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.

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 1228 New predictors of sudden cardiac death in hypertrophic cardiomyopathy

2020 ◽  
Vol 21 (Supplement_1) ◽  
Author(s):  
D M Adamczak ◽  
A Rogala ◽  
M Antoniak ◽  
Z Oko-Sarnowska
Keyword(s):  
Risk Factors ◽  
Hypertrophic Cardiomyopathy ◽  
Sudden Cardiac Death ◽  
Cardiac Death ◽  
Left Ventricular ◽  
Volume Index ◽  
Lv Function ◽  
Icd Implantation ◽  
Significant Difference

Abstract BACKGROUND Hypertrophic cardiomyopathy (HCM) is a heart disease characterized by hypertrophy of the left ventricular myocardium. HCM is the most common cause of sudden cardiac death (SCD) in young people and competitive athletes due to fatal ventricular arrhythmias. However, in most patients, HCM has a benign course. That is why it is of utmost importance to properly evaluate patients and identify those who would benefit from a cardioverter-defibrillator (ICD) implantation. The HCM SCD-Risk Calculator is a useful tool for estimating the risk of SCD. The parameters included in the model at evaluation are: age, maximum left ventricular (LV) wall thickness, left atrial (LA) dimension, maximum gradient in left ventricular outflow tract, family history of SCD, non-sustained ventricular tachycardia (nsVT) and unexplained syncope. Nevertheless, there is potential to improve and optimize the effectiveness of this tool in clinical practice. Therefore, the following new risk factors are proposed: LV global longitudinal strain (GLS), LV average strain (ASI) and LA volume index (LAVI). GLS and ASI are sensitive and noninvasive methods of assessing LV function. LAVI more accurately characterizes the size of the left atrium in comparison to the LA dimension. METHODS 252 HCM patients (aged 20-88 years, of which 49,6% were men) treated in our Department from 2005 to 2018, were examined. The follow-up period was 0-13 years (average: 3.8 years). SCD was defined as sudden cardiac arrest (SCA) or an appropriate ICD intervention. All patients underwent an echocardiographic examination. The medical and family histories were collected and ICD examinations were performed. RESULTS 76 patients underwent an ICD implantation during the follow-up period. 20 patients have reached an SCD end-point. 1 patient died due to SCA and 19 had an appropriate ICD intervention. There were statistically significant differences of GLS and ASI values between SCD and non-SCD groups; p = 0.026389 and p = 0.006208, respectively. The average GLS in the SCD group was -12.4% ± 3.4%, and -15.1% ± 3.5% in the non-SCD group. The average ASI values were -9.9% ± 3.8% and -12.4% ± 3.5%, respectively. There was a statistically significant difference between LAVI values in SCD and non-SCD groups; p = 0.005343. The median LAVI value in the SCD group was 45.7 ml/m2 and 37.6 ml/m2 in the non-SCD group. The ROC curves showed the following cut-off points for GLS, ASI and LAVI: -13.8%, -13.7% and 41 ml/m2, respectively. Cox’s proportional hazards model for the parameters used in the Calculator was at the borderline of significance; p = 0.04385. The model with new variables (GLS and LAVI instead of LA dimension) was significant; p = 0.00094. The important factors were LAVI; p = 0.000075 and nsVT; p = 0.012267. CONCLUSIONS The proposed new SCD risk factors were statistically significant in the study population and should be taken into account when considering ICD implantation.

Secondary prevention of sudden cardiac death in hypertrophic cardiomyopathy

ESC CardioMed ◽  
2018 ◽  
pp. 2351-2354
Author(s):  
Wilhelm Haverkamp ◽  
Philipp Attanasio
Keyword(s):  
Ventricular Tachycardia ◽  
Hypertrophic Cardiomyopathy ◽  
Sudden Cardiac Death ◽  
Secondary Prevention ◽  
Cardiac Death ◽  
Recurrent Events ◽  
Sudden Cardiac Arrest ◽  
Heart Association ◽  
Level Of Evidence ◽  
Icd Implantation

The European Society of Cardiology and the American Heart Association guidelines recommend implantable cardioverter defibrillator (ICD) implantation in patients with hypertrophic cardiomyopathy who survived sudden cardiac arrest due to ventricular tachycardia or ventricular fibrillation or who experienced spontaneously occurring ventricular tachycardia causing syncope or haemodynamic compromise (class I indication, level of evidence B). These recommendations are based on several non-randomized trials which have shown that these patients remain at high risks of recurrent events. However, ICD therapy is associated with inappropriate shocks and device-related complications. In this chapter, the efficacy as well as the associated risks of treating hypertrophic cardiomyopathy patients with ICDs for secondary prevention of sudden cardiac death are discussed. Additionally, the chapter describes selection and appropriate programming of the devices.

