scholarly journals Possibility of the Subcutaneous Implantable Cardioverter-Defibrillator for Prevention of Sudden Cardiac Death in Patients with Arrhythmogenic Right Ventricular Cardiomyopathy

2021 ◽  
Author(s):  
Shingo Sasaki
Keyword(s):  
Sudden Cardiac Death ◽  
Right Ventricular ◽  
Implantable Cardioverter Defibrillator ◽  
Cardiac Death ◽  
Arrhythmogenic Right Ventricular Cardiomyopathy ◽  
Young Patients ◽  
Ventricular Myocardium ◽  
Hereditary Disease ◽  
Ventricular Cardiomyopathy ◽  
Cardioverter Defibrillator

The EMBLEM™ entirely subcutaneous implantable cardioverter-defibrillator (S-ICD) system (Boston Scientific, Marlborough, Massachusetts, USA) was introduced as a new alternative to the conventional transvenous implantable cardioverter-defibrillator and has been expected to reduce device-related complications, especially in young patients who require long-term lead placement. Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a well-known hereditary disease recognized as a cause of sudden cardiac death (SCD) in young adults. However, the precise clinical role of S-ICD in patients with ARVC remains to be defined because of the low QRS amplitude of subcutaneous electrocardiogram (S-ECG) followed by the high incidence of inappropriate shock (IAS) delivery due to oversensing. It is well known that the sensing of S-ICD is largely dependent on the QRS/T ratio of S-ECG. The decrease in the QRS amplitude is more likely to lead to oversensing such as T wave or myopotential oversensing. In patients with ARVC, the decrease in the QRS amplitude due to degeneration of the right ventricular myocardium progresses overtime. In this chapter, we would like to discuss the usefulness of S-ICD lead repositioning for young adult patients with ARVC based on our experience of patients with IAS.

scholarly journals Arrhythmogenic right ventricular cardiomyopathy as a cause of sudden death in young people: Literature review

2012 ◽  
Vol 65 (9-10) ◽  
pp. 396-404
Author(s):  
Sanja Mazic ◽  
Biljana Lazovic ◽  
Marina Djelic
Keyword(s):  
Young People ◽  
Sudden Death ◽  
Right Ventricular ◽  
Implantable Cardioverter Defibrillator ◽  
Arrhythmogenic Right Ventricular Cardiomyopathy ◽  
Ventricular Myocardium ◽  
Fatty Tissue ◽  
Ventricular Cardiomyopathy ◽  
Cardioverter Defibrillator ◽  
Asymptomatic Patients

Arrhythmogenic right ventricular cardiomyopathy/dysplasia is a progressive condition with right ventricular myocardium being replaced by fibro-fatty tissue. It is a hereditary disorder mostly caused by desmosome gene mutations. The prevalence of arrhythmogenic right ventricular cardiomyopathy is about 1/1000-5000. Clinical presentation is usually related to ventricular tachycardias, syncope or presyncopa, or ventricular fibrillation leading to cardiac arrest, mostly in young people and athletes. It may be difficult to make the diagnosis of arrhythmogenic right ventricular cardiomyopathy due to several problems arising from the specificity of electrocardiograph abnormalities, different potential etiologies of ventricular arrhythmias with a left bundle branch morphology, the assessment of the right ventricular structure and function, and the interpretation of endomyocardial biopsy findings. Therefore, standardized diagnostic criteria have been proposed by the Study Group on arrhythmogenic right ventricular cardiomyopathy of the European Society of Cardiology. In order to make the diagnosis of arrhythmogenic right ventricular cardiomyopathy, a number of clinical tests are employed, including the electrocardiogram, echocardiography, myocardial perfusion scintigraphy, myocardial biopsy, right ventricular angiography, cardiac magnetic resonance imaging and genetic testing. The therapeutic options include beta blockers, antiarrhythmic drugs, catheter ablation, and implantable cardioverter defibrillator. The implantable cardioverter defibrillator is the most effective safe-guard against arrhythmic sudden death. Preparticipation screening for sport eligibility has been proven to be effective in detecting asymptomatic patients and sport disqualification has been lifesaving, substantially declining sudden death in young athletes.

