Patients with arrhythmogenic right ventricular cardiomyopathy/dysplasia remain at risk of sudden death despite therapy with beta-blockers and/or class III antarrhythmic drugs

Heart Rhythm ◽  
2005 ◽  
Vol 2 (5) ◽  
pp. S229
Author(s):  
Domenico Corrado ◽  
Loira Leoni ◽  
Barbara Tokajuk ◽  
Luca Rebellato ◽  
Gianfranco Buja ◽  
...  
Keyword(s):  
At Risk ◽  
Sudden Death ◽  
Right Ventricular ◽  
Beta Blockers ◽  
Arrhythmogenic Right Ventricular Cardiomyopathy ◽  
Class Iii ◽  
Ventricular Cardiomyopathy

Abstract 3003: Programmed Ventricular Stimulation Does Not Predict the Outcome of Patients with Arrhythmogenic Right Ventricular Cardiomyopathy (from DARVIN study)

Circulation ◽  
2007 ◽  
Vol 116 (suppl_16) ◽  
Author(s):  
Domenico Corrado ◽  
Loira Leoni ◽  
Mark S Link ◽  
Hugh Calkins ◽  
Thomas Wichter ◽  
...  
Keyword(s):  
Sudden Death ◽  
Right Ventricular ◽  
Predictive Value ◽  
Arrhythmogenic Right Ventricular Cardiomyopathy ◽  
Ventricular Cardiomyopathy ◽  
Icd Therapy ◽  
Programmed Ventricular Stimulation ◽  
Ventricular Stimulation ◽  
History Of

Background: The Defibrillator in Arrhythmogenic Right Ventricular Cardiomyopathy International (DARVIN) study was a multicenter investigation that enrolled patients with arrhythmogenic right ventricular cardiomyopathy (ARVC) who received an implantable defibrillator (ICD) for either secondary or primary prevention of sudden death. Methods: In this DARVIN substudy, we examined whether programmed ventricular stimulation (PVS) is able to predict the arrhythmic risk in a large cohort of 201 ARVC patients (133 males, 68 females, aged 36 ± 12 years) who received an ICD. Implant indications were a history of cardiac arrest in 13 (6%) patients; sustained ventricular tachycardia (VT) in 82 (41%); syncope in 42 (21%); asymptomatic nonsustained VT in 40 (20%); and a family history of sudden death in 24 (12%). PVS prior to ICD implantation was carried out in 143 of 201 patients (71%). All antiarrhythmic drugs were discontinued ≥ 5 half-lives (≥ 6 weeks for amiodarone) before the study. PVS included a minimum of 2 drive cycles length and up to 3 ventricular extrastimuli while pacing from two right ventricular sites. Results: One hundred-nine patients (76%) were inducible to either sustained VT (patients 70; 64%), with a mean cycle length of 287 ± 66ms (range 220 to 410 ms), or ventricular fibrillation/flutter (VF) (patients 39; 36%). Of 109 patients who were inducible at PVS, 56 (52%) did not experience ICD therapy during a mean follow-up of 47 ± 22 months, whereas 11 of 34 (33%) noninducible patients had appropriate ICD interventions. Overall, the positive predictive value of PVS was 48%, the negative predictive value 67%, and the test accuracy 53%. The incidence of ICD discharges on VF, which in all likelihood would have been fatal in the absence of ICD therapy, did not differ between patients who were and were not inducible at PVS (26 of 109, 24% vs 7 of 34, 21%; p=0.87), regardless of clinical presentation. The type of ventricular arrhythmia inducible at PVS did not predict VF during the follow-up. Conclusions: The presence (or absence) of an inducible arrhythmia on PVS did not correlate with subsequent appropriate ICD interventions, suggesting a limited role for PVS in arrhythmic risk stratification of ARVC patient population. A negative PVS may not indicate better prognosis.

Arrhythmogenic right ventricular cardiomyopathy: management of symptoms and prevention of sudden cardiac death

2018 ◽  
Author(s):  
Perry Elliott ◽  
Alexandros Protonotarios
Keyword(s):  
Heart Failure ◽  
Ventricular Tachycardia ◽  
Right Ventricular ◽  
Ventricular Arrhythmias ◽  
Ventricular Dysfunction ◽  
Beta Blockers ◽  
Arrhythmogenic Right Ventricular Cardiomyopathy ◽  
Sustained Ventricular Tachycardia ◽  
Carrier Status ◽  
Ventricular Cardiomyopathy

Patients with arrhythmogenic right ventricular cardiomyopathy (ARVC) have arrhythmia-related symptoms or are identified during screening of an affected family. Heart failure symptoms occur late in the disease’s natural history. As strenuous exercise has been associated with disease acceleration and worsening of ventricular arrhythmias, lifestyle modification with restricted athletic activities is recommended upon disease diagnosis or even identification of mutation carrier status. An episode of an haemodynamically unstable, sustained ventricular tachycardia or ventricular fibrillation as well as severe systolic ventricular dysfunction constitute definitive indications for implantable cardioverter defibrillator (ICD) implantation, which should also be considered following tolerated sustained or non-sustained ventricular tachycardia episodes, syncope, or in the presence of moderate ventricular dysfunction. Antiarrhythmic medications are used as an adjunct to device therapy. Catheter ablation is recommended for incessant ventricular tachycardia or frequent appropriate ICD interventions despite maximal pharmacological therapy. Amiodarone alone or in combination with beta blockers is most effective for symptomatic ventricular arrhythmias. Beta blockers are considered for use in all patients with a definite diagnosis but evidence for their prognostic benefit is sparse. Heart failure symptoms are managed using standard protocols and heart transplantation is considered for severe ventricular dysfunction or much less commonly uncontrollable ventricular arrhythmias.

