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.

scholarly journals Characteristics of Patients With Arrhythmogenic Left Ventricular Cardiomyopathy

2020 ◽  
Vol 13 (12) ◽  
Author(s):  
Michela Casella ◽  
Alessio Gasperetti ◽  
Rita Sicuso ◽  
Edoardo Conte ◽  
Valentina Catto ◽  
...  
Keyword(s):  
Late Gadolinium Enhancement ◽  
Right Ventricular ◽  
Genetic Variants ◽  
Task Force ◽  
Arrhythmogenic Right Ventricular Cardiomyopathy ◽  
Left Ventricular ◽  
Ventricular Cardiomyopathy ◽  
Gadolinium Enhancement ◽  
Voltage Mapping ◽  
Fatty Replacement

Background: Arrhythmogenic left ventricular cardiomyopathy (ALVC) is an under-characterized phenotype of arrhythmogenic cardiomyopathy involving the LV ab initio. ALVC was not included in the 2010 International Task Force Criteria for arrhythmogenic right ventricular cardiomyopathy diagnosis and data regarding this phenotype are scarce. Methods: Clinical characteristics were reported from all consecutive patients diagnosed with ALVC, defined as a LV isolated late gadolinium enhancement and fibro-fatty replacement at cardiac magnetic resonance plus genetic variants associated with arrhythmogenic right ventricular cardiomyopathy and of an endomyocardial biopsy showing fibro-fatty replacement complying with the 2010 International Task Force Criteria in the LV. Results: Twenty-five patients ALVC (53 [48–59] years, 60% male) were enrolled. T wave inversion in infero-lateral and left precordial leads were the most common ECG abnormalities. Overall arrhythmic burden at study inclusion was 56%. Cardiac magnetic resonance showed LV late gadolinium enhancement in the LV lateral and posterior basal segments in all patients. In 72% of the patients an invasive evaluation was performed, in which electroanatomical voltage mapping and electroanatomical voltage mapping-guided endomyocardial biopsy showed low endocardial voltages and fibro-fatty replacement in areas of late gadolinium enhancement presence. Genetic variants in desmosomal genes (desmoplakin and desmoglein-2) were identified in 12/25 of the cohort presenting pathogenic/likely pathogenic variants. A definite/borderline 2010 International Task Force Criteria arrhythmogenic right ventricular cardiomyopathy diagnosis was reached only in 11/25 patients. Conclusions: ALVC presents with a preferential involvement of the lateral and postero-lateral basal LV and is associated mostly with variants in desmoplakin and desmoglein-2 genes. An amendment to the current International Task Force Criteria is reasonable to better diagnose patients with ALVC.

P992Incidence, predictors, and success of ventricular tachycardia catheter ablation in arrhythmogenic right ventricular cardiomyopathy (ARVC): A long-term cohort study from the Nordic ARVC registry

2019 ◽  
Vol 40 (Supplement_1) ◽  
Author(s):  
M K Christiansen ◽  
K Haugaa ◽  
A Svensson ◽  
T Gilljam ◽  
T Madsen ◽  
...  
Keyword(s):  
Ventricular Tachycardia ◽  
Catheter Ablation ◽  
Right Ventricular ◽  
Ventricular Arrhythmias ◽  
Task Force ◽  
Arrhythmogenic Right Ventricular Cardiomyopathy ◽  
Young Age ◽  
Clinical Indication ◽  
Ventricular Cardiomyopathy ◽  
Vt Ablation

Abstract Background Catheter ablation may reduce ventricular tachycardia (VT) burden in arrhythmogenic right ventricular cardiomyopathy (ARVC) patients. However, little is known about factors predicting need for ablation and various outcomes have been reported. Purpose We sought to investigate predictors and use of VT ablation and to evaluate the post-procedural outcome in ARVC patients. Methods We studied 435 patients from the Nordic ARVC registry including 220 probands with definite ARVC according to the 2010 task force criteria and 215 mutation-carrying relatives identified through cascade screening. Patients were followed until first-time VT ablation, death, heart transplantation, or January 1st 2018. Additionally, patients undergoing VT ablation were further followed from the time of ablation for recurrent ventricular arrhythmias. Results Cumulative use of VT ablation was 4% (95% CI 3%-6%) and 11% (95% CI 8%-15%) after 1 and 10 years. All procedures were performed in probands in whom the cumulative use was 8% (95% CI 5%-12%) and 20% (95% CI 15%-26%). In adjusted analyses restricted to probands, only young age predicted need for ablation. In patients undergoing ablation, risk of recurrent arrhythmias was 59% (95% CI 44%-71%) and 74% (95% CI 59%-84%) 1 and 5 years after the procedure. Despite high recurrence rates, the burden of ventricular arrhythmias was reduced after ablation (p=0.0042). Young age, use of several antiarrhythmic drugs and inducibility to VT immediately after ablation were associated with an unfavorable outcome. Conclusions Twenty percent of ARVC probands developed a clinical indication for VT ablation within 10 years after diagnosis whereas mutation-carrying relatives were without such need. Although the burden of ventricular arrhythmias decreased after ablation, risk of recurrence was substantial.

