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.

N-terminal pro-brain natriuretic peptide and sudden cardiac death in hypertrophic cardiomyopathy

Heart ◽  
2020 ◽  
pp. heartjnl-2020-317701
Author(s):  
Guixin Wu ◽  
Jie Liu ◽  
Shuiyun Wang ◽  
Shiqin Yu ◽  
Ce Zhang ◽  
...  
Keyword(s):  
Hypertrophic Cardiomyopathy ◽  
Sudden Cardiac Death ◽  
Brain Natriuretic Peptide ◽  
Natriuretic Peptide ◽  
Cardiac Death ◽  
Cardiac Fibrosis ◽  
Discrimination Performance ◽  
Net Reclassification Improvement ◽  
C Statistic ◽  
Increased Risk

ObjectiveElevated levels of N-terminal pro-brain natriuretic peptide (NT-proBNP) are associated with heart failure-related death in hypertrophic cardiomyopathy (HCM), but the relationship between NT-proBNP level and sudden cardiac death (SCD) in HCM remains undefined.MethodsThe study prospectively enrolled 977 unrelated patients with HCM with available NT-proBNP results who were prospectively enrolled and followed for 3.0±2.1 years. The Harrell’s C-statistic under the receiver operating characteristic curve was calculated to evaluate discrimination performance. A combination model was constructed by adding NT-proBNP tertiles to the HCM Risk-SCD model. The correlation between log NT-proBNP level and cardiac fibrosis as measured by late gadolinium enhancement (LGE) or Masson’s staining was analysed.ResultsDuring follow-up, 29 patients had SCD. Increased log NT-proBNP levels were associated with an increased risk of SCD events (adjusted HR 22.27, 95% CI 10.93 to 65.63, p<0.001). The C-statistic of NT-proBNP in predicting SCD events was 0.80 (p<0.001). The combined model significantly improved the predictive efficiency of the HCM Risk-SCD model from 0.72 to 0.81 (p<0.05), with a relative integrated discrimination improvement of 0.002 (p<0.001) and net reclassification improvement of 0.67 (p<0.001). Furthermore, log NT-proBNP levels were significantly correlated with cardiac fibrosis as detected either by LGE (r=0.257, p<0.001) or by Masson’s trichrome staining in the myocardium (r=0.198, p<0.05).ConclusionNT-proBNP is an independent predictor of SCD in patients with HCM and may help with risk stratification of this disease.

Abstract 11447: Low Incidence of Appropriate Therapy Following Defibrillator Implantation for Primary Prevention of Sudden Cardiac Death in Hypertrophic Cardiomyopathy

Circulation ◽  
2015 ◽  
Vol 132 (suppl_3) ◽  
Author(s):  
Amalie C Thavikulwat ◽  
Todd T Tomson ◽  
Bradley P Knight ◽  
Robert O Bonow ◽  
Lubna Choudhury
Keyword(s):  
Risk Factors ◽  
Risk Factor ◽  
Hypertrophic Cardiomyopathy ◽  
Primary Prevention ◽  
Sudden Cardiac Death ◽  
Secondary Prevention ◽  
Cardiac Death ◽  
Icd Implantation ◽  
Icd Therapy ◽  
Appropriate Therapy

