Abstract 13804: Correlation between Electrocardiographic Features and Local Activation Pattern in Arrhythmogenic Right Ventricular Dysplasia

Circulation ◽  
2014 ◽  
Vol 130 (suppl_2) ◽  
Author(s):  
Tanyanan Tanawuttiwat ◽  
Anneline S Te Riele ◽  
Binu Philips ◽  
Cynthia A James ◽  
Brittney Murray ◽  
...  

Background: Depolarization abnormalities in the terminal portion of the QRS are frequently seen in Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy (ARVD/C). The purpose of this study was to correlate the electroanatomic activation pattern of the RV endocardium and epicardium to the surface ECG. Methods: Thirty consecutive ARVD/C patients (Mean age 33.1 +/- 11.2 years, 16 (53%) men) underwent detailed endocardial and epicardial electroanatomical mapping (EAM). Local sinus rhythm activation was annotated at the sharpest intrinsic deflection of the bipolar electrogram, including late potentials. ECG features were classified into 5 major patterns; 1. Normal QRS (12 patients) 2. Epsilon wave (5 patients) 3. Incomplete RBBB (5 patients) 4. Atypical complete RBBB (6 patients) and 5. Prolonged terminal activation duration (TAD) (2 patients) Results: The earliest endocardial and epicardial RV activation occurred on the mid anteroseptal wall on all ECG patterns. Figure 1 represented activation area (purple) after the QRS or during the delayed depolarization phase. Nearly all endocardial and epicardial RV was activated well within the QRS duration in patients with normal QRS but was activated during R’ in patients with CRBBB. The delayed activation during Epsilon wave consistently occurred in basal anterior wall and basal angle of RV. In patients with TAD, the activation of RVOT and basal angle RV represented slurred S wave. Conclusion: ECG features in ARVD/C are correlated with late activation in specific regions of RV and total endocardial activation time. The delay activation of basal anterior wall and basal angle of RV represents the Epsilon wave in the right precordial ECG.

2021 ◽  
Vol 2 (1) ◽  
pp. 109-114
Author(s):  
E. V. Solovyoval ◽  
N. A. Popova ◽  
T. V. Vlasoval ◽  
M. L. Gorbunova ◽  
L. N. Antsygina

Arhythmogenic right ventricular dysplasia (ADP) refers to hereditary myocardial diseases, in which there are structural and functional disorders in the right ventricular myocardium, causing rhythm and conduction disorders, including fatal ventricular arrhythmias. ADP is considered one of the most common causes of sudden cardiac death in young people and people who are engaged in sports. However, in practice, there are cases of this disease in people of an older age category. Diagnosis of ADP is still difficult due to the possible long-term asymptomatic course of the disease. The article describes a clinical case of ADP in a 48-year-old man.


Kardiologiia ◽  
2019 ◽  
Vol 59 (6) ◽  
pp. 86-90
Author(s):  
M. N. Baranova ◽  
M. Yu. Ogarkov ◽  
A. E. Skripchenko ◽  
F. N. Chavdar ◽  
A. Yu. Yankin ◽  
...  

Arrhythmogenic dysplasia of the right ventricle is a rare pathology of the myocardium, the diagnosis of which is difficult. Isolated myocardial infarction of the right ventricle occurs and is diagnosed extremely rarely. In this article we describe a case of arrhythmogenic right ventricular dysplasia, complicated by transmural infarction of the anterolateral wall of the right ventricle, myocardial rupture, and cardiac tamponade.


2020 ◽  
Vol 5 (1) ◽  
Author(s):  
Selvaraja GR

A sudden unexpected death in a healthy individual often leads to panic within a community and it’s frequently associated with malicious mischief. Suicide, infections, genetic or congenital abnormalities may well lead to sudden deaths. All these possibilities are vital in determining the cause of death and facilitate investigation, hence must be considered judiciously. Here, we report a case of a 49-year-old gentleman without underlying medical illness or prior history of heart disease found dead in his home by his wife. Postmortem and histopathology findings determined the cause of death. External examination revealed no obvious injuries whereas 50% narrowing was disclosed in the left anterior descending artery at internal examination. Histology finding showed fibro-fatty replacement of the right ventricle myocardium. Toxicological analyses were unrewarding. It was concluded that the cause of death was arrhythmogenic right ventricular dysplasia. In conclusion, the findings of the forensic autopsy and histopathology facilitated the investigation and established the cause of death. The cause of death attributed to arrhythmogenic right ventricular dysplasia (ARVD). This case demonstrates ARVD can have a late presentation and it should always be borne in mind as a potential cause of death, necessitating this case report.


Circulation ◽  
2007 ◽  
Vol 116 (suppl_16) ◽  
Author(s):  
Harikrishna Tandri ◽  
Darshan Dalal ◽  
Aditya Jain ◽  
Daniel P Judge ◽  
Theodore Abraham ◽  
...  

