scholarly journals Laminopathies: Should Wenckebach be a cause for concern? A case report

Author(s):  
Gautam Sen ◽  
Tom Jackson

Abstract Background LMNA cardiomyopathy is a cause of dilated cardiomyopathy (DCM) characterised by aggressive heart failure, high risk of arrhythmias and sudden cardiac death. We present a case of a male presenting with a LMNA mutation with an aggressive DCM leading to sudden cardiac death (SCD). Case summary A 42-year-old male presented with the feeling of lethargy and intermittent dizziness. ECG demonstrated AV block in keeping with Mobitz type 1, at a rate of 40 b.p.m. and cardiac monitoring showed non-sustained VT. CMR imaging showed preserved left ventricular function (EF 59%) but features suggesting DCM. These included mild LV dilatation with an EDV of 213 mL and late enhancement showing a single mid myocardial focus of high signal over the distal RV insertion point inferiorly and a linear area of high signal over the basal septum. After discussion at cardiology multi-disciplinary meeting a pacemaker was implanted so that beta-blockers could be initiated to suppress the ventricular arrhythmias. A laminopathy was suspected and if this was confirmed from genetic testing the plan was to upgrade to an implantable defibrillator. Due to stability this was decided to be done in an outpatient setting. He unfortunately had an out of hospital VF arrest and died. Post-mortem showed subtle cardiomyopathy in keeping with a DCM. Genetic tests results were returned a few months later which confirmed a pathogenic variant in LMNA. Discussion Because of the complexity of LMNA-related cardiac disease, they should be managed and followed up in centres with special expertise in inherited cardiomyopathy.

Circulation ◽  
2015 ◽  
Vol 131 (suppl_1) ◽  
Author(s):  
Muammar M Kabir ◽  
Elyar Ghafoori ◽  
Jonathan W Waks ◽  
Sunil K Agarwal ◽  
Dan E Arking ◽  
...  

Background: Respiration causes heart movement in the chest and proportional change in the heart’s electrical axis. The ECG can be used to measure respiration-related heart motion. The effect of respiration on the ECG is usually considered an artifact. However, it is unknown whether pattern of heart motion due to respiration holds any prognostic value. Method: After excluding those with atrial fibrillation, or atrial or ventricular premature contractions at baseline visit, 14613 ARIC cohort participants (mean age 54.0±5.8 y; 6595 [45.1%] men; 10744 [73.5%] white, 1311 [9.0%] with prevalent cardiovascular disease (CVD)) were included. The digital resting ECG was analyzed using custom Matlab software. The absolute magnitude of the displacement of the heart due to respiration was calculated on X (left-right), Y (up-down), and Z (anterior-posterior) axes. Sudden cardiac death (SCD) and non-coronary heart disease (CHD) death served as competing outcomes in our analysis. Results: In CVD-free participants (as compared to prevalent CVD group) heart moved more on X axis (137±46 vs. 128±47 μV; P<0.0001), and less on Z axis (123±52 vs. 127±60 μV; P=0.05). During a median follow-up of 14 years 278 SCDs (96 in CVD group) and 1619 non-CHD (279 in CVD group) deaths occurred. In competing risk analysis that adjusted for age, gender, race, history of myocardial infarction, CHD, heart failure, systolic blood pressure, anti-hypertensive medications, diabetes, smoking, total cholesterol, high density lipoprotein, level of physical activity, use of beta-blockers, left ventricular hypertrophy on ECG and QRS duration, the absolute magnitude of respiration-related heart movement on X axis (SHR 0.74; 95%CI 0.59-0.93; P=0.009) and Z axis (SHR 1.19; 95%CI 1.01-1.41; P=0.042) associated with SCD (but not with non-CHD death) in CVD group, but not in CVD-free participants. Conclusion: Greater respiration-caused heart motion on Z axis and smaller - on X axis likely reflects cardiomegaly and is associated with increased risk of SCD in patients with CVD.