Long-Term Follow-Up in Patients with Arrhythmogenic Right Ventricular Cardiomyopathy

2012 ◽  
Vol 23 (7) ◽  
pp. 750-756 ◽  
Author(s):  
CHENG-HUNG LI ◽  
YENN-JIANG LIN ◽  
JIN-LONG HUANG ◽  
TSU-JUEY WU ◽  
CHEN-CHUAN CHENG ◽  
...  
Keyword(s):  
Right Ventricular ◽  
Arrhythmogenic Right Ventricular Cardiomyopathy ◽  
Ventricular Cardiomyopathy ◽  
Long Term Follow Up

scholarly journals First-in-world assessment of outcomes of catheter ablation for atrial arrhythmias in arrhythmogenic right ventricular cardiomyopathy

2020 ◽  
Vol 41 (Supplement_2) ◽  
Author(s):  
A Gasperetti ◽  
C.A James ◽  
L Chen ◽  
P Compagnucci ◽  
M Casella ◽  
...  
Keyword(s):  
Catheter Ablation ◽  
General Population ◽  
Right Ventricular ◽  
Left Atrial ◽  
Arrhythmogenic Right Ventricular Cardiomyopathy ◽  
Right Atrial ◽  
Atrial Arrhythmias ◽  
Ventricular Cardiomyopathy

Abstract Introduction Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a genetically inherited disease characterized by fibro-fatty infiltrations (FFI). FFI in ARVC patients usually originates in the ventricles, but recent imaging studies showed FFI at the atrial level as well. Effectiveness of catheter ablation (CA) for atrial arrhythmias (AA) in this subset of patients is currently unknown. Purpose Aim of our study is to describe acute and long-term effectiveness of CA for AA in ARVC patients. Methods Nine ARVC registries from Europe, US, and China were retrospectively searched for ARVC patients undergoing CA for AA (namely: atrial fibrillation (AF), atrial tachycardia (AT), and cavo-tricuspid dependent atrial flutter (CTI-FL)). Baseline, procedural, and long-term outcome data were collected. Results Thirty-five pts (86% male, median CHA2DS2-VASc 1 [1–2], HAS-BLED 1 [0–2], and EHRA scores 2 [2–3]) were enrolled, in which a total of 45 CA procedures for AA were performed (left atrial CA: n=19 AF, n=10 AT; right atrial CA: n=16 CTI). Mean age at AA CA was 48.2±14.8 y.o. At baseline, 63% of pts were on oral anticoagulants (OAC) (n=9 warfarin; n=13 NOAC). Catheter ablation was successful and sinus rhythm obtained at the end of the procedure in all patients, with 2 (6%) AF patients requiring electrical cardioversion. Over a median follow-up of 36 [14–74] months, 12 (27%) pts experienced arrhythmia recurrence (left atrial group: n=6 AF recurrences, n=3 AT recurrences; CTI-FL group: n=1 CTI-FL recurrence; n=1 new AF with previous CTI-dependent flutter ablation), with a 1-year follow-up resulting comparable to what has been reported in the literature for the general population. [Figure 1 and 2]. 61% pts were on OAC at last follow-up. Conclusion Age at the time of CA for AA is about 10 years younger in patients with ARVC as compared to the general population. CA for AA in ARVC pts is safe and effective; surprisingly, long-term CA outcomes for AF and left AT result comparable to those reported in the general population, whereas recurrence rates of CTI-dependent flutter seem to be higher. Funding Acknowledgement Type of funding source: None

scholarly journals Profile of arrythmogenic right ventricular cardiomyopathy/dysplasia (ARCV/D) patients presenting with sustained ventricular tachycardia in a tertiary care center

2018 ◽  
Vol 6 (4) ◽  
pp. 1211
Author(s):  
Rahul Sudan ◽  
Irfan Yaqoob ◽  
Khursheed Aslam ◽  
Mehroz Ahmad
Keyword(s):  
Ventricular Tachycardia ◽  
Right Ventricular ◽  
Task Force ◽  
Arrhythmogenic Right Ventricular Cardiomyopathy ◽  
Care Center ◽  
Tertiary Care ◽  
Sustained Ventricular Tachycardia ◽  
Clinical Profile ◽  
Haemodynamic Instability ◽  
Ventricular Cardiomyopathy

Background: Arrhythmogenic right ventricular cardiomyopathy /dysplasia (ARVC/D) is a genetic form of cardiomyopathy and is one among the most common causes of sudden cardiac death (SCD). The aim of our study was to analyze the clinical profile of (ARVC/D) patients presenting with sustained Ventricular Tachycardia (VT).Methods: This single center cohort study evaluated 107 patients who presented with sustained ventricular tachycardia (VT) in our hospital. After aetiological evaluation of all these patients, 15 patients were found to have ARVC/D as the cause of sustained ventricular tachycardia (VT) as per the Modified Task Force Criteria. The clinical profile of these patients was observed in detail to enhance our knowledge about this entity in our part of the world.Results: Mean age at presentation was 30 years and 12 patients were males. Nine patients were haemodynamically stable at the time of sustained VT and the rest of patients were haemodynamically unstable. Left Bundle Branch Block (LBBB) was the most common ECG morphology present in 11 patients. Antiarrhythmic drugs terminated VT in 7 patients. All the 6 patients presenting in a state of haemodynamic instability received DC cardioversion. Mortality occurred in 2 patients during the hospital stay.Conclusions: ARVC/D presenting with sustained VT is an important manifestation of the disease. Males are more commonly affected than females. Haemodynamic instability at the time of presentation carries a poor prognosis.

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