P887Atrial fibrillation is associated with sudden cardiac death risk factors in patients with hypertrophic cardiomyopathy

2019 ◽  
Vol 40 (Supplement_1) ◽  
Author(s):  
I A Marcuschamer ◽  
O Zusman ◽  
S Schwartzenberg ◽  
M Vaturi ◽  
Y Shapira ◽  
...  
Keyword(s):  
Risk Factors ◽  
Ventricular Tachycardia ◽  
Hypertrophic Cardiomyopathy ◽  
Sudden Cardiac Death ◽  
Cardiac Death ◽  
Nearest Neighbor ◽  
Nyha Class ◽  
Left Ventricular ◽  
Similar Distribution ◽  
Icd Implantation

Abstract Background Atrial Fibrillation (AF) is prevalent in a fourth of patients with Hypertrophic Cardiomyopathy (HCM), but its clinical impact in these patients remains ill-defined. Aim To compare clinical characteristics in HCM patients with vs without AF and assess indirectly potential sudden cardiac death (SCD) risk. Methods Retrospective study in a single tertiary referral HCM center. Patients with HCM and AF were compared with matched controlled HCM patients without AF. NYHA class was assessed by a single physician. Propensity score matching was performed with a ratio of 2:1 by nearest neighbor with adjustment for age, sex, and left ventricular tract obstruction (LVOTO). Ordinal regression was used with NYHA as outcome. Results Among 298 patients with HCM, 68 patients (22.8%) had AF. After propensity matching, 66 patients with AF and 112 without AF had similar distribution of age (67.1 vs. 65.1 years), gender (57.6% vs. 61% males) and Basal Surface Area (1.88 vs. 1.87 m2) respectively. The prevalence of LVOTO (57.6% vs. 58.5%) and apical hypertrophy (19.7% vs 19.5%) was similar in the two groups. Cardiac risk factors including Hypertension (60.6% vs. 60.2%) and Diabetes Mellitus (15.2 vs. 20.3%) were similar in both groups. AF patients were diagnosed with HCM at a younger age than patients without HCM (48.5 vs. 55 years; p=0.01). HCM patients with AF had significantly lower LVOT gradients compared with patients without AF (28.1 mmHg vs 47.4 mmHg, p=0.005), had a higher prevalence of non-sustained ventricular tachycardia (39.4% vs. 9.4%; p<0.01), and ventricular tachycardia (9.1% vs 1.7%; p<0.04) and were more likely to have undergone implantation of an internal cardioverter defibrillator (ICD) (23.1% vs. 8.5%; p=0.001), respectively. Dyspnea was the most prevalent symptom in both groups (51.1% and 46.6% in AF and non-AF respectively). NYHA Class was similar in both groups: 1.88±0.69 in patients with AF vs. 1.73±0.74 in patients without AF (p=0.17). NYHA class did not differ in 26 patients with chronic persistent AF vs. 42 patients with paroxysmal AF (being in sinus rhythm at evaluation). Conclusion AF does not seem to impact functional level class in patients with HCM, but carries a higher burden of sudden cardiac death prognostic factors, incurring a higher rate of ICD implantation. Acknowledgement/Funding None

scholarly journals Prevention of sudden cardiac death by the implantable cardioverter-defibrillator in adults with inherited heart disease. long term follow-up

EP Europace ◽  
2021 ◽  
Vol 23 (Supplement_3) ◽  
Author(s):  
M Herruzo Rojas ◽  
A Gomez Lopez ◽  
MA Martin Toro ◽  
PJ Gonzalez Perez ◽  
FJ Morales Ponce
Keyword(s):  
Heart Disease ◽  
Genetic Testing ◽  
Hypertrophic Cardiomyopathy ◽  
Primary Prevention ◽  
High Risk ◽  
Sudden Cardiac Death ◽  
Cardiac Death ◽  
Supraventricular Arrhythmias ◽  
Icd Implantation