2020 ◽  
Vol 6 (5) ◽  
Author(s):  
Nabil El-Sherif ◽  
Mohamed Boutjdir ◽  
Gioia Turitto

Sudden cardiac death accounts for approximately 360,000 annually in the United States and is the cause of half of all cardiovascular deaths. Ischemic heart disease is the major cause of death in the general adult population. Sudden cardiac death can be due to arrhythmic or non-arrhythmic cardiac causes, for example, myocardial rupture. Arrhythmic sudden cardiac death may be caused by ventricular tachyarrhythmia (ventricular tachycardia/ventricular fibrillation) or pulseless electrical activity/asystole. The majority of research in risk stratification centers on ventricular tachyarrhythmias simply because of the availability of a successful management strategy, the implantable cardioverter/ defibrillator. Currently the main criterion of primary defibrillator prophylaxis is the presence of organic heart disease and depressed left ventricular systolic function assessed as left ventricular ejection fraction. However, only one third of eligible patients benefit from the implantable defibrillator, resulting in significant redundancy in the use of the device. The cost to the health care system of sustaining this approach is substantial. Further, the current low implantation rate among eligible population probably reflects a perceived low benefit-to-cost ratio of the device. Therefore, attempts to optimize the selection process for primary implantable defibrillator prophylaxis are paramount. The present report will review the most recent pathophysiology and risk stratification strategies for sudden cardiac death beyond the single criterion of depressed ejection fraction. Emphasis will be placed on electrophysiological surrogates of conduction disorder, dispersion of repolarization, and autonomic imbalance, which represent our current understanding of the electrophysiological mechanisms that underlie the initiation of ventricular tachyarrhythmias. Further, factors that modify arrhythmic death, including noninvasive risk variables, biomarkers, and genomics will be addressed. These factors may have great utility in predicting sudden cardiac arrhythmic death in the general public.


Author(s):  
Hans-Richard Arntz

Even if sudden cardiac death is considered to be the most frequent cause of death in adults in industrialized countries, its incidence varies widely, depending on the definition and the source and quality of underlying data. It is estimated that about 70-80% of cases are due to coronary heart disease. The remaining 20% are attributable to a wide variety of inborn, genetically determined or acquired diseases, including a small group with hitherto undefined background. Prevention primarily encompasses the treatment of cardiovascular risk factors to avoid manifestations of coronary heart disease. Furthermore, preventive strategies are targeted to define groups of patients with an increased risk for sudden cardiac death or individuals at risk in specific populations, e.g. competitive athletes. A major target group are patients with impaired left ventricular function, preferentially due to myocardial infarction. These patients, and some less clearly defined patient groups with non-ischaemic cardiomyopathy and heart failure, may benefit from the insertion of an implantable cardioverter-defibrillator. With regard to pharmacological prevention, treatment of the underlying condition is the mainstay, since no antiarrhythmic substance-with the exemption of beta-blockers in some situations-has shown to be of efficacy.


EP Europace ◽  
2020 ◽  
Author(s):  
Konstantinos A Gatzoulis ◽  
Christos-Konstantinos Antoniou ◽  
Petros Arsenos ◽  
Dimitrios Tsiachris ◽  
Polychronis Dilaveris ◽  
...  

2018 ◽  
Vol 58 (2) ◽  
pp. 93-96 ◽  
Author(s):  
Lydia Krexi ◽  
Mary N Sheppard

Background In forensic practice, a blow to the chest can lead to sudden cardiac death (SCD). Commotio cordis and contusio cordis are leading causes. Methods From a database of 4678 patients who suffered from SCD, we found three patients with commotio cordis and two patients with contusio cordis. All the patients were examined macroscopically and microscopically and had negative toxicology screen. Results The three patients who died due to commotio cordis were young males (16, 23 and 38 years old). The circumstances of death were: a blow to the chest by a football, by a friend during a party and during an assault. The hearts were completely normal at autopsy. The two patients who had contusio cordis were older males (42 and 63 years old). Both patients died during traffic accidents. At autopsy, one had significant contusion over the left ventricle, and the second had contusion over the right ventricle. Conclusion This study indicates that a blow to the chest is very important to document in the circumstances of death, and a detailed history is vital. It raises the left ventricular intra-cavitary pressure, leading to commotio cordis with immediate death with a normal heart. Blunt chest trauma can cause direct myocardial lesions, with acute changes leading to contusio cordis.


