scholarly journals Efficacy of epicardial and endocardial ablation in the prevention of ventricular tachycardia in arrythmogenic right ventricular cardiomyopathy- a meta analysis

2020 ◽  
Vol 41 (Supplement_2) ◽  
Author(s):  
Y Sattar ◽  
W Ullah ◽  
S Mamtani ◽  
C Alraies
Keyword(s):  
Ventricular Tachycardia ◽  
Catheter Ablation ◽  
Sudden Cardiac Death ◽  
Right Ventricular ◽  
Cardiac Death ◽  
Polymorphic Ventricular Tachycardia ◽  
Secondary Outcome ◽  
Ventricular Cardiomyopathy ◽  
Mapping Techniques ◽  
Post Hoc

Abstract Introduction Ventricular tachycardia is a major complication associated with increased risk of sudden cardiac death in arrhythmogenic ventricular cardiomyopathy. Recurrence of VT status post catheter endocardial ablation with conventional mapping is a evolving discussion in management of VT prevention in ARVC. With the evolution of new mapping techniques to locate ectopic foci of VT, a combination of endo- and epicardial catheter ablation have proven to be efficacious in the prevention of frequency of VT recurrence and its duration. Methods Using PubMed, Ovid (MEDLINE) and Cochrane database we searched using the MeSH terms including: “arrhythmogenic right ventricular cardiomyopathy”, “arrhythmogenic right ventricular dysplasia”, “monomorphic ventricular tachycardia”, “polymorphic ventricular Tachycardia”, “endocardial catheter ablation”, “epicardial catheter ablation”. The primary outcomes were to assess VT frequency and duration status post endocardial or epicardial or a combination of both types of ablation. The secondary outcome includes sudden cardiac arrest or sudden cardiac death after procedure. ANOVA with post HOC analysis was performed using SPSS v.26 (IBM Corp, NY, USA) Results A total of 33 studies included 1437 patients with a mean male=67%. The data analysis showed a mean VT prevention for endocardial ablation was 65%, epicardial 78%, and for combined epi-endocardial was 89% (figure-1). The mean procedural mortality rate was 2%. In order to test the hypothesis that combined epi-endocardial ablation was more successful in the prevention of VT recurrence, we performed a one-way analysis of variance (ANOVA). The analysis was statistically significant F(2,14)=5.879, 95% CI, p=0.014. Post Hoc test (Tukey HSD test) with multiple comparisons indicated that patients who underwent combined epi-endocardial ablation experienced a statistically significant difference in VT prevention of 89% (95% CI p=0.01) compared to only endocardial ablation, mean VT prevention of 65% (95% CI, p=0.189) or only epicardial, mean VT prevention of 78% (95% CI, p=0.353). Conclusion With new mapping techniques, use of endocardial, and epicardial ablation is linked to decrease VT frequency, duration, ICD shocks, and sudden cardiac death in patients with ARVC in cohorts with prior failure of antiarrhythmics. Total VT Prevention across target sites Funding Acknowledgement Type of funding source: None

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.

Risk stratification for sudden cardiac death in arrhythmogenic right ventricular cardiomyopathy

ESC CardioMed ◽  
2018 ◽  
pp. 2320-2322
Author(s):  
Firat Duru ◽  
Corinna Brunckhorst
Keyword(s):  
Risk Factors ◽  
Ventricular Tachycardia ◽  
Sudden Cardiac Death ◽  
Left Ventricle ◽  
Right Ventricular ◽  
Cardiac Death ◽  
Arrhythmogenic Right Ventricular Cardiomyopathy ◽  
Sustained Ventricular Tachycardia ◽  
Ventricular Cardiomyopathy ◽  
The Right

Arrhythmogenic right ventricular cardiomyopathy is an inherited disease characterized by fibrofatty infiltration of the myocardium. Patients suffer from palpitations, presyncope, or syncope, which typically present during or after physical exercise. However, in a minority of cases, sudden cardiac death (SCD) may be the first disease manifestation. SCD in patients with arrhythmogenic right ventricular cardiomyopathy is difficult to predict and there are often no alarming signs or symptoms. Patients with a history of an aborted SCD due to ventricular fibrillation and those who have sustained ventricular tachycardia, or severe dysfunction of the right or left ventricle, or both, are considered to be at high risk for future arrhythmic events. The occurrence of unexplained syncope, non-sustained ventricular tachycardia, and moderate dysfunction of the right or left ventricle, or both, puts the patient at an intermediate risk. Other independent risk factors for adverse events include inducibility at programmed ventricular stimulation, young age at the time of diagnosis, male sex, and compound/digenic heterozygosity of desmosomal gene mutations. Electrocardiographic risk factors for adverse arrhythmic events include T-wave inversion across precordial and inferior leads, low QRS amplitude, and QRS fragmentation. Healthy gene carriers are considered to be at low risk for future arrhythmic events.

scholarly journals Late Presentation of Arrhythmogenic Right Ventricular Cardiomyopathy : Role of Non Invasive Modalities for The Diagnosis

2019 ◽  
Vol 6 (2) ◽  
pp. 131-136
Author(s):  
Safir Sungkar ◽  
Mochamad Arif Nugroho
Keyword(s):  
Ventricular Tachycardia ◽  
Sudden Cardiac Death ◽  
Right Ventricular ◽  
Cardiac Death ◽  
Arrhythmogenic Right Ventricular Cardiomyopathy ◽  
The Elderly ◽  
Late Presentation ◽  
Ventricular Cardiomyopathy ◽  
Non Invasive