Abstract Funding Acknowledgements Type of funding sources: None. BACKGROUND Inherited heart diseases include all inheritable channelopathies or cardiomyopathies. Sudden cardiac death (SCD) might be the first and last clinical presentation. ICD implantation  is established as a therapeutic tool according to risk criteria. OBJETIVES The aim of this study is to analyze the population characteristics in ICD/ CRT-ICD recipients in patients with inherited heart disease and establish possible risk predictor factors of arrhytmogenic events during follow-up. MÉTHODS: This is a prospective single-center registry. We included all patients from January1, 2012, to December 31, 2020 who subsequently underwent ICD/ ICD-CRT implantation. RESULTS A total of 172 patients were included with a mean age of 60.47 ± 13,1 years and a mean follow-up duration of 49.71 ± 41.8 months. The most frequent underlying cardiac condition was dilated cardiomyopathy (58.1%), followed by hypertrophic cardiomyopathy (15.7%), arrythmogenic cardiomyopathy (2.3%), long QT syndrome (1.7%), Brugada syndrome (1.2%), catecholaminergic (2.9%) , idiopathic (7%) and others (11%). 89 ICDs (51.7%) and 83 ICD-CRTs (48.2%) were implanted for primary prevention of SCD. During follow-up, 13.4% of the patients experienced arrhythmogenic events. Only 4.1% of the patients experienced inappropriate shock, secondary to supraventricular arrhythmias all of them. Genetic testing was done in only 14.1% of our patients. In the 27 cases of hypertrophic cardiomyopathy, 25 (92.6%) underwent ICD implantation for primary prevention. 14 patients (51.9%) had family history of SCD, 8 (29.6%) had unexplained syncope and 16 (59.3%) had ventricular tachycardia in the Holter monitoring. The mean left atrial size was 44.15 ± 7.2mm, mean maximum LV-wall thickness 22.85 ± 5,25mm, and a mean LVOT gradient of 30.56 ± 31.3mmHg, with a mean actual HCM SCD-score of SCD over 5 years of 5.84 ± 3.53%. According to traditional parameters, 100% of the HCM sample meet criteria for ICD implantation in primary prevention compared to 66.7% according to the new score. During follow-up 2 patients (7.4% ) experienced arrhythmogenic events, and 3 patients (11.1%) had inappropriate shocks secondary to supraventricular arrhythmias. Genetic testing was done in 22.2% of the patients and was positive for a known pathogenic mutation in half of the cases. None of the parameters evaluated in the current score, a high risk of SCD according to it, having old high risk criteria or implanted of ICD in secondary prevention were related in our study with more arrhytmogenic events. CONCLUSIONS ICD implatation for primary prevention is the main indication in our population. Either classic factors or the new HCM SCD-score were no related in our study with the presence of arrhytmogenic events during follow-up.

Long-term Clinical Follow-up of Japanese Heart Failure Patients Received ICD Therapy for Primary Prevention of Sudden Cardiac Death

2008 ◽  
Vol 14 (7) ◽  
pp. S140-S141
Author(s):  
Kenji Ando ◽  
Yoshimitsu Soga ◽  
Masahiko Goya ◽  
Shinichi Shirai ◽  
Shinya Nagayama ◽  
...  
Keyword(s):  
Heart Failure ◽  
Primary Prevention ◽  
Sudden Cardiac Death ◽  
Cardiac Death ◽  
Icd Therapy ◽  
Heart Failure Patients

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.

Worldwide differences in primary prevention implantable cardioverter defibrillator utilization and outcomes in hypertrophic cardiomyopathy

2021 ◽  
Author(s):  
Victor Nauffal ◽  
Peter Marstrand ◽  
Larry Han ◽  
Victoria N Parikh ◽  
Adam S Helms ◽  
...  
Keyword(s):  
Hypertrophic Cardiomyopathy ◽  
Primary Prevention ◽  
Implantable Cardioverter Defibrillator ◽  
Cox Proportional Hazards ◽  
Icd Implantation ◽  
Icd Therapy ◽  
Cardioverter Defibrillator ◽  
Significant Difference ◽  
Implantation Rates ◽  
The Impact

