scholarly journals Sudden Cardiac Death in Hereditary Dilated Cardiomyopathy

Author(s):  
Marianna Leopoulou ◽  
Jo Ann LeQuang ◽  
Joseph V. Pergolizzi ◽  
Peter Magnusson

Dilated cardiomyopathy (DCM) is characterized by the phenotype of a dilated left ventricle with systolic dysfunction. It is classified as hereditary when it is deemed of genetic origin; more than 50 genes are reported to be related to the condition. Symptoms include, among others, dyspnea, fatigue, arrhythmias, and syncope. Unfortunately, sudden cardiac death may be the first manifestation of the disease. Risk stratification regarding sudden death in hereditary DCM as well as preventive management poses a challenge due to the heterogeneity of the disease. The purpose of this chapter is to present the epidemiology, risk stratification, and preventive strategies of sudden cardiac death in hereditary DCM.

Author(s):  
R. W. Roudijk ◽  
K. Taha ◽  
M. Bourfiss ◽  
P. Loh ◽  
L. van den Heuvel ◽  
...  

AbstractIn relatives of index patients with dilated cardiomyopathy and arrhythmogenic cardiomyopathy, early detection of disease onset is essential to prevent sudden cardiac death and facilitate early treatment of heart failure. However, the optimal screening interval and combination of diagnostic techniques are unknown. The clinical course of disease in index patients and their relatives is variable due to incomplete and age-dependent penetrance. Several biomarkers, electrocardiographic and imaging (echocardiographic deformation imaging and cardiac magnetic resonance imaging) techniques are promising non-invasive methods for detection of subclinical cardiomyopathy. However, these techniques need optimisation and integration into clinical practice. Furthermore, determining the optimal interval and intensity of cascade screening may require a personalised approach. To address this, the CVON-eDETECT (early detection of disease in cardiomyopathy mutation carriers) consortium aims to integrate electronic health record data from long-term follow-up, diagnostic data sets, tissue and plasma samples in a multidisciplinary biobank environment to provide personalised risk stratification for heart failure and sudden cardiac death. Adequate risk stratification may lead to personalised screening, treatment and optimal timing of implantable cardioverter defibrillator implantation. In this article, we describe non-invasive diagnostic techniques used for detection of subclinical disease in relatives of index patients with dilated cardiomyopathy and arrhythmogenic cardiomyopathy.


Author(s):  
Gustav Mattsson ◽  
Peter Magnusson

Heart failure implies a considerable burden for patients and resources for the health care system. Dilated cardiomyopathy is defined as left ventricular dilation and reduced systolic function, not solely explained by ischemic heart disease or abnormal loading conditions. Numerous genes have been identified in familial cases of dilated cardiomyopathy. Heart failure with reduced ejection fraction increases the risk for sudden cardiac death. Implantable cardioverter defibrillator therapy can provide a means of preventing sudden cardiac death in those deemed to be at high risk. Health care providers are in need of better tools in order to improve risk stratification. This chapter aims to provide an overview of the current knowledge about risk of arrhythmia and sudden death in patients with familial dilated cardiomyopathy, in particular for those patients with a specific mutation.


2014 ◽  
Vol 5 (4) ◽  
pp. 41-48
Author(s):  
D V Duplyakov ◽  
I V Kondratyeva ◽  
S V Garkina

The review discusses accumulated to date scientific data on dilated cardiomyopathy: causative factors, clinical manifestations, sudden cardiac death in patients with the disease and its risk-stratification in the light of National guidelines on the definition of risk and prevention of sudden cardiac death (2012). A case of a patient with dilated cardiomyopathy and high-risk of sudden cardiac death is presented.


2006 ◽  
Vol 134 (11-12) ◽  
pp. 482-487 ◽  
Author(s):  
Branislav Milovanovic ◽  
Mirjana Krotin ◽  
Dejana Vukovic ◽  
Vesna Bisenic ◽  
Tijana Mirjanic ◽  
...  

Introduction: It has been shown that depolarization disorders, autonomic dysfunction, and systolic dysfunction of the left ventricle are associated with sudden cardiac death after myocardial infarction. Objective: The Objective of study was to examine the prognostic value of the most important predictors in the first week after myocardial infarction. Method: Study included 881 patients who were followed up from 1 to 60 months. During the first week after myocardial infarction, following examination were performed: ECG with standard leads and X, Y, Z orthogonal leads, vectorcardiogram, QT interval, late potentials, short-time spectral analysis of RR variability, nonlinear (Poincare plot) analysis and echocardiogram. Results: In univariate analysis, the following parameters measured on the first day were important predictors of sudden cardiac death: lower LF/HF ratio(<1.5) (p=0.000), T wave inversion in X lead (p=0.000), high P wave in D2 lead (p=0.030), and diminished systolic function (p=0.000). In multivariate analysis, the following parameters were significant risk predictors: T wave inversion in X lead, lower LF/HF ratio, positive late potentials and the left ventricle systolic dysfunction. Conclusion: The parameters of the left ventricle systolic dysfunction with sympathicovagal imbalance and electric instability are the key risk predictors in the first few days after myocardial infarction.


Heart ◽  
2017 ◽  
Vol 103 (Suppl 1) ◽  
pp. A2.1-A2
Author(s):  
Brian P Halliday ◽  
Ankur Gulati ◽  
Aamir Ali ◽  
Kaushik Guha ◽  
Simon Newsome ◽  
...  

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