Introduction: Hypertrophic cardiomyopathy (HCM) is a leading cause of sudden cardiac death (SCD) in young adults. Implantable cardioverter defibrillators (ICD) effectively terminate ventricular tachycardia (VT) and fibrillation (VF) that cause SCD, but the reported prevalence of and patient characteristics leading to appropriate ICD therapy in HCM have been variable. Hypothesis: We hypothesized that some risk factors may be more prevalent than others in patients with HCM who receive appropriate ICD therapy and that the overall incidence of appropriate therapy may be lower than that reported previously. Methods: We retrospectively studied all patients with HCM who were treated with ICDs at our referral center from 2000-2013 to determine the rates of appropriate and inappropriate ICD therapies. Results: Of 1136 patients with HCM, we identified 135 who underwent ICD implantation (125 for primary and 10 for secondary prevention), aged 18-81 years (mean 48±17) at the time of implantation. The mean follow-up time was 5.2±4.5 years. Appropriate ICD intervention occurred in 20 of 135 patients (2.8%/year) by providing a shock or antitachycardia pacing in response to VT or VF. The annual rate of appropriate ICD therapy was 2.4%/year for primary and 7.2%/year for secondary prevention devices. Commonly used risk factors were equally prevalent among patients who received appropriate therapy and those who did not; furthermore, the likelihood of receiving appropriate therapy in the presence of each risk factor was similar (Figure). Inappropriate ICD therapy occurred in 27 patients (3.8%/year). Conclusions: ICDs provide clear benefit to patients who experience life-threatening arrhythmias, particularly those being treated for secondary prevention. However, the appropriate therapy rate for primary prevention was lower than previously reported, and no single risk factor appeared to have stronger association with appropriate ICD therapy than others.

scholarly journals Sudden Cardiac Death Risk in Downhill Skiers and Mountain Hikers and Specific Prevention Strategies

2021 ◽  
Vol 18 (4) ◽  
pp. 1621
Author(s):  
Josef Niebauer ◽  
Martin Burtscher
Keyword(s):  
Risk Factors ◽  
Risk Factor ◽  
Sudden Cardiac Death ◽  
High Intensity ◽  
Cardiac Death ◽  
High Intensity Exercise ◽  
Systemic Hypertension ◽  
Physical Strain ◽  
Mountainous Regions ◽  
Downhill Skiing

Sudden cardiac death (SCD) still represents an unanticipated and catastrophic event eliciting from cardiac causes. SCD is the leading cause of non-traumatic deaths during downhill skiing and mountain hiking, related to the fact that these sports are very popular among elderly people. Annually, more than 40 million downhill skiers and mountain hikers/climbers visit mountainous regions of the Alps, including an increasing number of individuals with pre-existing chronic diseases. Data sets from two previously published case-control studies have been used to draw comparisons between the SCD risk of skiers and hikers. Data of interest included demographic variables, cardiovascular risk factors, medical history, physical activity, and additional symptoms and circumstances of sudden death for cases. To establish a potential connection between the SCD risk and sport-specific physical strain, data on cardiorespiratory responses to downhill skiing and mountain hiking, assessed in middle-aged men and women, have been included. It was demonstrated that previous myocardial infarction (MI) (odds ratio; 95% CI: 92.8; 22.8–379.1; p < 0.001) and systemic hypertension (9.0; 4.0–20.6; p < 0.001) were predominant risk factors for SCD in skiers, but previous MI (10.9; 3.8–30.9; p < 0.001) and metabolic disorders like hypercholesterolemia (3.4; 2.2–5.2; p < 0.001) and diabetes (7.4; 1.6–34.3; p < 0.001) in hikers. More weekly high-intensity exercise was protective in skiers (0.17; 0.04–0.74; p = 0.02), while larger amounts of mountain sports activities per year were protective in hikers (0.23; 0.1–0.4; <0.001). In conclusion, previous MI history represents the most important risk factor for SCD in recreational skiers and hikers as well, and adaptation to high-intensity exercise is especially important to prevent SCD in skiers. Moreover, the presented differences in risk factor patterns for SCDs and discussed requirements for physical fitness in skiers and hikers will help physicians to provide specifically targeted advice.

scholarly journals Vital exhaustion and sudden cardiac death in the Atherosclerosis Risk in Communities Study

Heart ◽  
2017 ◽  
Vol 104 (5) ◽  
pp. 423-429 ◽  
Author(s):  
Brittany M Bogle ◽  
Nona Sotoodehnia ◽  
Anna M Kucharska-Newton ◽  
Wayne D Rosamond
Keyword(s):  
Risk Factors ◽  
Sudden Cardiac Death ◽  
Cardiac Death ◽  
Disease Risk ◽  
Ventricular Tachyarrhythmia ◽  
Atherosclerosis Risk In Communities ◽  
Vital Exhaustion ◽  
Increased Risk ◽  
Atherosclerosis Risk ◽  
Aric Study

