scholarly journals Risk of sudden cardiac death in childhood hypertrophic cardiomyopathy: Time to solve the mystery

2018 ◽  
Vol 8 (1) ◽  
Author(s):  
Gabrielle Norrish ◽  
Juan Pablo Kaski
Keyword(s):  
Hypertrophic Cardiomyopathy ◽  
Sudden Cardiac Death ◽  
Cardiac Death ◽  
Inborn Errors Of Metabolism ◽  
Ventricular Hypertrophy ◽  
Young Patients ◽  
Left Ventricular ◽  
Inborn Errors ◽  
True Prevalence ◽  
Malformation Syndromes

Hypertrophic cardiomyopathy (HCM) is defined as left ventricular hypertrophy in the absence of loading conditions sufficient to cause the observed abnormality. The true prevalence in childhood is unknown; the aetiology is more heterogeneous than that seen in adult populations, and includes inborn errors of metabolism, malformation syndromes and neuromuscular syndromes. However, one of the greatest clinical challenges in managing young patients with HCM is identifying those at greatest risk of sudden cardiac death.

Primary prevention of sudden cardiac death in hypertrophic cardiomyopathy

ESC CardioMed ◽  
2018 ◽  
pp. 2348-2351
Author(s):  
Barry J. Maron ◽  
Martin S. Maron
Keyword(s):  
Hypertrophic Cardiomyopathy ◽  
Primary Prevention ◽  
Sudden Cardiac Death ◽  
Left Ventricular Hypertrophy ◽  
Cardiac Death ◽  
Ventricular Hypertrophy ◽  
Left Ventricular ◽  
Intervention Selection ◽  
Initial Intervention ◽  
Subaortic Obstruction

Hypertrophic cardiomyopathy (HCM) has been regarded as the most common non-trauma-related cause of sudden cardiac death (SCD) in young people, including competitive athletes. The traditional clinical profile of HCM patients at highest risk of SCD is largely comprised of young asymptomatic patients, particularly children and adolescents. Prophylactic drug treatment to prevent SCD is ineffective and obsolete whereas the introduction of implantable cardioverter defibrillators (ICD) has changed the clinical course and natural history of the disease. The ICDs are largely responsible for reducing HCM-related mortality to 0.5%/year and have been shown to be effective despite the often complex HCM phenotype, including extreme and diverse patterns of left ventricular hypertrophy, dynamic subaortic obstruction, microvascular ischaemia, and diastolic dysfunction. An important principle related to ICD decisions in HCM patients surrounds the unpredictable nature of the arrhythmic substrate, often associated with substantial delays of 5–10 years between implant and initial intervention. Selection of patients most likely to benefit from primary prevention ICD therapy is based on non-invasive testing including echocardiography or cardiac magnetic resonance imaging, history taking, exercise testing, and ambulatory electrocardiogram (ECG). Conventional risk markers are (1) family history (one or more HCM-related SCDs in close relatives); (2) recent unexplained syncope; (3) multiple repetitive (or prolonged) non-sustained ventricular tachycardia on ambulatory ECG; (4) massive left ventricular hypertrophy (wall thickness ≥30 mm); and (5) hypotensive blood pressure response to exercise, although this is not a sole indication for ICD implants. ICD decisions within current risk stratification guidelines can be challenging, particularly in patients with just one risk factor or when available evidence is ambiguous or insufficient to assign SCD risk level with confidence.

P4457Cardiac screening is improved by the addition of vectorcardiography

2019 ◽  
Vol 40 (Supplement_1) ◽  
Author(s):  
D Cortez ◽  
H Morrell
Keyword(s):  
Hypertrophic Cardiomyopathy ◽  
Sudden Cardiac Death ◽  
Left Ventricular Hypertrophy ◽  
Cardiac Death ◽  
Ventricular Hypertrophy ◽  
Screening Program ◽  
Color Doppler ◽  
Left Ventricular ◽  
Screening Programs ◽  
Septal Defects