scholarly journals Integrin β1D Deficiency–Mediated RyR2 Dysfunction Contributes to Catecholamine-Sensitive Ventricular Tachycardia in Arrhythmogenic Right Ventricular Cardiomyopathy

Circulation ◽  
2020 ◽  
Vol 141 (18) ◽  
pp. 1477-1493 ◽  
Author(s):  
Yihui Wang ◽  
Chunyan Li ◽  
Ling Shi ◽  
Xiuyu Chen ◽  
Chen Cui ◽  
...  
Keyword(s):  
Ventricular Tachycardia ◽  
Sudden Cardiac Death ◽  
Right Ventricular ◽  
Cardiac Death ◽  
Arrhythmogenic Right Ventricular Cardiomyopathy ◽  
Left Ventricular ◽  
Polymorphic Ventricular Tachycardia ◽  
Ventricular Cardiomyopathy ◽  
Open Probability ◽  
Increased Risk

Background: Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a hereditary heart disease characterized by fatty infiltration, life-threatening arrhythmias, and increased risk of sudden cardiac death. The guideline for management of ARVC in patients is to improve quality of life by reducing arrhythmic symptoms and to prevent sudden cardiac death. However, the mechanism underlying ARVC-associated cardiac arrhythmias remains poorly understood. Methods: Using protein mass spectrometry analyses, we identified that integrin β1 is downregulated in ARVC hearts without changes to Ca 2+ -handling proteins. As adult cardiomyocytes express only the β1D isoform, we generated a cardiac specific β1D knockout mouse model and performed functional imaging and biochemical analyses to determine the consequences of integrin β1D loss on function in the heart in vivo and in vitro. Results: Integrin β1D deficiency and RyR2 Ser-2030 hyperphosphorylation were detected by Western blotting in left ventricular tissues from patients with ARVC but not in patients with ischemic or hypertrophic cardiomyopathy. Using lipid bilayer patch clamp single channel recordings, we found that purified integrin β1D protein could stabilize RyR2 function by decreasing RyR2 open probability, mean open time, and increasing mean close time. Also, β1D knockout mice exhibited normal cardiac function and morphology but presented with catecholamine-sensitive polymorphic ventricular tachycardia, consistent with increased RyR2 Ser-2030 phosphorylation and aberrant Ca 2+ handling in β1D knockout cardiomyocytes. Mechanistically, we revealed that loss of DSP (desmoplakin) induces integrin β1D deficiency in ARVC mediated through an ERK1/2 (extracellular signal–regulated kinase 1 and 2)–fibronectin–ubiquitin/lysosome pathway. Conclusions: Our data suggest that integrin β1D deficiency represents a novel mechanism underlying the increased risk of ventricular arrhythmias in patients with ARVC.

scholarly journals Arrhythmogenic right ventricular cardiomyopathy or athlete's heart? Challenges in assessment of right heart morphology and function

2019 ◽  
Vol 89 (1) ◽  
Author(s):  
Francesco Antonini-Canterin ◽  
Concetta Di Nora
Keyword(s):  
Sudden Cardiac Death ◽  
Right Ventricular ◽  
Cardiac Death ◽  
Arrhythmogenic Right Ventricular Cardiomyopathy ◽  
Ventricular Cardiomyopathy ◽  
Young Athletes ◽  
Right Heart ◽  
Lack Of Information ◽  
And Function ◽  
Athlete's Heart

The incidence of sudden cardiac death (SCD) in young athletes varies among studies, due to the disagreement in the definitions and the lack of information in this field.

scholarly journals Arrhythmogenic Right Ventricular Cardiomyopathy Causing Sudden Cardiac Death in Boxer Dogs

Circulation ◽  
2004 ◽  
Vol 109 (9) ◽  
pp. 1180-1185 ◽  
Author(s):  
Cristina Basso ◽  
Philip R. Fox ◽  
Kathryn M. Meurs ◽  
Jeffrey A. Towbin ◽  
Alan W. Spier ◽  
...  
Keyword(s):  
Sudden Cardiac Death ◽  
Right Ventricular ◽  
Cardiac Death ◽  
Arrhythmogenic Right Ventricular Cardiomyopathy ◽  
Ventricular Cardiomyopathy ◽  
Boxer Dogs
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