scholarly journals J wave is associated with increased risk of sudden cardiac arrest in patients with hypertrophic cardiomyopathy

2013 ◽  
Vol 41 (4) ◽  
pp. 1281-1290 ◽  
Author(s):  
Yanbing Li ◽  
Jun Mao ◽  
Qian Yan ◽  
Shuyuan Qi ◽  
Xiaoyan Liu ◽  
...  
Keyword(s):  
Cardiac Arrest ◽  
Hypertrophic Cardiomyopathy ◽  
Sudden Cardiac Arrest ◽  
Increased Risk ◽  
J Wave

Clinical outcomes in adult athletes with hypertrophic cardiomyopathy: a 7-year follow-up study

2020 ◽  
Vol 54 (16) ◽  
pp. 1008-1012 ◽  
Author(s):  
Antonio Pelliccia ◽  
Stefano Caselli ◽  
Matteo Pelliccia ◽  
Maria Beatrice Musumeci ◽  
Erika Lemme ◽  
...  
Keyword(s):  
Cardiac Arrest ◽  
Hypertrophic Cardiomyopathy ◽  
Sport Participation ◽  
Sudden Cardiac Arrest ◽  
Left Ventricular ◽  
Rank Test ◽  
Competitive Sport ◽  
Increased Risk ◽  
Exercise Programmes

ObjectiveCurrent guidelines recommend precautionary disqualification from competitive sports in patients with hypertrophic cardiomyopathy (HCM). We assessed the incidence of cardiovascular events in a cohort of patients with HCM engaged in long-term exercise programmes and competitive sport.MethodsWe reviewed data on 88 consecutive athletes diagnosed with HCM, from 1997 to 2017; 92% male, 98% Caucasian, median age 31 (IQR: 19–44) years. All participated in regular exercise programmes and competitive sport at study entry.We performed follow-up evaluation after 7±5 (1–21) years. 61 (69%) of the athletes had substantially reduced or stopped exercise and sport (ie, HCM-detrained), and 27 had continued with regular training and sport competitions (HCM-trained). At baseline evaluation, both groups were similar for age, gender balance, symptoms, ECG abnormalities, extent of left ventricular hypertrophy, arrhythmias and risk profile for sudden cardiac death/arrest.ResultsDuring the follow-up period, two participants suffered sudden cardiac arrest or death (0.3% per year) both outside of sport participation. In addition, 19 (22%) reported symptoms (syncope in 3, palpitations in 10, chest pain in 4 and dyspnoea in 2). The Kaplan-Meier analyses of freedom from combined sudden cardiac arrest/death and symptoms (log-rank test p=0.264) showed no differences between HCM-trained and detrained patients.ConclusionIn this adult cohort of low-risk HCM athletes, voluntary decision to pursue in participation in competitive sport events was not associated with increased risk for major cardiac events or clinical worsening compared with decision to reduce or withdraw from exercise programmes and sport. Similar results may not be seen in younger or racially diverse athlete populations, or in patients with more severe HCM phenotypes.

scholarly journals Mechanisms of Arrhythmogenicity of Hypertrophic Cardiomyopathy-Associated Troponin T (TNNT2) Variant I79N

2021 ◽  
Vol 9 ◽  
Author(s):  
Sanam Shafaattalab ◽  
Alison Y Li ◽  
Marvin G Gunawan ◽  
BaRun Kim ◽  
Farah Jayousi ◽  
...  
Keyword(s):  
Cardiac Arrest ◽  
Hypertrophic Cardiomyopathy ◽  
Action Potential ◽  
Natriuretic Peptide ◽  
Troponin T ◽  
Sudden Cardiac Arrest ◽  
Human Action ◽  
Significant Shift ◽  
Ecm Remodeling ◽  
Increased Risk

