A RARE CASE OF AN ABERRANT CORONARY ARTERY CAUSING INCREASED RISK OF SUDDEN CARDIAC ARREST IN APICAL HYPERTROPHIC CARDIOMYOPATHY

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.

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Sudden cardiac arrest in apical hypertrophic cardiomyopathy

BMJ Case Reports ◽

10.1136/bcr.04.2009.1753 ◽

2009 ◽

Vol 2009 (sep06 1) ◽

pp. bcr0420091753-bcr0420091753

Author(s):

I. Ahmed ◽

S. J Smalley ◽

D. W X Zhu ◽

R. Dahiya ◽

C. M House ◽

...

Keyword(s):

Cardiac Arrest ◽

Hypertrophic Cardiomyopathy ◽

Sudden Cardiac Arrest ◽

Apical Hypertrophic Cardiomyopathy

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Natural internal thoracic-to-coronary artery collateral network

Asian Cardiovascular and Thoracic Annals ◽

10.1177/0218492319891017 ◽

2019 ◽

Vol 28 (2) ◽

pp. 118-119

Author(s):

Tomoki Nagata ◽

Takao Hasegawa ◽

Hiroyuki Johno

Keyword(s):

Cardiac Arrest ◽

Coronary Artery ◽

Rare Case ◽

Internal Mammary Artery ◽

Left Internal Mammary Artery ◽

Artery Bypass ◽

Sudden Cardiac Arrest ◽

Bypass Grafting ◽

Internal Mammary ◽

Collateral Network

We describe a rare case of three-vessel coronary disease in a 67-year-old man who had spontaneous communication between the left internal mammary artery and the left anterior descending artery. We decided to perform elective coronary artery bypass grafting. However, he died from sudden cardiac arrest before surgery.

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Sudden Cardiac Arrest in a Patient With Apical Hypertrophic Cardiomyopathy

American Journal of Therapeutics ◽

10.1097/mjt.0000000000000107 ◽

2016 ◽

Vol 23 (1) ◽

pp. e276-e282 ◽

Cited By ~ 4

Author(s):

Tanush Gupta ◽

Neha Paul ◽

Chandrasekar Palaniswamy ◽

Nivas Balasubramaniyam ◽

Wilbert S. Aronow ◽

...

Keyword(s):

Cardiac Arrest ◽

Hypertrophic Cardiomyopathy ◽

Sudden Cardiac Arrest ◽

Apical Hypertrophic Cardiomyopathy

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Ventricular fibrillation and sudden cardiac arrest in apical hypertrophic cardiomyopathy: Two case reports

World Journal of Clinical Cases ◽

10.12998/wjcc.v9.i35.11102 ◽

2021 ◽

Vol 9 (35) ◽

pp. 11102-11107

Author(s):

Yae Min Park ◽

Albert Youngwoo Jang ◽

Wook-Jin Chung ◽

Seung Hwan Han ◽

Christopher Semsarian ◽

...

Keyword(s):

Cardiac Arrest ◽

Hypertrophic Cardiomyopathy ◽

Ventricular Fibrillation ◽

Case Reports ◽

Sudden Cardiac Arrest ◽

Apical Hypertrophic Cardiomyopathy

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

Frontiers in Cell and Developmental Biology ◽

10.3389/fcell.2021.787581 ◽

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.

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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 ◽

10.1136/openhrt-2020-001554 ◽

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.

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Paediatric-onset coronary artery anomalies in pregnancy: a single-centre experience and systematic literature review

Cardiology in the Young ◽

10.1017/s1047951117000658 ◽

2017 ◽

Vol 27 (8) ◽

pp. 1529-1537 ◽

Cited By ~ 3

Author(s):

Michelle Keir ◽

Catriona Bhagra ◽

Debra Vatenmakher ◽

Francisca Arancibia-Galilea ◽

Katrijn Jansen ◽

...

Keyword(s):

Myocardial Infarction ◽

Cardiac Arrest ◽

Coronary Artery ◽

Cardiovascular Events ◽

Coronary Anomalies ◽

Coronary Artery Anomalies ◽

Major Cardiovascular Events ◽

Coronary Aneurysms ◽

Increased Risk ◽

In Pregnancy

AbstractObjectivesIndividuals with childhood-onset coronary artery anomalies are at increased risk of lifelong complications. Although pregnancy is thought to confer additional risk, a few data are available regarding outcomes in this group of women. We sought to define outcomes of pregnancy in this unique population.MethodsWe performed a retrospective survey of women with paediatric-onset coronary anomalies and pregnancy in our institution, combined with a systematic review of published cases. We defined paediatric-onset coronary artery anomalies as congenital coronary anomalies and inflammatory arteriopathies of childhood that cause coronary aneurysms. Major cardiovascular events were defined as pulmonary oedema, sustained arrhythmia requiring treatment, stroke, myocardial infarction, cardiac arrest, or death.ResultsA total of 25 surveys were mailed, and 20 were returned (80% response rate). We included 46 articles from the literature, which described cardiovascular outcomes in 82 women (138 pregnancies). These data were amalgamated for a total of 102 women and 194 pregnancies; 59% of women were known to have paediatric-onset coronary artery anomalies before pregnancy. In 23%, the anomaly was unmasked during or shortly after pregnancy. The remainder, 18%, was diagnosed later in life. Major cardiovascular events occurred in 14 women (14%) and included heart failure (n=5, 5%), myocardial infarction (n=7, 7%), maternal death (n=2, 2%), cardiac arrest secondary to ventricular fibrillation (n=1, 1%), and stroke (n=1, 1%). The majority of maternal events (13/14, 93%) occurred in women with no previous diagnosis of coronary disease.ConclusionsWomen with paediatric-onset coronary artery anomalies have a 14% risk of adverse cardiovascular events in pregnancy, indicating the need for careful assessment and close follow-up. Prospective, multicentre studies are required to better define risk and predictors of complications during pregnancy.

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