scholarly journals Risk stratification for sudden cardiac death in hypertrophic cardiomyopathy: systematic review of clinical risk markers

EP Europace ◽  
2010 ◽  
Vol 12 (3) ◽  
pp. 313-321 ◽  
Author(s):  
Imke Christiaans ◽  
Klaartje van Engelen ◽  
Irene M. van Langen ◽  
Erwin Birnie ◽  
Gouke J. Bonsel ◽  
...  
Keyword(s):  
Systematic Review ◽  
Hypertrophic Cardiomyopathy ◽  
Sudden Cardiac Death ◽  
Risk Stratification ◽  
Cardiac Death ◽  
Risk Markers ◽  
Clinical Risk

Abstract 14760: Myocardial Scarring by Magnetic Resonance Predicts Sudden Cardiac Death in Pediatric Patients With Hypertrophic Cardiomyopathy

Circulation ◽  
2020 ◽  
Vol 142 (Suppl_3) ◽  
Author(s):  
Lars Grosse-Wortmann ◽  
Laurine van der Wal ◽  
Aswathy Vaikom House ◽  
Lee Benson ◽  
Raymond Chan
Keyword(s):  
Risk Factors ◽  
Risk Factor ◽  
Hypertrophic Cardiomyopathy ◽  
Sudden Cardiac Death ◽  
Cardiac Death ◽  
Pediatric Patients ◽  
Risk Markers ◽  
C Statistic ◽  
Increased Risk ◽  
Traditional Risk Factors

Introduction: Cardiovascular magnetic resonance (CMR) with late gadolinium enhancement (LGE) has been shown to be an independent predictor of sudden cardiac death (SCD) in adults with hypertrophic cardiomyopathy (HCM). The clinical significance of LGE in pediatric HCM patients is unknown. Hypothesis: LGE improves the SCD risk prediction in children with HCM. Methods: We retrospectively analyzed the CMR images and reviewed the outcomes pediatric HCM patients. Results: Amongst the 720 patients from 30 centers, 73% were male, with a mean age of 14.2±4.8 years. During a mean follow up of 2.6±2.7 years (range 0-14.8 years), 34 experienced an episode of SCD or equivalent. LGE (Figure 1A) was present in 34%, with a mean burden of 14±21g, or 2.5±8.2g/m2 (6.2±7.7% of LV myocardium). The presence of ≥1 adult traditional risk factor (family history of SCD, syncope, LV thickness >30mm, non-sustained ventricular tachycardia on Holter) was associated with an increased risk of SCD (HR=4.6, p<0.0001). The HCM Risk-Kids score predicted SCD (p=0.002). The presence of LGE was strongly associated with an increased risk (HR=3.8, p=0.0003), even after adjusting for traditional risk factors (HR adj =3.2, p=0.003) or the HCM Risk-Kids score (HR adj =3.5, p=0.003). Furthermore, the burden of LGE was associated with increased risk (HR=2.1/10% LGE, p<0.0001). LGE burden remained independently associated with an increased risk for SCD after adjusting for traditional risk factors (HRadj=1.5/10% LGE, p=0.04) or HCM Risk-Kids (HRadj=1.9/10% LGE, p=0.0018, Figure 1B). The addition of LGE burden improved the predictive model using traditional risk markers (C statistic 0.67 vs 0.77, p=0.003) and HCM Risk-Kids (C statistic 0.68 vs 0.74, p=0.045). Conclusions: Quantitative LGE is an independent risk factor for SCD in pediatric patients with HCM and improves the performance of traditional risk markers and the HCM Risk-Kids Score for SCD risk stratification in this population.

scholarly journals A SYSTEMATIC REVIEW FOR SUDDEN CARDIAC DEATH IN HYPERTROPHIC CARDIOMYOPATHY PATIENTS WITH MYOCARDIAL FIBROSIS: A CMR LGE STUDY

2012 ◽  
Vol 59 (13) ◽  
pp. E1228 ◽  
Author(s):  
Sahadev T Reddy ◽  
Antonio Tito Paladino Filho ◽  
Nackle Jibran Silva ◽  
Mark Doyle ◽  
June Yamrozik ◽  
...  
Keyword(s):  
Systematic Review ◽  
Hypertrophic Cardiomyopathy ◽  
Sudden Cardiac Death ◽  
Myocardial Fibrosis ◽  
Cardiac Death

