Usefulness of Electrocardiographic Parameters for Risk Prediction in Arrhythmogenic Right Ventricular Dysplasia

Saguner, Ardan M.; Ganahl, Sabrina; Baldinger, Samuel H.; Kraus, Andrea; Medeiros-Domingo, Argelia; Nordbeck, Sebastian; Saguner, Arhan R.; Mueller-Burri, Andreas S.; Haegeli, Laurent; Wölber, Thomas; Steffel, Jan; Krasniqi, Nazmi; Delacrétaz, Etienne; Lüscher, Thomas F.; Held, Leonhard; Brunckhorst, Corinna; Duru, Fırat

doi:10.1016/j.amjcard.2014.02.031

Cited by 58 publications

(39 citation statements)

References 29 publications

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“…TWI ≥ V1–V3 is an important sign used in diagnosis, reflecting abnormalities in ventricular repolarization . Previous studies have demonstrated that TWI in multiple precordial leads is an independent predictor of arrhythmic events in patients with ARVC . In the current study, no significant difference in the cumulative survival rate was seen between patients with TWI ≥ V1–V3 and those without.…”

Section: Discussioncontrasting

confidence: 55%

Long‐term follow‐up of arrhythmogenic right ventricular cardiomyopathy patients with an implantable cardioverter‐defibrillator for prevention of sudden cardiac death

Yin

Ding

et al. 2017

Clinical Cardiology

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Background Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a rare inherited cardiomyopathy with a high burden of ventricular arrhythmia, which is an important cause of sudden cardiac death (SCD). Implantable cardioverter‐defibrillator (ICD) is believed to be the most reliable management against SCD. Hypothesis Ventricular arrhythmia does not necessarily confer a poor prognosis in ARVC patients with an ICD. Methods A total of 39 ARVC patients (34 male) implanted with an ICD at our electrophysiology center and followed up continuously were included in this study. The mean age at diagnosis was 42.1 ± 14.8 years. Results Thirty‐three patients (84.6%) had suffered ventricular arrhythmia with hemodynamic compromise before ICD implantation. During a median follow‐up of 48.6 months (interquartile range, 32.3–73.3), 3 patients (7.7%) died, 1 of sudden death, 1 of heart failure, and 1 of cerebral infarction. Twenty‐eight patients (71.8%) experienced 540 appropriate ICD interventions. The first appropriate ICD intervention occurred more than 2 years after initial ICD implantation in 5 patients (12.8%). Twelve patients (30.8%) suffered from electrical storm. The event‐free period was significantly shorter in patients who did not have broad precordial T wave inversion ≥V1–V3 (hazard ratio = 0.39, 95% confidence interval: 0.16‐0.96). No significant difference was shown in antiarrhythmic drugs and radiofrequency catheter ablation before ICD implantation between patients with and without appropriate ICD therapies (P > 0.05). Conclusions Recurrence of sustained ventricular tachycardia/ventricular fibrillation is frequent in high‐risk patients with ARVC. The prognosis is favorable for ARVC patients treated with an ICD for prevention of SCD.

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Section: Discussioncontrasting

confidence: 55%

Long‐term follow‐up of arrhythmogenic right ventricular cardiomyopathy patients with an implantable cardioverter‐defibrillator for prevention of sudden cardiac death

Yin

Ding

et al. 2017

Clinical Cardiology

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“…For example, studies have shown that patients with a greater number of T wave inversions on a 12 lead ECG have a higher arrhythmic risk 39, 40, 46 . There are reports that patients with more extensive abnormalities detected on a SAECG have a higher arrhythmic risk 47 . Several studies have shown that patients with more extensive RV and/or LV disease have a higher arrhythmic risk 35, 48–49 .…”

Section: Selected Topics In Risk Stratification Of Arvc Patientsmentioning

confidence: 99%

Risk Stratification in Arrhythmogenic Right Ventricular Cardiomyopathy

2017

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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.

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“…Risk stratification in patients with ARVD/C Several factors have been proposed [2,[14][15][16] for stratifying the risk of mortality and/or ventricular tachyarrhythmias in ARVD/C. Corrado et al [17] established an arrhythmic risk stratification pyramid, which categorized patients with ARVD/C into highest, intermediate, and lowest risk groups according to the variables shown in Fig.…”

Section: Risk Stratification and Disease Progression Of Arvd/cmentioning

confidence: 99%

Current and state of the art on the electrophysiologic characteristics and catheter ablation of arrhythmogenic right ventricular dysplasia/cardiomyopathy

Chung

Lin

Chang³

et al. 2015

Journal of Cardiology

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Arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C) is an inherited genetic disease caused by defective desmosomal proteins, and it has typical histopathological features characterized by predominantly progressive fibro-fatty infiltration of the right ventricle. Clinical presentations of ARVD/C vary from syncope, progressive heart failure (HF), ventricular tachyarrhythmias, and sudden cardiac death (SCD). The 2010 modified Task Force criteria were established to facilitate the recognition and diagnosis of ARVD/C. An implantable cardiac defibrillator (ICD) remains to be the cornerstone in prevention of SCD in patients fulfilling the diagnosis of definite ARVD/C, especially among ARVD/C patients with syncope, hemodynamically unstable ventricular tachycardia (VT), ventricular fibrillation, and aborted SCD. Further risk stratification is clinically valuable in the management of patients with borderline or possible ARVD/C and mutation carriers of family members. However, given the entity of heterogeneous penetrance and non-uniform phenotypes, the standardization of clinical practice guidelines for at-risk individuals will be the next frontier to breakthrough. Antiarrhythmic drugs are prescribed frequently to patients experiencing frequent ventricular tachyarrhythmias and/or appropriate ICD shocks. Amiodarone is the recommended drug of choice. Radiofrequency catheter ablation (RFCA) has been demonstrated to effectively eliminate the drug-refractory VT in patients with ARVD/C. However, the efficacy and clinical prognosis of RFCA via endocardial approach alone was disappointing prior to the era of epicardial approach. In recent years, it has been proven that the integration of endocardial and epicardial ablation by targeting the critical isthmus or eliminating abnormal electrograms within the diseased substrates could yield higher acute success and lower recurrence of ventricular tachyarrhythmias during long-term follow-up. Heart transplantation is the final option for patients with extensive disease, biventricular HF with uncontrollable hemodynamic compromise, and refractory ventricular tachyarrhythmias despite aggressive medical and ablation therapies.

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Usefulness of Electrocardiographic Parameters for Risk Prediction in Arrhythmogenic Right Ventricular Dysplasia

Cited by 58 publications

References 29 publications

Long‐term follow‐up of arrhythmogenic right ventricular cardiomyopathy patients with an implantable cardioverter‐defibrillator for prevention of sudden cardiac death

Long‐term follow‐up of arrhythmogenic right ventricular cardiomyopathy patients with an implantable cardioverter‐defibrillator for prevention of sudden cardiac death

Risk Stratification in Arrhythmogenic Right Ventricular Cardiomyopathy

Current and state of the art on the electrophysiologic characteristics and catheter ablation of arrhythmogenic right ventricular dysplasia/cardiomyopathy

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