Hypertrophic cardiomyopathy in children

2021 ◽  
pp. 021849232110412
Author(s):  
Shuiyun Wang ◽  
Changsheng Zhu
Keyword(s):  
Surgical Treatment ◽  
Hypertrophic Cardiomyopathy ◽  
Cardiac Death ◽  
Treatment Results ◽  
Tertiary Referral ◽  
Good Discrimination ◽  
Icd Implantation ◽  
Tertiary Referral Center ◽  
Cardioverter Defibrillator ◽  
Septal Hypertrophy

Hypertrophic cardiomyopathy (HCM) characterized by asymmetric ventricular septal hypertrophy, is the commonest cause of sudden cardiac death (SCD) in the young. The underlying etiology of HCM in the childhood and adolescent patients is diverse. Moreover, the prognosis of pediatric HCM depends on the age of presentation and etiology. Despite the complexity of children with obstructive HCM, surgical treatment results in a favorable outcome for carefully selected patients in experienced tertiary referral center in contemporary era. Implantable cardioverter-defibrillator (ICD) remains the most effective and reliable treatment to prevent SCD. New pediatric SCD risk prediction model, which has good discrimination and calibration and can distinguish patients who are most benefit from an ICD implantation, is expected to be further refined in the future.

scholarly journals Subcutaneous Implantable Cardioverter Defibrillator Explantation -- A Single Tertiary Center Experience

2021 ◽  
Author(s):  
Naga Venkata Pothineni ◽  
Tharian Cherian ◽  
Neel Patel ◽  
Jeffrey Smietana ◽  
David Frankel ◽  
...  
Keyword(s):  
Implantable Cardioverter Defibrillator ◽  
Tertiary Referral ◽  
Icd Implantation ◽  
Tertiary Referral Center ◽  
Cardioverter Defibrillator ◽  
Device Implantation ◽  
Tertiary Center ◽  
Qrs Duration ◽  
The Mean ◽  
Selection For

Background: The subcutaneous implantable-cardioverter defibrillator (S-ICD) is an appealing alternative to transvenous ICD systems. However, data on indications for S-ICD explantations are sparse. Objectives: To assess incidence and indications for S-ICD explantation at a large tertiary referral center. Methods: We conducted a retrospective study of all S-ICD explantations performed from 2014 to 2020. Data on demographics, comorbidities, implantation characteristics, and indications for explantation, were collected. Results: A total of 64 patients underwent S-ICD explantation during the study period. During that time, there were 410 S-ICD implantations at our institution of which 53 (12.9%) were explanted with a mean duration from implant to explant of 19.7±20.1 months. The mean age of the patients at explantation was 44.8±15.3 years, and 42% (n=27) were female. The indication for S-ICD implantation was primary prevention in 58% and secondary prevention in 42% of the patients. The most common reason for explantation was infection (32.8%) followed by abnormal sensing (25%) and need for pacing (18.8%). Those who underwent S-ICD explantation for pacing indications were significantly older (55.7±13.6 vs 42.3± 14.6 years, p = 0.005) with a wider QRS duration (111±19 ms vs 98±19 ms, p = 0.03) at device implantation compared to patients who underwent explantation for other indications. Conclusion: Incidence of S-ICD explantation in a large tertiary practice was 12.9%. While infection was the indication for a third of the explantations, a significant number were due to sensing abnormalities and need for pacing. These data may have implications for patient selection for S-ICD implantation.

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.

scholarly journals LEFT VENTRICULAR LEAD IMPLANTATION FOR DETECTION OF VENTRICULAR ARRHYTHMIAS IN PATIENT WITH IMPLANTABLE CARDIOVERTER DEFIBRILLATOR AND LOW R WAVE IN RIGHT VENTRICLE

2021 ◽  
pp. 17
Author(s):  
Keyword(s):  
Sudden Cardiac Death ◽  
Right Ventricle ◽  
Implantable Cardioverter Defibrillator ◽  
Cardiac Death ◽  
Ventricular Arrhythmias ◽  
Systolic Function ◽  
Left Ventricular ◽  
Icd Implantation ◽  
Cardioverter Defibrillator ◽  
Ventricular Lead

Dilated cardiomyopathy (DCM) is a disease characterised as left ventricular (LV) or biventricular dilatation with impaired systolic function. Regardless of underlying cause patients with DCM have a propensity to ventricular arrhythmias and sudden cardiac death. Implantable Cardioverter Defibrillator (ICD) implantation for these patients results in significant reduction of sudden cardiac death [1-3]. ICD devices may be limited by right ventricle (RV) sensing dysfunction with low RV sensing amplitude. We present a clinical case of patient with DCM, implanted ICD and low R wave sensing on RV lead.

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.

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.

scholarly journals CHARACTERISATION OF HYPERTROPHIC CARDIOMYOPATHY BY CARDIAC MAGNETIC RESONANCE IMAGING

2021 ◽  
Vol 9 (11) ◽  
pp. 643-648
Author(s):  
Nava Udaya N.R ◽  
◽  
Ramiah Rajesh Kannan ◽  
Srikanth Moorthy ◽  
Resmi Sekhar ◽  
...  
Keyword(s):  
Hypertrophic Cardiomyopathy ◽  
Sudden Cardiac Death ◽  
Left Ventricle ◽  
Cardiac Mri ◽  
Cardiac Death ◽  
Final Diagnosis ◽  
Left Ventricular ◽  
Categorical Variables ◽  
Diagnostic Parameters ◽  
Septal Hypertrophy

