PRESENTATION AND DISEASE PROGRESSION OF CHILDREN AND ADOLESCENTS EVALUATED FOR ARRHYTHMOGENIC RIGHT VENTRICULAR CARDIOMYOPATHY

2015 ◽  
Vol 31 (10) ◽  
pp. S188-S189
Author(s):  
P. Chungsomprasong ◽  
R. Hamilton ◽  
M. Fatah ◽  
M. Seed ◽  
C. Manlhiot ◽  
...  
Keyword(s):  
Right Ventricular ◽  
Children And Adolescents ◽  
Disease Progression ◽  
Arrhythmogenic Right Ventricular Cardiomyopathy ◽  
Ventricular Cardiomyopathy

scholarly journals Angiotensin-Converting Enzyme Inhibitors/Angiotensin Receptor Blockers: Anti-arrhythmic Drug for Arrhythmogenic Right Ventricular Cardiomyopathy

2021 ◽  
Vol 8 ◽  
Author(s):  
Bin Tu ◽  
Lingmin Wu ◽  
Lihui Zheng ◽  
Shangyu Liu ◽  
Lishui Sheng ◽  
...  
Keyword(s):  
Ventricular Arrhythmia ◽  
Right Ventricular ◽  
Disease Progression ◽  
Mixed Model ◽  
Linear Mixed Model ◽  
Angiotensin Receptor Blockers ◽  
Arrhythmogenic Right Ventricular Cardiomyopathy ◽  
Right Ventricular Outflow Tract ◽  
Ventricular Cardiomyopathy ◽  
Life Threatening

Background: Current treatment guidelines for arrhythmogenic right ventricular cardiomyopathy (ARVC) mainly emphasize on prevention of ventricular arrhythmic events. Despite the progressive nature of ARVC, therapeutic options focusing on decelerating disease progression are scarce.Methods and Results: This retrospective observational cohort study included 311 patients [age, 39.1 ± 14.4 years; male, 233 (74.9%)] with a definite diagnosis of ARVC as determined by the 2010 Task Force Diagnostic Criteria. Among them, 113 patients (36.3%) received ACEI/ARB treatment. Disease progression was evaluated according to repeat transthoracic echocardiograms with a linear mixed model. Patients receiving ACEI/ARB treatment were associated with slower disease progression reflected by a gradual decrease in tricuspid annular plane systolic excursion than those not receiving ACEI/ARB treatment (0.37 vs. 0.61 mm per year decrease, P < 0.001) and slower dilation of right ventricular outflow tract (0.57 vs. 1.06 mm per year increased, P = 0.003). Cox proportional hazard regression models were used to evaluate the association between life-threatening ventricular tachycardia events and ACEI/ARB treatment. A reduced risk of life-threatening ventricular arrhythmia was associated with ACEI/ARB treatment compared to that without ACEI/ARB treatment (adjusted HR: 0.71, 95% CI: 0.52–0.96, P = 0.031).Conclusions: ACEI/ARB treatment is associated with slower disease progression and lower risk of life-threatening ventricular arrhythmia in patients with ARVC. Delaying disease progression may pave way for reducing life-threatening ventricular arrhythmia risk.

scholarly journals Exercise Dose Associated With Military Service: Implications for the Clinical Management of Inherited Risk for Arrhythmogenic Right Ventricular Cardiomyopathy

2020 ◽  
Vol 185 (9-10) ◽  
pp. e1447-e1452
Author(s):  
Elena M Segre ◽  
Lydia D Hellwig ◽  
Clesson Turner ◽  
Craig P Dobson ◽  
Mark C Haigney
Keyword(s):  
Physical Activity ◽  
Physical Fitness ◽  
Right Ventricular ◽  
Aerobic Exercise ◽  
Disease Progression ◽  
Clinical Outcomes ◽  
Military Service ◽  
Arrhythmogenic Right Ventricular Cardiomyopathy ◽  
Therapeutic Window ◽  
Ventricular Cardiomyopathy