Background : Arrhythmogenic right ventricular cardiomyopathy(ARVC) is an inherited myocardial disease affecting predominantly young people and manifests as sustained ventricular tachycardia, sudden cardiac death (SCD) or heart failure. However, its first manifestation in older patients is infrequent. Diagnosis of ARVC remains a clinical challenge and need further investigation. Our case report investigated role of non invasive modalities for diagnosis of ARVC patient. Case Presentation : A 65 year old man was admitted to the hospital with symptoms of palpitationand near syncope. An Electrocardiogram (ECG) showed a sustained VT with LBBB morphology and inferior axis. The patient was cardioverted to sinus rhytm with a single 100J shock. Postcardioversion ECG showed an epsilon wave in right precordial leads. Echocardiography revealed extensive RV enlargement and reduce function. Our patient had three major (RV aneurysm, epsilon wave and T wave inversion) and one minor criteria (sustained LBBB type-VT with inferior axis)making the diagnosis of ARVC definite according to the revised Task Forced Criteria. Conclusion: ARVC may have a very late presentation and this diagnosis should be considered as a potential cause of sustained VT of RV origin among the elderly. ECG and echocardiography as non invasive modalities have an important role for the diagnosis of patients with suspected ARVC. Keywords :Arrhythmogenic right ventricular cardiomyopathy,ventricular tachycardia, sudden cardiac death, diagnosis.

Sustained monomorphic ventricular tachycardia: the role of catheter ablation

ESC CardioMed ◽  
2018 ◽  
pp. 2279-2288
Author(s):  
Tilman Maurer ◽  
William G. Stevenson ◽  
Karl-Heinz Kuck
Keyword(s):  
Heart Disease ◽  
Ventricular Tachycardia ◽  
Catheter Ablation ◽  
Sudden Cardiac Death ◽  
Cardiac Death ◽  
Therapeutic Option ◽  
Structural Heart Disease ◽  
Premature Ventricular Contractions ◽  
Monomorphic Ventricular Tachycardia ◽  
Premature Beats

Monomorphic ventricular tachycardia (VT) may occur in the presence or absence of structural heart disease. The standard therapy for patients with structural heart disease at high risk of sudden cardiac death due to VT is the implantable cardioverter defibrillator (ICD). While ICDs effectively terminate VT and prevent sudden cardiac death, they do not prevent recurrent episodes of VT, since the underlying arrhythmogenic substrate remains unchanged. However, shocks from an ICD increase mortality and impair quality of life. These limitations as well as continuous advancements in technology have made catheter ablation an important treatment strategy for patients with structural heart disease presenting with VT. Idiopathic ventricular arrhythmias include premature ventricular contractions and VT occurring in the absence of overt structural heart disease. In this setting, catheter ablation has evolved as the primary therapeutic option for symptomatic ventricular premature beats and sustained VTs and is curative in most cases. This chapter presents an overview of the principles of invasive diagnosis and treatment of monomorphic VTs in patients with and without structural heart disease and delineates the clinical outcome of catheter ablation. Finally, the chapter provides an outlook to the future, discussing potential directions and upcoming developments in the field of catheter ablation of monomorphic VT.

P992Incidence, predictors, and success of ventricular tachycardia catheter ablation in arrhythmogenic right ventricular cardiomyopathy (ARVC): A long-term cohort study from the Nordic ARVC registry

2019 ◽  
Vol 40 (Supplement_1) ◽  
Author(s):  
M K Christiansen ◽  
K Haugaa ◽  
A Svensson ◽  
T Gilljam ◽  
T Madsen ◽  
...  
Keyword(s):  
Ventricular Tachycardia ◽  
Catheter Ablation ◽  
Right Ventricular ◽  
Ventricular Arrhythmias ◽  
Task Force ◽  
Arrhythmogenic Right Ventricular Cardiomyopathy ◽  
Young Age ◽  
Clinical Indication ◽  
Ventricular Cardiomyopathy ◽  
Vt Ablation

Abstract Background Catheter ablation may reduce ventricular tachycardia (VT) burden in arrhythmogenic right ventricular cardiomyopathy (ARVC) patients. However, little is known about factors predicting need for ablation and various outcomes have been reported. Purpose We sought to investigate predictors and use of VT ablation and to evaluate the post-procedural outcome in ARVC patients. Methods We studied 435 patients from the Nordic ARVC registry including 220 probands with definite ARVC according to the 2010 task force criteria and 215 mutation-carrying relatives identified through cascade screening. Patients were followed until first-time VT ablation, death, heart transplantation, or January 1st 2018. Additionally, patients undergoing VT ablation were further followed from the time of ablation for recurrent ventricular arrhythmias. Results Cumulative use of VT ablation was 4% (95% CI 3%-6%) and 11% (95% CI 8%-15%) after 1 and 10 years. All procedures were performed in probands in whom the cumulative use was 8% (95% CI 5%-12%) and 20% (95% CI 15%-26%). In adjusted analyses restricted to probands, only young age predicted need for ablation. In patients undergoing ablation, risk of recurrent arrhythmias was 59% (95% CI 44%-71%) and 74% (95% CI 59%-84%) 1 and 5 years after the procedure. Despite high recurrence rates, the burden of ventricular arrhythmias was reduced after ablation (p=0.0042). Young age, use of several antiarrhythmic drugs and inducibility to VT immediately after ablation were associated with an unfavorable outcome. Conclusions Twenty percent of ARVC probands developed a clinical indication for VT ablation within 10 years after diagnosis whereas mutation-carrying relatives were without such need. Although the burden of ventricular arrhythmias decreased after ablation, risk of recurrence was substantial.

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