Hypertrophic cardiomyopathy (HCM) is the most common heritable cardiovascular disease and often results in cardiac remodeling and an increased incidence of sudden cardiac arrest (SCA) and death, especially in youth and young adults. Among thousands of different variants found in HCM patients, variants of TNNT2 (cardiac troponin T—TNNT2) are linked to increased risk of ventricular arrhythmogenesis and sudden death despite causing little to no cardiac hypertrophy. Therefore, studying the effect of TNNT2 variants on cardiac propensity for arrhythmogenesis can pave the way for characterizing HCM in susceptible patients before sudden cardiac arrest occurs. In this study, a TNNT2 variant, I79N, was generated in human cardiac recombinant/reconstituted thin filaments (hcRTF) to investigate the effect of the mutation on myofilament Ca2+ sensitivity and Ca2+ dissociation rate using steady-state and stopped-flow fluorescence techniques. The results revealed that the I79N variant significantly increases myofilament Ca2+ sensitivity and decreases the Ca2+ off-rate constant (koff). To investigate further, a heterozygous I79N+/−TNNT2 variant was introduced into human-induced pluripotent stem cells using CRISPR/Cas9 and subsequently differentiated into ventricular cardiomyocytes (hiPSC-CMs). To study the arrhythmogenic properties, monolayers of I79N+/− hiPSC-CMs were studied in comparison to their isogenic controls. Arrhythmogenesis was investigated by measuring voltage (Vm) and cytosolic Ca2+ transients over a range of stimulation frequencies. An increasing stimulation frequency was applied to the cells, from 55 to 75 bpm. The results of this protocol showed that the TnT-I79N cells had reduced intracellular Ca2+ transients due to the enhanced cytosolic Ca2+ buffering. These changes in Ca2+ handling resulted in beat-to-beat instability and triangulation of the cardiac action potential, which are predictors of arrhythmia risk. While wild-type (WT) hiPSC-CMs were accurately entrained to frequencies of at least 150 bpm, the I79N hiPSC-CMs demonstrated clear patterns of alternans for both Vm and Ca2+ transients at frequencies >75 bpm. Lastly, a transcriptomic analysis was conducted on WT vs. I79N+/−TNNT2 hiPSC-CMs using a custom NanoString codeset. The results showed a significant upregulation of NPPA (atrial natriuretic peptide), NPPB (brain natriuretic peptide), Notch signaling pathway components, and other extracellular matrix (ECM) remodeling components in I79N+/− vs. the isogenic control. This significant shift demonstrates that this missense in the TNNT2 transcript likely causes a biophysical trigger, which initiates this significant alteration in the transcriptome. This TnT-I79N hiPSC-CM model not only reproduces key cellular features of HCM-linked mutations but also suggests that this variant causes uncharted pro-arrhythmic changes to the human action potential and gene expression.

scholarly journals Discovery of predictors of sudden cardiac arrest in diabetes: rationale and outline of the RESCUED (REcognition of Sudden Cardiac arrest vUlnErability in Diabetes) project

Open Heart ◽  
2021 ◽  
Vol 8 (1) ◽  
pp. e001554
Author(s):  
Laura H van Dongen ◽  
Peter P Harms ◽  
Mark Hoogendoorn ◽  
Dominic S Zimmerman ◽  
Elisabeth M Lodder ◽  
...  
Keyword(s):  
Cardiac Arrest ◽  
Prognostic Model ◽  
Early Recognition ◽  
Sudden Cardiac Arrest ◽  
Clinical Genetic ◽  
Innovative Strategy ◽  
Rare Variant Analysis ◽  
Increased Risk ◽  
Gp Care

IntroductionEarly recognition of individuals with increased risk of sudden cardiac arrest (SCA) remains challenging. SCA research so far has used data from cardiologist care, but missed most SCA victims, since they were only in general practitioner (GP) care prior to SCA. Studying individuals with type 2 diabetes (T2D) in GP care may help solve this problem, as they have increased risk for SCA, and rich clinical datasets, since they regularly visit their GP for check-up measurements. This information can be further enriched with extensive genetic and metabolic information.AimTo describe the study protocol of the REcognition of Sudden Cardiac arrest vUlnErability in Diabetes (RESCUED) project, which aims at identifying clinical, genetic and metabolic factors contributing to SCA risk in individuals with T2D, and to develop a prognostic model for the risk of SCA.MethodsThe RESCUED project combines data from dedicated SCA and T2D cohorts, and GP data, from the same region in the Netherlands. Clinical data, genetic data (common and rare variant analysis) and metabolic data (metabolomics) will be analysed (using classical analysis techniques and machine learning methods) and combined into a prognostic model for risk of SCA.ConclusionThe RESCUED project is designed to increase our ability at early recognition of elevated SCA risk through an innovative strategy of focusing on GP data and a multidimensional methodology including clinical, genetic and metabolic analyses.

Sudden cardiac arrest in 15-year-old girl. Could it be avoided?

