Abstract 2256: Predictors of “Life-Threatening” Fast VT or VF in Arrhythmogenic Right Ventricular Dysplasia

Circulation ◽  
2007 ◽  
Vol 116 (suppl_16) ◽  
Author(s):  
Douglas W Laidlaw ◽  
David Lucier ◽  
James P Daubert ◽  
Frank I Marcus ◽  
Melvin M Scheinman ◽  
...  
Keyword(s):  
Primary Prevention ◽  
Secondary Prevention ◽  
Right Ventricular ◽  
Task Force ◽  
Cumulative Risk ◽  
Arrhythmogenic Right Ventricular Dysplasia ◽  
Icd Therapy ◽  
Right Ventricular Dysplasia ◽  
Life Threatening

Introduction: The Multidisciplinary Study of Right Ventricular Dysplasia is an NIH-funded, multicenter, prospective registry of patients meeting Task-Force criteria for arrhythmogenic right ventricular dysplasia (ARVD). Previous studies have used the occurrence of “life-threatening” fast VT or VF as a surrogate for sudden cardiac death in patients with an ICD, however the patient characteristics associated with sudden cardiac death in ARVD remain poorly defined. Methods: All patients in the ARVD registry that underwent implantation of an ICD were studied. Baseline characteristics of patients who received ICD therapy for fast VT/VF (with a cycle length of <250msec) were compared to those patients who did not receive ICD therapy. The cumulative risk of appropriate ICD therapy for fast VT/VF was determined by the Kaplan-Meier method for patients receiving an ICD for primary or secondary prevention. Results: 80 patients in the ARVD registry underwent implantation of an ICD. Of those, 61 patients (76%) had an ICD implanted for secondary prevention and 19 patients (24%) for primary prevention. Over a mean follow-up of 1.7 years, 17.5% of patients experienced appropriate ICD therapy for fast VT/VF, including 18% of patients with an ICD implanted for secondary prevention and 15.8% of patients with and ICD implanted for primary prevention. Among all baseline imaging, echocardiographic, and electrcardiographic characteristics, none were significantly associated with the occurance of fast VT/VF during follow-up. In a Kaplan-Meier analysis, there was no significant diffference in the cumulative risk of ICD therapy for fast VT/VF between primary or secondary prevention cohorts (log rank p-value = 0.69). Conclusions: In this registry of patients meeting the current Task Force criteria for ARVD, there is a high incidence of “life-threatening” fast VT/VF during follow-up, and the probability of fast VT/VF is similar in patients with an ICD for secondary prevention compared to those with an ICD for primary prevention. Furthermore, baseline characteristics do not reliably predict a future risk of life threatening arrhythmias in this cohort.

Abstract 9395: The Clinical Role of Atrial Fibrillation in Patients with Arrhythmogenic Right Ventricular Dysplasia

Circulation ◽  
2014 ◽  
Vol 130 (suppl_2) ◽  
Author(s):  
Ardan M Saguner ◽  
Samuel Baldinger ◽  
Argelia Medeiros-Domingo ◽  
Sabrina Ganahl ◽  
Felix C Tanner ◽  
...  
Keyword(s):  
Atrial Fibrillation ◽  
Right Ventricular ◽  
Cardiac Death ◽  
Task Force ◽  
Tertiary Care ◽  
Arrhythmogenic Right Ventricular Dysplasia ◽  
Right Atrial ◽  
Clinical Role ◽  
Right Ventricular Dysplasia ◽  
Increased Risk

Introduction: Atrial fibrillation/flutter (Afib/Aflu) in general, and clinical variables predicting Afib/Aflu in particular, are not well defined in patients with arrhythmogenic right ventricular dysplasia (ARVD). Hypothesis: We hypothesized that transthoracic echocardiography (TTE) and ECG could be helpful to predict Afib/Aflu in these patients. Methods and Results: 12-lead ECGs and TTEs of 90 patients from three tertiary-care centers diagnosed with definite or borderline ARVD according to the 2010 Task Force Criteria were analyzed. Data were compared in two patient groups: (1) patients with Afib/Aflu and (2) all other patients. Eighteen (20%) patients experienced Afib/Aflu during a follow-up period of 5.8 years (interquartile range 2.0-10.4 years). Kaplan-Meier analysis (Figure) revealed reduced times to Afib/Aflu among patients with echocardiographic RV fractional area change <27% (p<0.001), left atrial diameter ≥24.4 mm/m2 (p=0.001), and right atrial short axis diameter ≥22.1 mm/m2 (p=0.05). From all ECG variables, P sinistroatriale conferred the highest hazard ratio (3.37, 95% CI 0.92-12.36, p=0.067). Five patients with Afib/Aflu experienced inappropriate ICD shocks compared to four patients without Afib/Aflu (36% vs. 9%, p=0.03). Presence of Afib/Aflu was more prevalent in heart transplanted patients and in those who succumbed to cardiac death compared to the remaining patients (56% vs. 16%, p=0.014). Conclusions: Afib/Aflu are associated with inappropriate ICD shocks, heart transplantation, and cardiac death in patients with ARVD. Echocardiographic evidence of reduced RV function and atrial dilation helps to identify those ARVD patients being at increased risk for Afib/Aflu, which may help to guide individual patient management.

Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy According to Revised 2010 Task Force Criteria With Inclusion of Non-Desmosomal Phospholamban Mutation Carriers

2013 ◽  
Vol 112 (8) ◽  
pp. 1197-1206 ◽  
Author(s):  
Judith A. Groeneweg ◽  
Paul A. van der Zwaag ◽  
Louise R.A. Olde Nordkamp ◽  
Hennie Bikker ◽  
Jan D.H. Jongbloed ◽  
...  
Keyword(s):  
Right Ventricular ◽  
Task Force ◽  
Arrhythmogenic Right Ventricular Dysplasia ◽  
Right Ventricular Dysplasia ◽  
Mutation Carriers

16-year follow-up of arrhythmogenic right ventricular dysplasia

1984 ◽  
Vol 108 (5) ◽  
pp. 1363-1365 ◽  
Author(s):  
Seiji Higuchi ◽  
Nail M Çaǧlar ◽  
Rihei Shimada ◽  
Akira Yamada ◽  
Akira Takeshita ◽  
...  
Keyword(s):  
Right Ventricular ◽  
Arrhythmogenic Right Ventricular Dysplasia ◽  
Right Ventricular Dysplasia

scholarly journals Risk of subsequent ventricular arrhythmia is higher in primary prevention patients with implantable cardioverter defibrillator than in secondary prevention patients

2019 ◽  
Vol 19 (1) ◽  
Author(s):  
You Zhou ◽  
Shuang Zhao ◽  
Keping Chen ◽  
Wei Hua ◽  
Yangang Su ◽  
...  
Keyword(s):  
Primary Prevention ◽  
Ventricular Arrhythmia ◽  
Secondary Prevention ◽  
Implantable Cardioverter Defibrillator ◽  
Incidence Rate ◽  
Home Monitoring ◽  
Icd Therapy ◽  
Cardioverter Defibrillator ◽  
All Cause Mortality

Abstract Background Because of previous ventricular arrhythmia (VA) episodes, patients with implantable cardioverter-defibrillator (ICD) for secondary prevention (SP) are generally considered to have a higher burden of VAs than primary prevention (PP) patients. However, when PP patients experienced VA, the difference in the prognosis of these two patient groups was unknown. Methods The clinical characteristics and follow-up data of 835 ICD patients (364 SP patients and 471 PP patients) with home monitoring feature were retrospectively analysed. The incidence rate and risk of subsequent VA and all-cause mortality were compared between PP patients after the first appropriate ICD therapy and SP patients. Results During a mean follow-up of 44.72 ± 20.87 months, 210 (44.59%) PP patients underwent appropriate ICD therapy. In the Kaplan-Meier survival analysis, the PP patients after appropriate ICD therapy were more prone to VA recurrence and all-cause mortality than SP patients (P<0.001 for both endpoints). The rate of appropriate ICD therapy and all-cause mortality in PP patients after the first appropriate ICD therapy was significantly higher than that in SP patients (for device therapy, 59.46 vs 20.64 patients per 100 patient-years; incidence rate ratio [IRR] 2.880, 95% confidence interval [CI]: 2.305–3.599; P<0.001; for all-cause mortality, 14.08 vs 5.40 deaths per 100 patient-years; IRR 2.607, 95% CI: 1.884–3.606; P<0.001). After propensity score matching for baseline characteristics, the risk of VA recurrence in PP patients with appropriate ICD therapy was still higher than that in SP patients (41.80 vs 19.10 patients per 100 patient-years; IRR 2.491, 95% CI: 1.889–3.287; P<0.001), but all-cause mortality rates were similar between the two groups (12.61 vs 9.33 deaths per 100 patient-years; IRR 1.352, 95% CI: 0.927–1.972; P = 0.117). Conclusions Once PP patients undergo appropriate ICD therapy, they will be more prone to VA recurrence and death than SP patients.

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