Background: There are many causes of left ventricular hypertrophy which can result in arrhythmias and sudden cardiac death. Hypertrophic cardiomyopathy (HCM) is one of the commonly encountered cause of sudden cardiac death in young adults. Aim: Identifying the role of Cardiac MRI in characterising the diagnostic parameters of HCM. Materials and methods: 125 patients with clinical suspicion or genetic evidence of HCM referred for cardiac MRI between June 2013 to June 2021 were included under the study. Image interpretations were done by fellowship qualified cardiac imaging radiologist. Categorical variables were expressed using frequency and percentage. Numerical variables were presented using mean and standard deviation. Results: Out of the total population, 119 patients (95 %) had LV wall thickness > 13 mm, 48 patients (38.4%) had Left ventricle outflow tract obstruction (LVOTO) and 32 patients (25.6 %) had mid cavity obstruction, 39 patients (37.9 %) had myocardial scar > 15 % and asymmetric septal hypertrophy was the most frequently encountered left ventricle morphology Conclusion: Cardiac MRI detected HCM has a statistically significant association with the final diagnosis (histopathological and genetic correlation). CMRI hence serves as a reliable tool in identifying and characterising the various diagnostic and non- diagnostic parameters of HCM.

Hypertrophic cardiomyopathy: prevention of sudden cardiac death

ESC CardioMed ◽  
2018 ◽  
pp. 1462-1466
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.

Abstract 10947: Longer and Faster Non-sustained Ventricular Tachycardia in Hypertrophic Cardiomyopathy Patients Predicts for Implantable Cardioverter Defibrillator Shocks and Sudden Cardiac Death

Circulation ◽  
2015 ◽  
Vol 132 (suppl_3) ◽  
Author(s):  
Weijia Wang ◽  
Zhesi Lian ◽  
Ethan Rowin ◽  
Martin Maron ◽  
Mark Link
Keyword(s):  
Ventricular Tachycardia ◽  
Hypertrophic Cardiomyopathy ◽  
Primary Prevention ◽  
Sudden Cardiac Death ◽  
Implantable Cardioverter Defibrillator ◽  
Cardiac Death ◽  
Medical Center ◽  
Multivariable Analysis ◽  
Sustained Ventricular Tachycardia ◽  
Cardioverter Defibrillator

Introduction: Non-sustained ventricular tachycardia (NSVT) may be underestimated in patients with hypertrophic cardiomyopathy (HCM). Its impact on the risk of sudden cardiac death (SCD) in HCM is controversial. There is no distinction made in the guidelines as to the length or rate of NSVT as a risk marker for SCD. Hypothesis: NSVT may be nearly universal in HCM patients with high risk of SCD and not found because of the limited time frame of monitoring. NSVT may be associated with appropriate Implantable Cardioverter Defibrillator (ICD) shocks and SCD. Methods: A retrospective study of 181 HCM patients who had an ICD and were followed for at least 6 months from 2000 to 2013 at Tufts Medical Center was performed. The pre-operative evaluations as well as routine ICD follow up notes were reviewed. Results: ICD was implanted in 175 (96.7%) patients as primary prevention and in 6 (3.3%) patients as secondary prevention for SCD. Ninety six (53.0%) patients total had NSVT, including 48 (26.5%) before and 77 (42.5%) after ICD implantation. The agreement for detecting NSVT between Holter monitoring and ICD interrogation was poor (Kappa=0.18, p=0.054). Eighteen (18.75%) patients with NSVT and 6 (7.06%) patients without NSVT had appropriate ICD shocks or SCD (Figure 1). In multivariable analysis, NSVT was independently associated with appropriate ICD shocks and SCD (OR 3.69, 95%CI: 1.31 - 10.43) and remained significant in the 175 patients who had ICD implanted as primary prevention (OR 3.86, 95%CI: 1.13 - 13.18). More rapid NSVT (Cl < 310ms) predicted appropriate ICD shocks and SCD (OR 7.7, 95%CI: 1.6, 36.8), and longer NSVT (> 18beats) also predicted appropriate ICD shocks and SCD (OR=23.7, 95%CI: 2.7, 204.9). Conclusion: The agreement for detecting NSVT between Holter and ICD interrogation is poor. NSVT is significantly associated with appropriate ICD shocks and SCD. Faster and longer NSVT are even more predictive. Extending rhythm monitor time merits consideration in HCM patients.

Risk stratification for sudden cardiac death in hypertrophic cardiomyopathy

ESC CardioMed ◽  
2018 ◽  
pp. 2316-2319
Author(s):  
Philipp Attanasio ◽  
Wilhelm Haverkamp
Keyword(s):  
Hypertrophic Cardiomyopathy ◽  
High Risk ◽  
Sudden Cardiac Death ◽  
Risk Stratification ◽  
Prediction Model ◽  
Cardiac Death ◽  
Cardioverter Defibrillator ◽  
Life Threatening ◽  
Esc Guidelines ◽  
European Society Of Cardiology

Identification of patients with hypertrophic cardiomyopathy (HCM) who are at high risk of sudden cardiac death (SCD) is essential, as life-threatening arrhythmic events can be effectively treated with implantable cardioverter defibrillator therapy. Various models for risk stratification of patients with HCM have been proposed. The latest clinical risk prediction model was developed in 2013. It is based on the HCM Risk-SCD study that included 3675 patients. Risk stratification using this model is recommended in the 2014 European Society of Cardiology (ESC) Guidelines for management of HCM and in the 2015 ESC Guidelines for the management of patients with ventricular arrhythmias and the prevention of SCD. This chapter summarizes novelties in the prediction model and the resulting recommendations, and discusses potential limitations of this approach.

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.

Sign in / Sign up

Export Citation Format

Share Document