Author(s):  

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.


2021 ◽  
Vol 17 ◽  
Author(s):  
Issa Pour-Ghaz ◽  
Mark Heckle ◽  
Ikechukwu Ifedili ◽  
Sharif Kayali ◽  
Christopher Nance ◽  
...  

: Implantable cardioverter-defibrillator (ICD) therapy is indicated for patients at risk for sudden cardiac death due to ventricular tachyarrhythmia. The most commonly used risk stratification algorithms use left ventricular ejection fraction (LVEF) to determine which patients qualify for ICD therapy, even though LVEF is a better marker of total mortality than ventricular tachyarrhythmias mortality. This review evaluates imaging tools and novel biomarkers proposed for better risk stratifying arrhythmic substrate, thereby identifying optimal ICD therapy candidates.


Author(s):  
Hyun-Jung Lee ◽  
Hyung-Kwan Kim ◽  
Sang Chol Lee ◽  
Jihoon Kim ◽  
Jun-Bean Park ◽  
...  

Abstract Aims We investigated the prognostic role of left ventricular global longitudinal strain (LV-GLS) and its incremental value to established risk models for predicting sudden cardiac death (SCD) in patients with hypertrophic cardiomyopathy (HCM). Methods and results LV-GLS was measured with vendor-independent software at a core laboratory in a cohort of 835 patients with HCM (aged 56.3 ± 12.2 years) followed-up for a median of 6.4 years. The primary endpoint was SCD events, including appropriate defibrillator therapy, within 5 years after the initial evaluation. The secondary endpoint was a composite of SCD events, heart failure admission, heart transplantation, and all-cause mortality. Twenty (2.4%) and 85 (10.2%) patients experienced the primary and secondary endpoints, respectively. Lower absolute LV-GLS quartiles, especially those worse than the median (−15.0%), were associated with progressively higher SCD event rates (P = 0.004). LV-GLS was associated with an increased risk for the primary endpoint, independent of the LV ejection fraction, apical aneurysm, and 2014 European Society of Cardiology (ESC) risk score [adjusted hazard ratio (aHR) 1.14, 95% confidence interval (CI) 1.02–1.28] or 2011 American College of Cardiology/American Heart Association (ACC/AHA) risk factors (aHR 1.18, 95% CI 1.05–1.32). LV-GLS was also associated with a higher risk for the composite secondary endpoint (aHR 1.06, 95% CI 1.01–1.12). The addition of LV-GLS enhanced the performance of the ESC risk score (C-statistic 0.756 vs. 0.842, P = 0.007) and the 2011 ACC/AHA risk factor strategy (C-statistic 0.743 vs. 0.814, P = 0.007) for predicting SCD. Conclusion LV-GLS is an important prognosticator in patients with HCM and provides additional information to established risk stratification strategies for predicting SCD.


2021 ◽  
Vol 19 ◽  
Author(s):  
Jean-Jacques Monsuez ◽  
Marilucy Lopez-Sublet

: Persons living with HIV infection (PLWH) have been recognized to have an increased risk of sudden cardiac death (SCD). Prevention of this risk should theoretically be included in their long-term management. However, only a few approaches have been proposed to optimize such interventions. Targeting detection of the commonly associated conditions such as coronary artery disease, left ventricular dysfunction, heart failure, QT interval prolongation and ventricular arrhythmias is the first step of this prevention. However, although detection of the risk of SCD is a suitable challenge in PLWH, it remains uncertain whether optimized treatment of the identified risks would unequivocally translate into a decrease in SCD rates.


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