Implantable Cardioverter‐Defibrillator Therapy in Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy: Predictors of Appropriate Therapy, Outcomes, and Complications

Orgeron, Gabriela M.; James, Cynthia A.; Riele, Anneline Te; Tichnell, Crystal; Murray, Brittney; Bhonsale, Aditya; Kamel, Ihab R.; Zimmerman, Stephan L.; Judge, Daniel P.; Crosson, Jane E.; Tandri, Harikrishna; Calkins, Hugh

doi:10.1161/jaha.117.006242

Cited by 77 publications

(83 citation statements)

References 26 publications

(113 reference statements)

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…The primary outcome was the first appropriate ICD therapy for a VA. Review of intracardiac ECGs or device interrogation interpretation by the referring electrophysiologists when the ECG was unavailable were used to adjudicate arrhythmias, as described previously 13. Decisions regarding implantation and programming of ICDs were made by individual managing electrophysiologists on a case‐by‐case basis.…”

Section: Methodsmentioning

confidence: 99%

“…Baseline data included sex, date of birth, date and type of presentation, family history, and results of cardiac testing consisting of 12‐lead ECG, 24‐hour Holter monitoring, 2‐dimensional transthoracic echocardiography, and cardiac magnetic resonance imaging. These covariates were selected based on reported risk factors for VA in ARVC 1, 13, 14, 15, 16. Genotype included the results of sequencing of the desmosomal genes PKP2 (plakophilin 2), DSG2 (desmoglein 2), DSC2 (desmocollin‐2), DSP (desmoplakin), and JUP (plakoglobin) at a minimum.…”

Section: Methodsmentioning

confidence: 99%

See 1 more Smart Citation

Impact of Exercise Restriction on Arrhythmic Risk Among Patients With Arrhythmogenic Right Ventricular Cardiomyopathy

Wang

Orgeron

Tichnell

et al. 2018

JAHA

Self Cite

View full text Add to dashboard Cite

BackgroundPrior studies have shown a close link between exercise and development of arrhythmogenic right ventricular cardiomyopathy. How much exercise restriction reduces ventricular arrhythmia (VA), how genotype modifies its benefit, and whether it reduces risk sufficiently to defer implantable cardioverter‐defibrillator (ICD) placement in arrhythmogenic right ventricular cardiomyopathy are unknown.Methods and ResultsWe interviewed 129 arrhythmogenic right ventricular cardiomyopathy patients (age: 34.0±14.8 years; male: 60%) with ICDs (36% primary prevention) about exercise participation. Exercise change was defined as annual exercise duration and dose in the 3 years before clinical presentation minus that after presentation. The primary outcome was appropriate ICD therapy for VA. During the 5.1 years (interquartile range: 2.7–10.8 years) after presentation, 74% (95/129) patients reduced exercise dose and 85 (66%) patients experienced the primary outcome. In multivariate analyses, top tertile reduction in exercise duration and dose were both associated with less VA (duration: hazard ratio: 0.23 [95% confidence interval, 0.07–0.81]; dose: hazard ratio: 0.14 [95% confidence interval, 0.04–0.44]). Greater reduction in exercise dose conferred greater reduction in VA (P=0.01 for trend). Patients without desmosomal mutations and those with primary‐prevention ICDs benefited more from exercise reduction (P=0.16 and P=0.06 for interaction); however, 58% (18/31) of athletes who reduced exercise dose by >80% still experienced VA.ConclusionsExercise restriction should be recommended to all arrhythmogenic right ventricular cardiomyopathy patients with ICDs. Patients who are “gene‐elusive” and those with primary‐prevention devices may particularly benefit. Exercise reduction is unlikely to reduce arrhythmia sufficiently in high‐risk patients to alter decision‐making regarding ICD implantation.

show abstract

Section: Methodsmentioning

confidence: 99%

Section: Methodsmentioning

confidence: 99%

Impact of Exercise Restriction on Arrhythmic Risk Among Patients With Arrhythmogenic Right Ventricular Cardiomyopathy

Wang

Orgeron

Tichnell

et al. 2018

JAHA

Self Cite

View full text Add to dashboard Cite

show abstract

“…Among these eight studies, the most recent and largest study reporting the outcomes of ICD therapy in patients with ARVC was published in 2017 41 . This study reported the outcomes of 312 ARVC patients (163 men, age at presentation 33.6±13.9) who received an ICD.…”

Section: Risk Stratification In Patients With Arvcmentioning

confidence: 99%

“…Relatively little is known regarding the outcomes of ARVC patients who have a subcutaneous ICD implanted 41, 44 . Among the 312 ARVC patients described in the recent large report of the outcomes of ICD therapy in ARVC patients, 7 had a subcutaneous ICD implanted 41 .…”

Section: Risk Stratification In Patients With Arvcmentioning

confidence: 99%

Risk Stratification in Arrhythmogenic Right Ventricular Cardiomyopathy

2017

Self Cite

View full text Add to dashboard Cite

Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC) is an inherited cardiomyopathy that is characterized by ventricular arrhythmias, and an increased risk of sudden death (SCD). Although structural abnormalities of the right ventricle predominate, it is well recognized that left ventricular involvement is common particularly in advanced disease, and that left dominant forms occur. The pathologic characteristic of ARVC/D is myocyte loss with fibrofatty replacement. Since the first detailed clinical description of the disorder in 1982, significant advances have been made in understanding this disease. Once the diagnosis of ARVC is established, the single most important clinical decision is whether a particular patient’s SCD risk is sufficient to justify placement of an implantable cardioverter defibrillator (ICD). The importance of this decision reflects the fact that ARVC is a common cause of sudden death in young people, and that sudden cardiac death may be the first manifestation of the disease. This decision is particularly important since these are often young patients who are expected to live for many years. While an ICD can save lives in individuals with this disease, it is also well recognized that ICD therapy is associated with both short and long term complications. Decisions regarding placement of an ICD are based on an estimate of a patient’s risk of SCD, as well as their preferences and values. The primary purpose of this article is to provide a review of literature that concerns risk stratification in patients with ARVC and to place this literature into the framework of the three authors considerable lifetime experiences in caring for patients with ARVC. The most important parameters to consider when determining arrhythmic risk include: 1) electrical instability including frequency of PVC’s and sustained VA, 2) proband status, 3) extent of structural disease, 4) cardiac syncope, 5) male gender, 6) the presence of multiple mutations or a mutation in TMEM 43, and 7) the patient’s willingness to restrict exercise and eliminate participation in competitive or endurance exercise.

show abstract

“…International guidelines recommend an ICD for secondary prevention in patients with an inherited cardiomyopathy who have suffered a cardiac arrest, and have no serious neurological sequelae or other life limiting illnesses 4344. Use of ICDs for primary prevention in selected patients is supported by several observational studies 41454647. However, in non-ischaemic heart failure, one high quality randomised trial (1116 patients) did not find survival benefit except in a subgroup of younger patients 48.…”

Section: How Are the Inherited Cardiomyopathies Managed?mentioning

confidence: 99%