Abstract Background The most common cause of sudden cardiac death in the United State of America is hypertrophic cardiomyopathy, while other causes such as left ventricular hypertrophy, ion-channelopathies, and other causes are also significant. Screening programs typically involve electrocardiograms with mainly false positive results. Little data regarding EKG and echocardiographic screening combined have been published. Methods During 2016–2019, over 15000 patients were screened by the Heartfelt Cardiac screening program. Screening electrocardiograms and echocardiograms including parasternal short/long axis, 4-chamber views, coronary assessment with color doppler and subcostal views with color doppler for assessment of septal defects, were performed. Results 15,329 patients were screened under 35 years of age, mean age 17 years, with 36 hypertrophic cardiomyopathy patients, 61 borderline left ventricular hypertrophy patients noted, 30 patients with left ventricular dysfunction were noted, 47 patients with mitral valve prolapse, 27 patients with atrial septal defects, 39 patients with Wolf-Parkinson-White, 21patients with ideopathic dilated cardiomyopathy were discovered. Screening electrocardiography was helpful for Wolf-Parkinson-White identification, while echocardiogram was most helpful for detection of the above other defects. Screening EKG only identified 45% of those patients with myocardial disease (per above) with the modified Seattle Screening and ESC screening criteria. Conclusion Screening echocardiogram adds value for screening athletes under 35 years at risk for sudden cardiac death. Consideration of cost-effective echo screening should be considered as part of routine screening.

Supplementary role of left ventricular global longitudinal strain for predicting sudden cardiac death in hypertrophic cardiomyopathy

2021 ◽  
Author(s):  
Hyun-Jung Lee ◽  
Hyung-Kwan Kim ◽  
Sang Chol Lee ◽  
Jihoon Kim ◽  
Jun-Bean Park ◽  
...  
Keyword(s):  
Hypertrophic Cardiomyopathy ◽  
Sudden Cardiac Death ◽  
Risk Score ◽  
Primary Endpoint ◽  
Cardiac Death ◽  
Longitudinal Strain ◽  
Global Longitudinal Strain ◽  
Left Ventricular ◽  
C Statistic

Abstract Aims We investigated the prognostic role of left ventricular global longitudinal strain (LV-GLS) and its incremental value to established risk models for predicting sudden cardiac death (SCD) in patients with hypertrophic cardiomyopathy (HCM). Methods and results LV-GLS was measured with vendor-independent software at a core laboratory in a cohort of 835 patients with HCM (aged 56.3 ± 12.2 years) followed-up for a median of 6.4 years. The primary endpoint was SCD events, including appropriate defibrillator therapy, within 5 years after the initial evaluation. The secondary endpoint was a composite of SCD events, heart failure admission, heart transplantation, and all-cause mortality. Twenty (2.4%) and 85 (10.2%) patients experienced the primary and secondary endpoints, respectively. Lower absolute LV-GLS quartiles, especially those worse than the median (−15.0%), were associated with progressively higher SCD event rates (P = 0.004). LV-GLS was associated with an increased risk for the primary endpoint, independent of the LV ejection fraction, apical aneurysm, and 2014 European Society of Cardiology (ESC) risk score [adjusted hazard ratio (aHR) 1.14, 95% confidence interval (CI) 1.02–1.28] or 2011 American College of Cardiology/American Heart Association (ACC/AHA) risk factors (aHR 1.18, 95% CI 1.05–1.32). LV-GLS was also associated with a higher risk for the composite secondary endpoint (aHR 1.06, 95% CI 1.01–1.12). The addition of LV-GLS enhanced the performance of the ESC risk score (C-statistic 0.756 vs. 0.842, P = 0.007) and the 2011 ACC/AHA risk factor strategy (C-statistic 0.743 vs. 0.814, P = 0.007) for predicting SCD. Conclusion LV-GLS is an important prognosticator in patients with HCM and provides additional information to established risk stratification strategies for predicting SCD.

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.

Sympathetic overactivity and sudden cardiac death among hemodialysis patients with left ventricular hypertrophy

2010 ◽  
Vol 142 (1) ◽  
pp. 80-86 ◽  
Author(s):  
Masato Nishimura ◽  
Toshiko Tokoro ◽  
Masasya Nishida ◽  
Tetsuya Hashimoto ◽  
Hiroyuki Kobayashi ◽  
...  
Keyword(s):  
Sudden Cardiac Death ◽  
Left Ventricular Hypertrophy ◽  
Cardiac Death ◽  
Ventricular Hypertrophy ◽  
Hemodialysis Patients ◽  
Left Ventricular ◽  
Sympathetic Overactivity
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