scholarly journals SUDDEN CARDIAC DEATH IN PATIENTS WITH DILATED CARDIOMYOPATHY

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.

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.


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

2021 ◽  
Vol 8 ◽  
Author(s):  
Xuefeng Wang ◽  
Yong Luo ◽  
Jian Feng

Dilated cardiomyopathy is an etiologically heterogeneous disorder. Early diagnosis and prompt treatment of the underlying disease are of great significance. Primary and secondary adrenal insufficiency are considered quite rare causes of dilated cardiomyopathy. However, to the best of our knowledge, no case of cardiomyopathy associated with tertiary adrenal insufficiency has been reported. Herein, we described a 68-year-old woman with a 15-year history of seasonal dermatitis presented with frequent heart failure and shock. At first, she was diagnosed with idiopathic dilated cardiomyopathy, but standard heart failure and antishock treatment failed. Given her long-term use of dexamethasone for treating seasonal dermatitis, and clinical manifestations consistent with adrenal insufficiency, we tested her basal plasma cortisol, simultaneous corticotropin, and other pituitary hormones, confirming that she had tertiary adrenal insufficiency. Additionally, abdominal enhanced computed tomography revealed atrophic bilateral adrenal glands, indicating long-standing and severe adrenal insufficiency. Then hydrocortisone replacement therapy was initiated, and she recovered rapidly. During the next 2 years of follow-up, she never experienced any episodes of heart failure and shock. Unfortunately, she refused the implantation of defibrillator with cardiac resynchronization therapy (CRT-D) and died of sudden cardiac death 2 years later. Although we could not exclude the coincidence of idiopathic dilated cardiomyopathy with tertiary adrenal insufficiency with 100% certainty, her unique clinical course strongly indicated that her cardiomyopathy resulted from tertiary adrenal insufficiency. This case demonstrates that patients on corticosteroids are at risk for tertiary adrenal insufficiency, which may result in refractory cardiomyopathy and even sudden cardiac death.


ESC CardioMed ◽  
2018 ◽  
pp. 2316-2319
Author(s):  
Philipp Attanasio ◽  
Wilhelm Haverkamp

Identification of patients with hypertrophic cardiomyopathy (HCM) who are at high risk of sudden cardiac death (SCD) is essential, as life-threatening arrhythmic events can be effectively treated with implantable cardioverter defibrillator therapy. Various models for risk stratification of patients with HCM have been proposed. The latest clinical risk prediction model was developed in 2013. It is based on the HCM Risk-SCD study that included 3675 patients. Risk stratification using this model is recommended in the 2014 European Society of Cardiology (ESC) Guidelines for management of HCM and in the 2015 ESC Guidelines for the management of patients with ventricular arrhythmias and the prevention of SCD. This chapter summarizes novelties in the prediction model and the resulting recommendations, and discusses potential limitations of this approach.


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