Abstract Aims  Risk stratification algorithms for sudden cardiac death (SCD) in hypertrophic cardiomyopathy (HCM) and regional differences in clinical practice have evolved over time. We sought to compare primary prevention implantable cardioverter defibrillator (ICD) implantation rates and associated clinical outcomes in US vs. non-US tertiary HCM centres within the international Sarcomeric Human Cardiomyopathy Registry. Methods and results We included patients with HCM enrolled from eight US sites (n = 2650) and five non-US (n = 2660) sites and used multivariable Cox-proportional hazards models to compare outcomes between sites. Primary prevention ICD implantation rates in US sites were two-fold higher than non-US sites (hazard ratio (HR) 2.27 [1.89–2.74]), including in individuals deemed at high 5-year SCD risk (≥6%) based on the HCM risk-SCD score (HR 3.27 [1.76–6.05]). US ICD recipients also had fewer traditional SCD risk factors. Among ICD recipients, rates of appropriate ICD therapy were significantly lower in US vs. non-US sites (HR 0.52 [0.28–0.97]). No significant difference was identified in the incidence of SCD/resuscitated cardiac arrest among non-recipients of ICDs in US vs. non-US sites (HR 1.21 [0.74–1.97]). Conclusion  Primary prevention ICDs are implanted more frequently in patients with HCM in US vs. non-US sites across the spectrum of SCD risk. There was a lower rate of appropriate ICD therapy in US sites, consistent with a lower-risk population, and no significant difference in SCD in US vs. non-US patients who did not receive an ICD. Further studies are needed to understand what drives malignant arrhythmias, optimize ICD allocation, and examine the impact of different ICD utilization strategies on long-term outcomes in HCM.

Abstract 14575: Implantable Cardiac Defibrillator in the Setting of Tetralogy of Fallot: Long-Term Follow-Up of a French National Multicenter Retrospective Registry

Circulation ◽  
2015 ◽  
Vol 132 (suppl_3) ◽  
Author(s):  
Abdeslam Bouzeman ◽  
Maxime De Guillebon ◽  
Guillaume Duthoit ◽  
Magalie Ladouceur ◽  
Raphael Martins ◽  
...  
Keyword(s):  
Risk Factors ◽  
Ventricular Tachycardia ◽  
Primary Prevention ◽  
Secondary Prevention ◽  
Tetralogy Of Fallot ◽  
Primary And Secondary Prevention ◽  
Long Term Follow Up ◽  
Appropriate Therapy

Background: Tetralogy of Fallot (TOF) is the most frequent form of congenital heart disease managed by EP physicians for potential ICD. However, few studies have reported long-term outcomes of TOF patients with ICD. Methods: Between 2005 and 2014, all TOF patients with ICD in 17 French centers were enrolled in a specific evaluation aiming to determine characteristics at implantation as well as outcomes (overall mortality, appropriate ICD therapies, and device-related complications). Results: Overall 78 patients (45±13 years, 64% males) were enrolled. A majority of patients were implanted in the setting of secondary prevention (73%), whereas the remaining (27%) in primary prevention. Among the latest group, known risk factors for sudden cardiac death were: severe pulmonary regurgitation (30%,) prior palliative shunt (50%), syncope with unknown origin (25%), inducible ventricular tachycardia (45%), QRS duration ≥180ms (18%), non-sustained ventricular tachycardia (25%), and documented sustained supra ventricular tachycardia (45%).Overall, patients implanted in the setting of primary prevention presented with a mean of 3.1±1.4 risk factors. After a mean follow-up of 4.9±3.8 years, 35 patients (45%) experienced at least one appropriate therapy (25% in the primary prevention group compared to 53% in the secondary prevention group), giving annual-incidences of 6.9% (95%CI 0.14-13.7) and 21.3% (12.4-30.3) respectively (P=0,01). The mean time between ICD implantation and the first appropriate therapy was 2.2±3.2 years, without significant differences between primary and secondary prevention. Overall, ≥one ICD-related complication occurred in 30 patients (38%), including inappropriate shock (n=9), major pocket hematoma (n=1), lead dysfunction (n=12), infection (n=4), shoulder algodystrophia (n=2), device failure or dislodgement needing reintervention (n=2). Eventually, four patients were transplanted (5%), and six patients (8%) died during the course of follow-up. Conclusions: Considering relatively long-term follow-up, patients with TOF and ICDs experience high rates of appropriate ICD therapies, in both primary and secondary prevention. Major ICD-related complications remain, however, high.

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