scholarly journals Incremental Value of an Insertable Cardiac Monitor in Patients with Hypertrophic Cardiomyopathy with Low or Intermediate Risk for Sudden Cardiac Death

Cardiology ◽  
2021 ◽  
pp. 1-6
Author(s):  
Rafi Sakhi ◽  
Roy Huurman ◽  
Dominic A.M.J. Theuns ◽  
Arend F.L. Schinkel ◽  
Amira Assaf ◽  
...  
Keyword(s):  
Hypertrophic Cardiomyopathy ◽  
Sudden Cardiac Death ◽  
Cardiac Death ◽  
De Novo ◽  
Diagnostic Yield ◽  
Control Group ◽  
Intermediate Risk ◽  
Implantable Cardioverter Defibrillators ◽  
Cardiac Monitor ◽  
Insertable Cardiac Monitor

<b><i>Aims:</i></b> The aim of the present study was to compare the rate of actionable arrhythmic events between patients with hypertrophic cardiomyopathy (HCM) who are monitored with an insertable cardiac monitor (ICM) or Holter monitoring. <b><i>Methods:</i></b> We studied 50 patients (mean age 52 years, 72% men) with HCM at low or intermediate risk for sudden cardiac death (SCD), of whom 25 patients received an ICM between November 2014 and February 2019. We retrospectively identified a control group of 25 patients who were matched on age, sex, and HCM Risk-SCD score category. The mean HCM Risk-SCD score was 3.41 ± 1.31 and 3.31 ± 1.43 for the ICM and Holter groups, respectively. The primary endpoint was an actionable event which was defined as an arrhythmic event resulting in a change in patient management. The secondary endpoint was the occurrence of ventricular tachycardia (VT). <b><i>Results:</i></b> The cumulative actionable event rate at 30 months was higher in the ICM group (51 vs. 27%, log-rank <i>p</i> value &#x3c;0.01). De novo atrial fibrillation requiring oral anticoagulation occurred only in the ICM group (<i>n</i> = 3). Overall, 4 implantable cardioverter-defibrillators were implanted for primary prevention (<i>n</i> = 2 in each group). The cumulative rate of VT episodes at 30 months was similar between groups (23% [ICM group] vs. 42% [Holter group], log-rank <i>p</i> value = 0.71). Furthermore, the characteristics of VT were similar between groups with regard to the number of beats and rate. <b><i>Conclusions:</i></b> In adults with HCM, an ICM will detect more arrhythmic events requiring an intervention than a conventional Holter strategy. In contrast, the diagnostic yield of detecting VT seems similar for both groups.

scholarly journals Implantable Cardioverter-Defibrillators and Prevention of Sudden Cardiac Death in Hypertrophic Cardiomyopathy

JAMA ◽  
2007 ◽  
Vol 298 (4) ◽  
Author(s):  
Barry J. Maron ◽  
Paolo Spirito ◽  
Win-Kuang Shen ◽  
Tammy S. Haas ◽  
Francesco Formisano ◽  
...  
Keyword(s):  
Hypertrophic Cardiomyopathy ◽  
Sudden Cardiac Death ◽  
Cardiac Death ◽  
Implantable Cardioverter Defibrillators ◽  
Cardioverter Defibrillators

scholarly journals Sudden Cardiac Death Caused by a Fatal Association of Hypertrophic Cardiomyopathy (MYH7, p.Arg719Trp), Heterozygous Familial Hypercholesterolemia (LDLR, p.Gly343Lys) and SARS-CoV-2 B.1.1.7 Infection

Diagnostics ◽  
2021 ◽  
Vol 11 (7) ◽  
pp. 1229
Author(s):  
Nicola Marziliano ◽  
Alessandro Medoro ◽  
Donatella Mignogna ◽  
Giovanni Saccon ◽  
Stefano Folzani ◽  
...  
Keyword(s):  
Hypertrophic Cardiomyopathy ◽  
Sudden Cardiac Death ◽  
Familial Hypercholesterolemia ◽  
Cardiac Death ◽  
De Novo ◽  
Plasma Concentrations ◽  
Young Age ◽  
Cardiac Complications ◽  
Heterozygous Familial Hypercholesterolemia ◽  
Fatal Arrhythmia