Abstract Introduction High levels of aerobic exercise in individuals who have a gene mutation associated with arrhythmogenic right ventricular cardiomyopathy (ARVC) are associated with clinical disease progression. Guidelines consequently restrict patients from competitive athletics. However, there is minimal literature to guide the safe dosing of physical activity outside of the setting of competitive athletics. Patients may be physically active pursuant to a variety of careers, including military service. This study aimed to define a therapeutic window for exercise for ARVC gene-positive individuals that are compatible with continuing military service and general health while maintaining a level of exercise below that which risks disease progression. Materials and Methods Using standard metabolic equations, we calculated the minimum VO2 max (amount of oxygen utilized at peak exercise capacity) required to pass the physical fitness tests for each branch. We then developed a sample exercise prescription to maintain this level of fitness. We compared the prescribed exercise load with the physical activity levels associated with non-inferior clinical outcomes in ARVC gene-positive individuals. Additionally, we determined the physical activity exposure sustained by service members based on self-report data and compared these values with the upper limit of safe exercise exposure. Results Based on a review of the currently available literature, aerobic exercise exposure less than 700 to 1,100 MET-hours/year (metabolic equivalent-hours per year) is not associated with inferior clinical outcomes for gene-positive individuals. A military service member needs 600 to 700 MET-hours/year to minimally pass the physical fitness test. However, many military members are exercising in excess of this minimum, with typical exposures between 900 and 2,400 MET-hours/year. Conclusions A therapeutic window of aerobic exercise may exist for ARVC gene-positive individuals which would allow continuation of military service while maintaining levels of exercise restriction associated with non-inferior clinical outcomes.

Abstract 12941: Disease Progression in Patients With Arrhythmogenic Right Ventricular Cardiomyopathy and Ventricular Tachycardia: A Longitudinal Study With Unipolar Voltage Mapping

Circulation ◽  
2014 ◽  
Vol 130 (suppl_2) ◽  
Author(s):  
Pasquale Santangeli ◽  
Ioan Liuba ◽  
Cory Tschabrunn ◽  
Michael Riley ◽  
Fermin Garcia ◽  
...  
Keyword(s):  
Ventricular Tachycardia ◽  
Right Ventricular ◽  
Disease Progression ◽  
Surface Area ◽  
Task Force ◽  
Arrhythmogenic Right Ventricular Cardiomyopathy ◽  
Ventricular Cardiomyopathy ◽  
Voltage Mapping

Introduction: Arrhythmogenic right ventricular cardiomyopathy (ARVC) is believed to result in progressive fibrofatty replacement of the RV myocardium with development of multiple ventricular tachycardia (VT) circuits. Endocardial unipolar voltage mapping has been shown to reliably identify epicardial and endocardial (epi-endo) scar in ARVC. Hypothesis: We studied the prevalence and mechanisms of disease progression in patients with ARVC and VT through longitudinal unipolar voltage mapping studies. Methods: Eighteen consecutive patients (age 38±14 years) who fulfilled the revised Task Force criteria for ARVC underwent two detailed sinus rhythm endocardial unipolar voltage maps (mean 348±118 points) performed a median of 36 months apart (interquartile range 21 to 36 months, minimum 9 months) as part of VT ablation procedures. Epi-endo scar was defined using established unipolar voltage cutoff (5.5 mV). The extent of RV scar and total RV volume was measured by a dedicated software. A >5% increase in RV scar area or volume over follow-up was considered significant. Results: At baseline, all patients had evidence of epi-endo RV scar (mean 141±82 cm 2 ; 56±27% of the RV surface area). After a mean follow-up of 49±36 months, no significant progression of unipolar voltage scar was observed (mean 159±83 cm 2 , P=0.14; 63±27% of the RV surface area, P=0.07). Specifically, only 3 (17%) patients presented with progression of the RV scar >5%. The RV volumes increased during follow-up (from 206±74 mL to 258±77 mL, P=0.0003), with the majority of patients (15/18, 83%) having a significant increase in the RV volume (mean increase = 38.9%). Only 3 (17%) patients had no change in both RV scar size and volume over time. Conclusions: In patients with ARVC and VT, progressive RV dilatation is almost uniformly observed, while rapid epi-endo scar progression is rare. These findings suggest that aggressive epi-endocardial substrate ablation should provide long-term VT control, and further research is needed to identify the mechanism(s) for and to prevent ongoing RV dilatation in these patients.

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