Risk Stratification in Arrhythmogenic Right Ventricular Cardiomyopathy

Abstract

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.

Characteristics of recurrent ventricular tachyarrhythmia after catheter ablation in patients with arrhythmogenic right ventricular cardiomyopathy

“…Endurance exercise can increase the pressure, afterload, and wall stress to a disproportionately greater extent in the RV than in the left ventricle . Exercise can also aggravate the mechanical uncoupling of cardiomyocytes, promote the progression of ARVC, and trigger ventricular arrhythmic events . Furthermore, participation in strenuous exercise after the diagnosis of ARVC was associated with lethal ventricular arrhythmia .…”

“…23 Exercise can also aggravate the mechanical uncoupling of cardiomyocytes, promote the progression of ARVC, and trigger ventricular arrhythmic events. [23][24][25][26][27][28] Furthermore, participation in strenuous exercise after the diagnosis of ARVC was associated with lethal ventricular arrhythmia. 29 The present study also firstly demon- The association between reasons of VA recurrence and previous ablation results in ARVC patients with multiple procedures.…”

Misdiagnosis of ARVC leading to inappropriate ICD implant and subsequent ICD removal – lessons learned

“…It is important for cardiologists to recognize that merely establishment of the diagnosis of ARVC does not automatically warrant ICD implantation. Tools to stratify sudden death risk are now available . The most recent contribution to this topic is the development of a personalized risk calculator that clinicians can use to estimate the annual and 5 year risk of a sustained ventricular in a patient with ARVC on the basis of simple criteria, such as age, sex, prior syncope, PVC frequency, and NSVT …”

Arrhythmic outcome of arrhythmogenic right ventricular cardiomyopathy patients without implantable defibrillators

“…1 Implantable cardiac defibrillators (ICD) are the only proven intervention that protects against sudden death. [3][4][5] Therefore, some have argued that the mere act of implanting an ICD inflates the malignant arrhythmia rate in ARVC. Large series show 40-70% patients have appropriate ICD therapy for sustained ventricular arrhythmias after implantation.…”

“…Large series show 40-70% patients have appropriate ICD therapy for sustained ventricular arrhythmias after implantation. [3][4][5] Therefore, some have argued that the mere act of implanting an ICD inflates the malignant arrhythmia rate in ARVC. However, there is a paucity of data characterizing arrhythmic outcomes in not affected by…”