Hypertrophic cardiomyopathy (HCM) and heterozygous familial hypercholesterolemia (HeFH), two of the most common genetic cardiovascular disorders, can lead to sudden cardiac death. These conditions could be complicated by concomitant severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection as in the case herein described. A young amateur soccer player died in late October 2020 after a fatal arrhythmia and the autopsy revealed the presence of HCM with diffuse non-obstructive coronary disease. The molecular autopsy revealed a compound condition with a first mutation in the MYH7 gene (p.Arg719Trp) and a second mutation in the LDLR gene (p.Gly343Cys): both have already been described as associated with HCM and HeFH, respectively. In addition, molecular analyses showed the presence of SARS-CoV-2 lineage B.1.1.7 (UK variant with high titer in the myocardium. Co-segregation analysis within the family (n = 19) showed that heterozygous LDLR mutation was maternally inherited, while the heterozygous MYH7 genetic lesion was de novo. All family member carriers of the LDLR mutation (n = 13) had systematic higher LDL plasma concentrations and positive records of cardiac and vascular ischemic events at young age. Considering that HCM mutations are in themselves involved in the predisposition to malignant arrhythmogenicity and HeFH could cause higher risk of cardiac complications in SARS-CoV-2 infection, this case could represent an example of a potential SARS-CoV-2 infection role in triggering or unmasking inherited cardiovascular disease, whose combination might represent the cause of fatal arrhythmia at such a young age. Additionally, it can provide clues in dating the presence of the SARS-CoV-2 lineage B.1.1.7 in Northern Italy in the early phases of the second pandemic wave.

Validation of the hypertrophic cardiomyopathy risk-sudden cardiac death calculator in Asians

Heart ◽  
2019 ◽  
Vol 105 (24) ◽  
pp. 1892-1897 ◽  
Author(s):  
You-Jung Choi ◽  
Hyung-Kwan Kim ◽  
Sang Chol Lee ◽  
Jun-Bean Park ◽  
Inki Moon ◽  
...  
Keyword(s):  
Hypertrophic Cardiomyopathy ◽  
Sudden Cardiac Death ◽  
Primary Endpoint ◽  
Predictive Value ◽  
Cardiac Death ◽  
Risk Group ◽  
Risk Groups ◽  
Low Risk ◽  
Intermediate Risk ◽  
Icd Therapy

ObjectiveThe hypertrophic cardiomyopathy (HCM) risk-sudden cardiac death (SCD) calculator endorsed by the 2014 European Society of Cardiology has not been independently validated in the Asians. We aimed to investigate whether the HCM Risk-SCD calculator effectively predicts SCD in Korean HCM population.MethodsAn observational, longitudinal cohort study was performed in 730 patients with HCM from 2007 to 2017. The primary endpoint was a composite of SCD and appropriate implantable cardioverter-defibrillator (ICD) therapy.ResultsDuring a follow-up period of 4288 person-years, 16 (2.2%) patients reached the primary endpoint. This validation study revealed a calibration slope of 0.892 and C-statistics of 0.718. The primary endpoint occurred in 1.1% (7/615), 4.6% (3/65) and 12.0% (6/50) of low-risk, intermediate-risk and high-risk groups, respectively. Although most patients (85.2%) without the primary endpoint were classified into the low-risk group, 7 of 11 SCD (63.6%) occurred in the low-risk group. In univariable and multivariable analysis, sex (woman) was significantly associated with the primary endpoint and emerged as independent predictor. The addition of sex to the HCM Risk-SCD calculator significantly improved the predictive value of the primary endpoint (net reclassification improvement 0.557, p=0.015).ConclusionsIn the Korean HCM population, the HCM Risk-SCD calculator had a high negative predictive value and accuracy for predicting SCD or appropriate ICD therapy, but misclassified a few patients experiencing the primary endpoint as low-risk or intermediate-risk groups.

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