantly more common in SD/CA group were further analysed with univariate and multivariate Cox hazard regression in the total cohort.ResultsRanked according to relative risk (RR) the ECG risk score >5 points had an RR of 46.5 (95% CI 6.6 to 331), sensitivity of 97% (83% to 100%) and specificity of 80% (71% to 88%) (p4.5: 9.9 (3.1 to 31.2); septal thickness ≥190% of upper limit of normal for age (septum in % of 95th centile for age (SEPPER) ≥190%): 7.9 (3.2 to 19.4); ventricular tachycardia: 9.1 (3.6 to 22.8); ventricular ectopics on exercise testing: 7.4 (2.7 to 20.2); and left ventricular outflow gradient (left ventricular outflow tract obstruction (LVOTO)) >50 mm Hg: 6.6 (4.0 to 11.0). Family history was non-significant. Multivariate Cox hazard analysis gives the following as early predictors: limb-lead QRS amplitude sum (p=0.020), SEPPER ≥190% (p