Validation of the 2014 European Society of Cardiology guidelines risk prediction model for the primary prevention of sudden cardiac death in hypertrophic cardiomyopathy.
BACKGROUND
The recently released 2014 European Society of Cardiology guidelines of hypertrophic cardiomyopathy (HCM) use a new clinical risk prediction model for sudden cardiac death (SCD), based on the HCM Risk-SCD study. Our study is the first external and independent validation of this new risk prediction model.


RESULTS
The study population consisted of a consecutive cohort of 706 patients with HCM without prior SCD event, from 2 tertiary referral centers. The primary end point was a composite of SCD and appropriate implantable cardioverter-defibrillator therapy, identical to the HCM Risk-SCD end point. The 5-year SCD risk was calculated using the HCM Risk-SCD formula. Receiver operating characteristic curves and C-statistics were calculated for the 2014 European Society of Cardiology guidelines, and risk stratification methods of the 2003 American College of Cardiology/European Society of Cardiology guidelines and 2011 American College of Cardiology Foundation/American Heart Association guidelines. During follow-up of 7.7±5.3 years, SCD occurred in 42 (5.9%) of 706 patients (ages 49±16 years; 34% women). The C-statistic of the new model was 0.69 (95% CI, 0.57-0.82; P=0.008), which performed significantly better than the conventional risk factor models based on the 2003 guidelines (C-statistic of 0.55: 95% CI, 0.47-0.63; P=0.3), and 2011 guidelines (C-statistic of 0.60: 95% CI, 0.50-0.70; P=0.07).


CONCLUSIONS
The HCM Risk-SCD model improves the risk stratification of patients with HCM for primary prevention of SCD, and calculating an individual risk estimate contributes to the clinical decision-making process. Improved risk stratification is important for the decision making before implantable cardioverter-defibrillator implantation for the primary prevention of SCD.
From the Department of Cardiology, Thoraxcenter, Erasmus Medical Center, Rotterdam, The Netherlands (P.A.V., A.F.L.S., D.A.M.J.T., F.J.t.C., M.M.); Department of Cardiology, St. Antonius Hospital Nieuwegein, Nieuwegein, The Netherlands (M.L.); and Department of Cardiovascular Diseases of the University of Leuven, Leuven, Belgium (J.v.C., R.W.). p.vriesendorp@erasmusmc.nl.
Pieter A
PA
Vriesendorp
From the Department of Cardiology, Thoraxcenter, Erasmus Medical Center, Rotterdam, The Netherlands (P.A.V., A.F.L.S., D.A.M.J.T., F.J.t.C., M.M.); Department of Cardiology, St. Antonius Hospital Nieuwegein, Nieuwegein, The Netherlands (M.L.); and Department of Cardiovascular Diseases of the University of Leuven, Leuven, Belgium (J.v.C., R.W.).
Arend F L
AF
Schinkel
From the Department of Cardiology, Thoraxcenter, Erasmus Medical Center, Rotterdam, The Netherlands (P.A.V., A.F.L.S., D.A.M.J.T., F.J.t.C., M.M.); Department of Cardiology, St. Antonius Hospital Nieuwegein, Nieuwegein, The Netherlands (M.L.); and Department of Cardiovascular Diseases of the University of Leuven, Leuven, Belgium (J.v.C., R.W.).
Max
M
Liebregts
From the Department of Cardiology, Thoraxcenter, Erasmus Medical Center, Rotterdam, The Netherlands (P.A.V., A.F.L.S., D.A.M.J.T., F.J.t.C., M.M.); Department of Cardiology, St. Antonius Hospital Nieuwegein, Nieuwegein, The Netherlands (M.L.); and Department of Cardiovascular Diseases of the University of Leuven, Leuven, Belgium (J.v.C., R.W.).
Dominic A M J
DA
Theuns
From the Department of Cardiology, Thoraxcenter, Erasmus Medical Center, Rotterdam, The Netherlands (P.A.V., A.F.L.S., D.A.M.J.T., F.J.t.C., M.M.); Department of Cardiology, St. Antonius Hospital Nieuwegein, Nieuwegein, The Netherlands (M.L.); and Department of Cardiovascular Diseases of the University of Leuven, Leuven, Belgium (J.v.C., R.W.).
Johan
J
van Cleemput
From the Department of Cardiology, Thoraxcenter, Erasmus Medical Center, Rotterdam, The Netherlands (P.A.V., A.F.L.S., D.A.M.J.T., F.J.t.C., M.M.); Department of Cardiology, St. Antonius Hospital Nieuwegein, Nieuwegein, The Netherlands (M.L.); and Department of Cardiovascular Diseases of the University of Leuven, Leuven, Belgium (J.v.C., R.W.).
Folkert J
FJ
Ten Cate
From the Department of Cardiology, Thoraxcenter, Erasmus Medical Center, Rotterdam, The Netherlands (P.A.V., A.F.L.S., D.A.M.J.T., F.J.t.C., M.M.); Department of Cardiology, St. Antonius Hospital Nieuwegein, Nieuwegein, The Netherlands (M.L.); and Department of Cardiovascular Diseases of the University of Leuven, Leuven, Belgium (J.v.C., R.W.).
Rik
R
Willems
From the Department of Cardiology, Thoraxcenter, Erasmus Medical Center, Rotterdam, The Netherlands (P.A.V., A.F.L.S., D.A.M.J.T., F.J.t.C., M.M.); Department of Cardiology, St. Antonius Hospital Nieuwegein, Nieuwegein, The Netherlands (M.L.); and Department of Cardiovascular Diseases of the University of Leuven, Leuven, Belgium (J.v.C., R.W.).
Michelle
M
Michels
D000328:Adult; D000368:Aged; D002309:Cardiology; D002312:Cardiomyopathy, Hypertrophic / Q000150:complications* / Q000175:diagnosis; D016757:Death, Sudden, Cardiac / Q000453:epidemiology / Q000209:etiology / Q000517:prevention & control*; D005060:Europe / Q000453:epidemiology; D005260:Female; D006801:Humans; D015994:Incidence; D008297:Male; D008875:Middle Aged; D017410:Practice Guidelines as Topic / Q000592:standards*; D011322:Primary Prevention / Q000592:standards*; D012372:ROC Curve; D018570:Risk Assessment / Q000379:methods*; D012307:Risk Factors; D012955:Societies, Medical; D015996:Survival Rate / Q000639:trends
cardiac; cardiomyopathy; death; hypertrophic; sudden