ObjectiveVital exhaustion (VE), a construct defined as lack of energy, increased fatigue and irritability, and feelings of demoralisation, has been associated with cardiovascular events. We sought to examine the relation between VE and sudden cardiac death (SCD) in the Atherosclerosis Risk in Communities (ARIC) Study.MethodsThe ARIC Study is a predominately biracial cohort of men and women, aged 45–64 at baseline, initiated in 1987 through random sampling in four US communities. VE was measured using the Maastricht questionnaire between 1990 and 1992 among 13 923 individuals. Cox proportional hazards models were used to examine the hazard of out-of-hospital SCD across tertiles of VE scores.ResultsThrough 2012, 457 SCD cases, defined as a sudden pulseless condition presumed due to a ventricular tachyarrhythmia in a previously stable individual, were identified in ARIC by physician record review. Adjusting for age, sex and race/centre, participants in the highest VE tertile had an increased risk of SCD (HR 1.48, 95% CI 1.17 to 1.87), but these findings did not remain significant after adjustment for established cardiovascular disease risk factors (HR 0.94, 95% CI 0.73 to 1.20).ConclusionsAmong participants of the ARIC study, VE was not associated with an increased risk for SCD after adjustment for cardiovascular risk factors.

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.

scholarly journals The association of sudden cardiac death with inflammation and other traditional risk factors

2008 ◽  
Vol 74 (10) ◽  
pp. 1335-1342 ◽  
Author(s):  
Rulan S. Parekh ◽  
Laura C. Plantinga ◽  
W.H. Linda Kao ◽  
Lucy A. Meoni ◽  
Bernard G. Jaar ◽  
...  
Keyword(s):  
Risk Factors ◽  
Sudden Cardiac Death ◽  
Cardiac Death ◽  
Traditional Risk Factors

scholarly journals The Potential of Late Gadolinium Enhancement to Serve as a Predictor of Ventricular Arrhythmias in Hypertrophic Cardio-myopathy Patients

2016 ◽  
Vol 8 (1) ◽  
pp. 1-11
Author(s):  
Thomas D. Gossios ◽  
Georgios K. Efthimiadis ◽  
Theodoros D. Karamitsos ◽  
Thomas Zegkos ◽  
Vasilios G. Athyros ◽  
...  
Keyword(s):  
Late Gadolinium Enhancement ◽  
Hypertrophic Cardiomyopathy ◽  
Sudden Cardiac Death ◽  
Sudden Death ◽  
Cardiac Death ◽  
Large Scale ◽  
Small Subset ◽  
Gadolinium Enhancement ◽  
Inherited Cardiomyopathy ◽  
Increased Risk

Hypertrophic cardiomyopathy, the most common inherited cardiomyopathy is well known to be the leading cause of sudden cardiac death in young people. However, amongst the population of patients, a small subset bears increased risk of sudden cardiac death and would benefit from implantation of a defibrillator, currently recognized utilizing a series of established risk factors. This risk stratification model is hampered by low positive predictive value. Therefore, novel predictors of sudden death are sought. The advent of cardiac magnetic resonance and late gadolinium enhancement has allowed accurate quantification of regional fibrosis, a key element of hypertrophic cardiomyopathy, pathophysiologically linked to increased arrhythmogenicity. We sought to review currently available data on the utility of late gadolinium enhancement to serve as a novel predictor of arrhythmias and sudden death. In conclusion, significantly diverse methodological approaches and subsequent findings between available studies on the topic have hampered such use, highlighting the need for uniformly designed large scale, prospective studies in order to clarify which aspects of myocardial fibrosis could serve as predictors of arrhythmic events.

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