Background: In this report we present the prevalence and significance of a novel MRI abnormality that we identified in desmosomal mutation positive asymptomatic relatives of arrhythmogenic right ventricular dysplasia (ARVD) patients. Methods: MRI of 38 first-degree relatives was analyzed for abnormalities blinded to the mutation status. Focal crinkling of the right ventricle (accordion sign) was observed in 15/25 mutation positive vs. 0/13 mutation negative patients. The presence of this finding was sought in 207 patients evaluated for ARVD blinded to clinical history and was correlated with electrophysiologic findings. Results: Mean age of the population was 32±16, 61% had a family history of ARVD. Based on non-invasive testing the 207 patients were classified into 4 groups 1) 106 normal, 2) 57 Idiopathic ventricular tachycardia (VT), 3) 33 probable ARVD, 4) 11 definite ARVD. A family history of ARVD was present in 74%, 0%, 67% and 36% respectively. 43 patients had the “accordion sign” and the prevalence in the four groups was 10%, 5%, 70% and 100% respectively. The abnormality was seen in the sub-tricuspid region in 62%, outflow tract in 30% in both regions in 8%. After excluding group 4, 48% of patients with the accordion sign had T wave inversion’s beyond V1 compared with 7% of those without the sign (p<0.001). During electrophysiologic testing, reentrant VT could be induced in 44% (7/16) of patients with accordion sign compared to 2% (1/48) without the sign (p<0.01). Conclusion: Focal crinkling of the RV termed “Accordion sign” may represent an early manifestation of ARVD that correlates with mutation positive status and predicts inducibility of ventricular arrhythmias.


Circulation ◽  
2008 ◽  
Vol 118 (suppl_18) ◽  
Author(s):  
Rahul Jain ◽  
Darshan Dalal ◽  
Ariana Evenson ◽  
Rohit Jain ◽  
Amy Daly ◽  
...  

Background: The Task Force criteria for diagnosis of arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C) rely on depolarization and repolarization abnormalities detected on a surface ECG. Terminal activation delay (TAD) has recently been presented as specific and sensitive marker of ARVD/C. The purpose of this study was to evaluate the sensitivity and specificity of TAD in patients with ARVD/C. Methods : In a cohort of 74 patients with definite ARVD/C (51% male); age 36 ± 12 years) and 48 phenotypically normal non cardiac patients (35% male); age 35 ± 14 years) we studied the ECG criteria used to diagnose ARVD/C with specific a focus on TAD. The parameters analyzed were: T wave inversion (TWI) in ≥ V2, TWI ≥ V3, epsilon wave, QRS ≥ 110ms in V1 to V3, parietal block defined as QRSd V1–V6 or V2–V6 or V3–V6 >25 ms in the presence of QRS ≥ 100ms in at least 2 right precordial leads, TAD in V1-V3 and QRSd in V1+V2+V3/V4+V5+V6 ≥ 1.2. Sigma scan software was used for the ECG analysis. Results : In the ARVD/C group, the prevalence of TWI ≥ V2 was 82%, TWI ≥ V3 was 66%, epsilon wave: 1%, QRSd ≥ 110ms V1-V3: 39%, parietal block: 16%, TAD: 46 %, QRSd ratio ≥ 1.2: 19%. In the non cardiac group the prevalence of TWI ≥ V2 was 6 %, TWI ≥ V3: 6%, epsilon wave: 0%, QRSd ≥ 110ms V1–V3: 4%, parietal block: 2%, TAD: 12%, and QRSd ratio ≥ 1.2: 4% (Figure). Conclusion : The results of this study reveal that terminal activation delay has only moderate sensitivity and specificity for the diagnosis of ARVD/C. Our results also reveal that T wave inversion in the right precordial leads is a very sensitive marker of ARVD/C.


2020 ◽  
Vol 41 (Supplement_2) ◽  
Author(s):  
O Yasin ◽  
V Vaidya ◽  
J Tri ◽  
M Van Zyl ◽  
A Ladejobi ◽  
...  

Abstract Background His bundle pacing aims to mimic the activation pattern of normal conduction to maintain ventricular synchrony. However, selective His capture can be challenging, and the activation sequence during His pacing may not replicate normal conduction. Purpose Compare the right ventricular (RV) and left ventricular (LV) activation pattern in sinus rhythm and His bundle pacing. Methods Baseline LV and RV map was created in sinus rhythm using Rhythmia mapping system (Boston Scientific Corporation) in canine animal model. Medtronic 3830 lead was placed near the bundle of His under fluoroscopic, intracardiac echocardiogram, and electroanatomic guidance. Conduction system capture was confirmed by observing a QRS duration &lt;120ms and an isoelectric segment between pacing artifact and QRS on surface ECG. Repeat LV and RV activation map was obtained during His pacing. Average QRS, HV and pacing to V intervals were calculated with standard deviation. Results Mapping was performed successfully in four animals. At baseline, the average QRS duration was 44±2.6ms and HV interval was 32±4.2ms. Earliest site of myocardial activation was in the mid-septal LV region. The earliest RV myocardial activation was also at the septum closer to the apex, but later than the LV (Figure1A). With His pacing, the average QRS duration was 70±17.0ms and the average stim to V interval was 31±8.7ms. During His pacing, the earliest site of activation was in the RV septum, with an activation pattern from base to apex in both the RV and LV. Conclusion Unlike normal physiology, the activation pattern during conduction system pacing is from base to apex with earliest site in the RV. Funding Acknowledgement Type of funding source: Public hospital(s). Main funding source(s): Mayo Clinic


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