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.

Arrhythmogenic right ventricular cardiomyopathy: management of symptoms and prevention of sudden cardiac death

ESC CardioMed ◽  
2018 ◽  
pp. 1502-1505
Author(s):  
Alexandros Protonotarios ◽  
Perry Elliott
Keyword(s):  
Heart Failure ◽  
Ventricular Tachycardia ◽  
Right Ventricular ◽  
Ventricular Arrhythmias ◽  
Ventricular Dysfunction ◽  
Beta Blockers ◽  
Arrhythmogenic Right Ventricular Cardiomyopathy ◽  
Pharmacological Therapy ◽  
Sustained Ventricular Tachycardia ◽  
Ventricular Cardiomyopathy

Patients with arrhythmogenic right ventricular cardiomyopathy (ARVC) have arrhythmia-related symptoms or are identified during screening of an affected family. Heart failure symptoms occur late in the disease’s natural history. As strenuous exercise has been associated with disease acceleration and worsening of ventricular arrhythmias, lifestyle modification with restricted athletic activities is recommended upon disease diagnosis or even identification of mutation carrier status. An episode of an haemodynamically unstable, sustained ventricular tachycardia or ventricular fibrillation as well as severe systolic ventricular dysfunction constitute definitive indications for implantable cardioverter defibrillator (ICD) implantation, which should also be considered following tolerated sustained or non-sustained ventricular tachycardia episodes, syncope, or in the presence of moderate ventricular dysfunction. Antiarrhythmic medications are used as an adjunct to device therapy. Catheter ablation is recommended for incessant ventricular tachycardia or frequent appropriate ICD interventions despite maximal pharmacological therapy. Amiodarone alone or in combination with beta blockers is most effective for symptomatic ventricular arrhythmias. Beta blockers are considered for use in all patients with a definite diagnosis but evidence for their prognostic benefit is sparse. Heart failure symptoms are managed using standard protocols and heart transplantation is considered for severe ventricular dysfunction or much less commonly uncontrollable ventricular arrhythmias.

Nationwide (Denmark) Study of Symptoms Preceding Sudden Death due to Arrhythmogenic Right Ventricular Cardiomyopathy

2014 ◽  
Vol 113 (7) ◽  
pp. 1250-1254 ◽  
Author(s):  
Golnaz Sadjadieh ◽  
Reza Jabbari ◽  
Bjarke Risgaard ◽  
Morten S. Olesen ◽  
Stig Haunsø ◽  
...  
Keyword(s):  
Sudden Death ◽  
Right Ventricular ◽  
Arrhythmogenic Right Ventricular Cardiomyopathy ◽  
Ventricular Cardiomyopathy

scholarly journals Arrhythmogenic Right Ventricular Cardiomyopathy – Antiarrhythmic Therapy

2015 ◽  
Vol 04 (2) ◽  
pp. 86 ◽  
Author(s):  
Simon Ermakov ◽  
Melvin Scheinman ◽  
◽  
Keyword(s):  
Right Ventricular ◽  
Beta Blockers ◽  
Arrhythmogenic Right Ventricular Cardiomyopathy ◽  
Single Agent ◽  
Ventricular Myocardium ◽  
Channel Blockers ◽  
Antiarrhythmic Agents ◽  
Implantable Cardioverter Defibrillators ◽  
Ventricular Cardiomyopathy ◽  
The Individual

Arrhythmogenic right ventricular cardiomyopathy is an inherited disorder characterised by progressive replacement of ventricular myocardium by fibrofatty tissue that predisposes patients to ventricular arrhythmias, heart failure and sudden death. Treatment focuses on slowing disease progression, decreasing the burden of arrhythmias and preventing sudden cardiac death through placement of implantable cardioverter-defibrillators (ICDs), catheter ablation and the use of antiarrhythmic medication. Although only ICDs have been demonstrated to affect patient mortality, antiarrhythmic medications are important adjuncts in reducing patient morbidity and inappropriate ICD therapy. Of the individual antiarrhythmic agents available, sotalol, beta-blockers and amiodarone appear to be most effective in arrhythmia suppression. Calcium-channel blockers may be effective in selected patients. For patients who are refractory to single agent therapy, combination therapy may be considered with the most effective combinations being sotalol + flecainide and amiodarone + beta-blockers.

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