2018 ◽  
Vol 1 (46) ◽  
pp. 43-48
Author(s):  
Maria Posadowska ◽  
Maria Miszczak-Knecht ◽  
Alicja Mirecka-Rola ◽  
Katarzyna Bieganowska
Keyword(s):  
Cardiac Arrest ◽  
Hypertrophic Cardiomyopathy ◽  
Cardiac Death ◽  
Sudden Cardiac Arrest ◽  
Postmortem Examination ◽  
Cardiac Care ◽  
Related Family ◽  
Asymptomatic Patients ◽  
History Of ◽  
Genetically Determined

We present a case of a 15-year-old girl after sudden cardiac arrest because of ventricular fibrillation. Hypertrophic cardiomyopathy was diagnosed. Family history of patient was positive – her uncle (mother’s brother) died suddenly at the age of 21, postmortem examination showed hypertrophic cardiomyopathy. The deceased man’s family was not under cardiac care. The presented case proves, that due to genetically determined cardiac diseases such as cardiomyopathies and channelopathies, all related family members should be examined cardiologically. Diagnosis of the disease in asymptomatic patients would allow the implementation of treatment and reduce the risk of a sudden cardiac arrest / sudden cardiac death.

scholarly journals Increased Risk of Sudden Cardiac Arrest in Obstructive Pulmonary Disease: A Case-Control Study

PLoS ONE ◽  
2013 ◽  
Vol 8 (6) ◽  
pp. e65638 ◽  
Author(s):  
Miriam Jacoba Warnier ◽  
Marieke Tabo Blom ◽  
Abdennasser Bardai ◽  
Jocelyn Berdowksi ◽  
Patrick Cyriel Souverein ◽  
...  
Keyword(s):  
Cardiac Arrest ◽  
Pulmonary Disease ◽  
Case Control Study ◽  
Sudden Cardiac Arrest ◽  
Case Control ◽  
Obstructive Pulmonary Disease ◽  
Increased Risk ◽  
Control Study

Sudden Cardiac Arrest in Hypertrophic Cardiomyopathy in the Absence of Conventional Criteria for High Risk Status

2008 ◽  
Vol 101 (4) ◽  
pp. 544-547 ◽  
Author(s):  
Barry J. Maron ◽  
Martin S. Maron ◽  
John R. Lesser ◽  
Robert G. Hauser ◽  
Tammy S. Haas ◽  
...  
Keyword(s):  
Cardiac Arrest ◽  
Hypertrophic Cardiomyopathy ◽  
High Risk ◽  
Sudden Cardiac Arrest ◽  
Risk Status

Abstract 16917: The Forgotten Prolapsed Mitral Valve in a Patient With Sudden Cardiac Arrest

Circulation ◽  
2020 ◽  
Vol 142 (Suppl_3) ◽  
Author(s):  
Mark W Vanderland ◽  
YinnShaung Ooi ◽  
Muhammad Shakir ◽  
Douglas Esberg
Keyword(s):  
Cardiac Arrest ◽  
High Risk ◽  
Mitral Valve ◽  
Coronary Angiography ◽  
Cardiac Mri ◽  
Sinus Bradycardia ◽  
Sudden Cardiac Arrest ◽  
Mri Findings ◽  
True Incidence ◽  
Increased Risk

Current literature suggests that the incidence of sudden cardiac arrest (SCA) attributable to mitral valve prolapse (MVP) is more common than historically presumed. Although its true incidence remains unknown, this novel realization suggests that the presence of MVP after resuscitated SCA should prompt exploratory identification of a causative association between MVP and SCA, even in the presence of other plausible explanatory findings. Case Description: A 60-year-old male with a history of hypertension presented with an out-of-hospital cardiac arrest due to ventricular fibrillation (VF). Coronary angiography revealed a 99% occluded 1st diagonal branch which was stented. TTE showed normal biventricular function and coincidentally found a myxomatous mitral valve with bileaflet prolapse. The patient was discharged without further work up under the rationale that the ischemic etiology of his arrest was reversed. 6 months later, he presented with another episode of VF arrest. EKG showed sinus bradycardia and a QTc of 534ms. Troponin was negative. Coronary angiography revealed a patent stent and coronary vessels. A cardiac MRI was obtained to investigate the arrhythmic significance of his MVP and showed late gadolinium enhancement in the basal inferolateral wall and tips of the mitral papillary muscles. A dual chamber implantable cardiac defibrillator was placed with a plan for future mitral valve repair. Discussion: Recent studies suggest that the estimated annual risk of SCA due to MVP is 0.2% to 1.9%, a figure higher than previously reported. Certain EKG, TTE and cardiac MRI findings have been associated with an increased risk of ventricular arrhythmias and SCA. In this patient, this included bileaflet MVP, mitral antral disjunction, and inferobasal wall and papillary muscle fibrosis. Currently, there are no society guidelines to assist in the identification of patients with MVP at high-risk for SCA. Nonetheless, in patients with MVP resuscitated from SCA, further workup to identify high risk features is reasonably justified to guide secondary SCA prophylaxis, even in the presence of other explanatory findings for SCA.

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