Hypertrophic cardiomyopathy: prevention of sudden cardiac death

2018 ◽  
Author(s):  
Constantinos O’Mahony
Keyword(s):  
Hypertrophic Cardiomyopathy ◽  
Sudden Cardiac Death ◽  
Risk Stratification ◽  
Cardiac Death ◽  
Clinical Care ◽  
Psychological Impact ◽  
Ventricular Outflow Tract ◽  
Left Ventricular ◽  
Icd Implantation ◽  
European Society Of Cardiology

Sudden cardiac death (SCD) secondary to ventricular arrhythmias is the most common mode of death in hypertrophic cardiomyopathy (HCM) and can be effectively prevented with an implantable cardioverter defibrillator (ICD). The risk of SCD in HCM relates to the severity of the phenotype and regular risk stratification is an integral part of routine clinical care. For the primary prevention of SCD, risk stratification involves the assessment of seven readily available clinical parameters (age, maximal left ventricular wall thickness, left atrial diameter, left ventricular outflow tract gradient, non-sustained ventricular tachycardia, unexplained syncope, and family history of SCD) which are used to estimate the risk of SCD within 5 years of clinical evaluation using a statistical risk prediction model (HCM Risk-SCD). The 2014 European Society of Cardiology Guidelines provide a framework to aid clinical decisions and consider patients with a 5-year risk of SCD of less than 4% as low risk and recommend regular assessment while those with a risk of 6% or higher should be considered for an ICD. In patients with an intermediate risk (4% to <6%) ICD implantation may also be considered after taking into account age, co-morbid conditions, socioeconomic factors, and the psychological impact of therapy. Survivors of ventricular fibrillation arrest should receive an ICD for secondary prevention unless their life expectancy is less than 1 year. Following device implantation, patients should be followed up for device- and disease-related complications, particularly heart failure and cerebrovascular disease.

Importance of Sudden Cardiac Death Risk Stratification in Hypertrophic Cardiomyopathy

2015 ◽  
Vol 68 (6) ◽  
pp. 544
Author(s):  
Marina Martínez-Moreno ◽  
Vicente Climent ◽  
Antonio García-Honrubia ◽  
Francisco Marín
Keyword(s):  
Hypertrophic Cardiomyopathy ◽  
Sudden Cardiac Death ◽  
Risk Stratification ◽  
Cardiac Death ◽  
Death Risk ◽  
Sudden Cardiac Death Risk

scholarly journals 649Reappraisal of the value of electrophysiological study in sudden cardiac death risk stratification in hypertrophic cardiomyopathy

EP Europace ◽  
2017 ◽  
Vol 19 (suppl_3) ◽  
pp. iii128-iii128
Author(s):  
C-K Antoniou ◽  
K. Gatzoulis ◽  
S. Georgopoulos ◽  
A. Anastasakis ◽  
A. Theopistou ◽  
...  
Keyword(s):  
Hypertrophic Cardiomyopathy ◽  
Sudden Cardiac Death ◽  
Risk Stratification ◽  
Cardiac Death ◽  
Electrophysiological Study ◽  
Death Risk ◽  
Sudden Cardiac Death Risk

scholarly journals CURRENT RECOMMENDATIONS ON RISK STRATIFICATION AND SUDDEN CARDIAC DEATH PREVENTION IN PATIENTS WITH HYPERTROPHIC CARDIOMYOPATHY (BASED ON THE 2011 RECOMMENDATIONS BY THE AMERICAN COLLEGE OF CARDIOLOGY / AMERICAN HEART ASSOCIATION)

2013 ◽  
Vol 12 (1) ◽  
pp. 73-79
Author(s):  
E. V. Privalova ◽  
V. Yu. Kaplunova ◽  
N. V. Khabarova ◽  
G. A. Shakaryants ◽  
Yu. N. Belenkov
Keyword(s):  
Hypertrophic Cardiomyopathy ◽  
Sudden Cardiac Death ◽  
Risk Stratification ◽  
Cardiac Death ◽  
Therapeutic Strategy ◽  
Heart Association ◽  
Preventive Treatment ◽  
Risk Levels ◽  
Life Saving ◽  
Clinical Scenarios

One of the clinical scenarios of hypertrophic cardiomyopathy (HCMP) is sudden cardiac death (SCD). The stratification of SCD risk is the key component of defining the therapeutic strategy in HCMP patients. Timely preventive treatment is the only life-saving intervention in patients with high SCD risk. The available clinical evidence suggests that SCD risk stratification is an effective algorithm for determining the need for preventive treatment. The latter should be individualised, based on the risk levels in each patient. 

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