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 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 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

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 Sudden Cardiac Death Prediction in Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC): A Multinational Collaboration

2020 ◽  
Author(s):  
Julia Cadrin-Tourigny ◽  
Laurens P. Bosman ◽  
Weijia Wang ◽  
Rafik Tadros ◽  
Aditya Bhonsale ◽  
...  
Keyword(s):  
Sudden Cardiac Death ◽  
Right Ventricular ◽  
Cardiac Death ◽  
Cox Regression ◽  
Arrhythmogenic Right Ventricular Cardiomyopathy ◽  
Right Ventricular Ejection Fraction ◽  
Ventricular Cardiomyopathy ◽  
Clinical Predictors ◽  
Ventricular Ejection Fraction

Background - Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC) is associated with ventricular arrhythmias (VA) and sudden cardiac death (SCD). A model was recently developed to predict incident sustained VA in ARVC patients. However, since this outcome may overestimate the risk for SCD, we aimed to specifically predict life-threatening VA (LTVA) as a closer surrogate for SCD. Methods - We assembled a retrospective cohort of definite ARVC cases from 15 centers in North America and Europe. Association of 8 pre-specified clinical predictors with LTVA (SCD, aborted SCD, sustained or ICD treated VT>250 bpm) in follow-up was assessed by Cox regression with backward selection. Candidate variables included age, sex, prior sustained VA (≥30s, hemodynamically unstable or ICD treated VT; or aborted SCD), syncope, 24-hour premature ventricular complexes (PVC) count, the number of anterior and inferior leads with T-wave inversion (TWI), left and right ventricular ejection fraction. The resulting model was internally validated using bootstrapping. Results - A total of 864 definite ARVC patients (40±16 years; 53% male) were included. Over 5.75 years [IQR 2.77, 10.58] of follow-up, 93 (10.8%) patients experienced LTVA including 15 with SCD/aborted SCD (1.7%). Of the 8 pre-specified clinical predictors, only 4 (younger age, male sex, PVC count and number of leads with TWI) were associated with LTVA. Notably, prior sustained VA did not predict subsequent LTVA (p=0.850). A model including only these 4 predictors had an optimism-corrected C-index of 0.74 (95% CI:0.69-0.80) and calibration slope of 0.95 (95% CI:0.94-0.98) indicating minimal over-optimism. Conclusions - LTVA events in patients with ARVC can be predicted by a novel simple prediction model using only 4 clinical predictors. Prior sustained VA and the extent of functional heart disease are not associated with subsequent LTVA events.

Secondary prevention of sudden cardiac death in arrhythmogenic right ventricular cardiomyopathy

ESC CardioMed ◽  
2018 ◽  
pp. 2358-2361
Author(s):  
Katja Zeppenfeld ◽  
Sebastiaan R. D. Piers
Keyword(s):  
Sudden Cardiac Death ◽  
Ventricular Arrhythmia ◽  
Secondary Prevention ◽  
Right Ventricular ◽  
Cardiac Death ◽  
Beta Blockers ◽  
Arrhythmogenic Right Ventricular Cardiomyopathy ◽  
Ventricular Cardiomyopathy ◽  
Recurrence Rates ◽  
First Line Therapy

Sustained ventricular tachycardia (VT) and (aborted) sudden cardiac death are the presenting symptoms in 23–57% and 3–13% of patients who are diagnosed with arrhythmogenic right ventricular cardiomyopathy (ARVC), respectively. Implantation of an implantable cardioverter defibrillator (ICD) is recommended in patients with a history of aborted sudden cardiac death, haemodynamically poorly tolerated VT, and unexplained syncope, and should be considered in patients with haemodynamically well-tolerated sustained VT. Appropriate ICD intervention rates of up to 15%/year are observed in patients implanted for secondary prevention, the majority triggered by rapid and thereby potentially fatal monomorphic VT. Although life-saving, ICD therapy does not prevent ventricular arrhythmias, and therapeutic options to control ventricular arrhythmia burden are required. Beta blockers and sotalol are typically applied as first-line therapy, the latter mainly based on a study with serial programmed stimulation testing. Amiodarone may be superior in selected patients but data are based on small cohorts - large, prospective, observational and randomized trials are lacking. Up to 97% of ventricular arrhythmia episodes in ARVC are monomorphic VT with scar-related reentry as the dominant underlying mechanism, often involving subepicardial scar layers. Catheter ablation can result in a significant reduction of the ventricular arrhythmia burden with VT recurrence rates of 10–15%/year if a combined endocardial–epicardial ablation approach is performed in experienced tertiary referral centres.

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