Abstract 13084: Epsilon Waves on Electrocardiograms in Patients With Arrhythmogenic Right Ventricular Cardiomyopathy: Relationship to Left Ventricular Invasion or Heart Compression Due to an Enlarged Right Ventricle

Circulation ◽  
2020 ◽  
Vol 142 (Suppl_3) ◽  
Author(s):  
Nobusada FUNABASHI ◽  
Yoshio Kobayashi
Keyword(s):  
Right Ventricular ◽  
Cardiac Ct ◽  
Task Force ◽  
Heart Diseases ◽  
Arrhythmogenic Right Ventricular Cardiomyopathy ◽  
Mitral Valve Regurgitation ◽  
Left Ventricular ◽  
Conduction Delay ◽  
Fatty Change ◽  
Valvular Diseases

Introduction: Epsilon waves on V1-3 leads are known as one of the specific lead ECG findings in patients with arrhythmogenic right ventricular (RV) cardiomyopathy (ARVC) that suggests the presence of RV conduction delay. Four dimensional (4D) cardiac CT visualizes ARVC characteristics, such as fat and fibrotic invasion in RV (RVM) and left ventricular (LV) myocardium (LVM), an enlarged RV, reduced RV motion, and bulging. Hypothesis: If epsilon waves are observed in V4-6 leads, this finding suggests the occurrence of LV invasion in ARVC. Another hypothesis exists in which extreme RV enlargement may compress the LV and cause clockwise rotation; an enlarged RV itself may cause epsilon waves in V4-6 leads. Methods: This is a retrospective analysis of 17 patients (11 males, 57 ± 17 years) with suspected ARVC who underwent cardiac CT. On 4D CT, nine met 2010 ARVC task force criteria. Results: All nine patients had epsilon waves on ECG. Five had fat and fibrotic invasion in the LVM but four did not. We divided nine into the following five groups by CT findings. On CT, a markedly enlarged RV compressed the LV to the left side and a fibro fatty change were observed exclusively in RVM (gp 1, N=1) or were observed in both RVM and LVM (gp 2, N=2). A moderately enlarged RV without compression of the LV to the left side and a fibro fatty change were observed exclusively in RVM (gp 3, N=3), or observed in both RVM and LVM (gp 4, N=2). The complications of severe mitral valve regurgitation, a markedly enlarged LV, and a fibro fatty change were observed in both RVM and LVM (gp 5, N=1). A patient in gp 1 showed epsilon waves in V1-6 leads. Patients in gp 2 showed epsilon waves in V1-6 (N=1), and V3-5 (N=1) leads, respectively. Patients in gp 3 showed epsilon waves in V1-4 (N=2), and V1-3 (N=1) leads, respectively. Patients in gp 4 showed epsilon waves in V1-3 (N=1), and V1, 2 (N=1) leads, respectively. A patient in gp 5 showed epsilon waves in V4-6 leads. Conclusions: The distribution of epsilon waves on ECG in patients with confirmed ARVC by cardiac CT was highly influenced by the degree of LV compression due to a markedly enlarged RV on CT rather than the presence of LV invasion. Also, structural change due to complicated heart diseases, such as valvular diseases, may also influence the distribution of epsilon waves in ARVC.

Abstract 13144: Epsilon Waves on Electrocardiograms in Pulmonary Hypertensive Patients With and Without Right Ventricular Hypertrophy or Fibrosis: Comparison With Arrhythmogenic Right Ventricular Cardiomyopathy

Circulation ◽  
2020 ◽  
Vol 142 (Suppl_3) ◽  
Author(s):  
Nobusada FUNABASHI ◽  
Yoshio Kobayashi
Keyword(s):  
Right Ventricular ◽  
Cardiac Ct ◽  
Task Force ◽  
Arrhythmogenic Right Ventricular Cardiomyopathy ◽  
Late Phase ◽  
Right Heart Catheterization ◽  
Conduction Delay ◽  
Heart Catheterization ◽  
T Wave ◽  
Pulmonary Arterial

Introduction: Epsilon waves on V1-3 leads are one of the specific ECG findings in patients with arrhythmogenic right ventricular (RV) cardiomyopathy (ARVC) that suggest the presence of RV conduction delay. Hypothesis: In patients with pulmonary hypertension (PH) due to RV pressure load, RV hypertrophy and fibrosis are frequently observed that may lead to RV conduction delay and epsilon waves. Methods: We retrospectively analyzed 43 PH patients (33 females, 55 ± 15 years, 31 chronic thromboembolic PH, seven idiopathic pulmonary arterial hypertension), with proven PH by right heart catheterization. On CT, RV fibrosis was defined as a contrast defect in the early phase and a conversely abnormal enhancement in the late phase. We also retrospectively analyzed 17 patients (11 males, 57 ± 17 years) with suspected ARVC who underwent cardiac CT. Of these, nine met 2010 ARVC task force criteria on cardiac CT. Results: All 9 ARVC patients (100%) had epsilon waves on ECG. In PH patients, 32 and 9 patients had RV hypertrophy and fibrosis, respectively. Of these, 4 patients had both. Among 32 PH patients with RV hypertrophy, 2 had complete right bundle branch block (RBBB) and 8 had incomplete RBBB. Of 22 PH patients with RV hypertrophy but without any RBBB, two (9%, P < 0.01, compared with ARVC) had waves with abnormal small upward spikes after the QRS wave. These were more marked in lead II and aVF leads than in V1 and 2 leads, and were not typical epsilon waves, suggesting RV conduction delay shown in ARVC. Fourteen patients had a negative T wave in V1-3 leads (5 had only a V1 lead, one had only V1 and 2 leads, four had only V1-3 leads, and four only had V1-4 leads, respectively). Of nine PH patients with RV hypertrophy, one had complete RBBB and one had incomplete RBBB. Of seven PH patients with RV fibrosis but without any RBBB, none (0%, P < 0.01, compared with ARVC) had epsilon waves in V1-3 leads; all seven had a negative T wave in V1-3 leads (three only had a V1 lead, one had only V1 and 2 leads, two had only V1-3 leads, and one had V1-4 leads). Conclusions: In PH patients with an organized RV myocardial change, such as RV hypertrophy and/or fibrosis, the frequency of epsilon waves was significantly less than those in ARVC. Epsilon waves seem not only to be caused by an organized RV myocardium but also by other factors.

Abstract 15314: Left Ventricular Dysfunction in Children and Adolescents With Arrhythmogenic Right Ventricular Cardiomyopathy

Circulation ◽  
2015 ◽  
Vol 132 (suppl_3) ◽  
Author(s):  
Paweena Chungsomprasong ◽  
Robert Hamilton ◽  
Wietske Luining ◽  
Shi-Joon Yoo ◽  
Meena Fatah ◽  
...  
Keyword(s):  
Right Ventricular ◽  
Task Force ◽  
Myocardial Dysfunction ◽  
Arrhythmogenic Right Ventricular Cardiomyopathy ◽  
Myocardial Strain ◽  
Young Patients ◽  
Left Ventricular ◽  
Lv Function ◽  
Ventricular Cardiomyopathy ◽  
End Diastolic Volume

Background: Involvement of the left ventricle (LV) is increasingly recognized in adults with arrhythmogenic right ventricular cardiomyopathy (ARVC) but it is unclear whether LV function is compromised in children with this condition. The aim of this study was examine myocardial contractility in pediatric patients with suspected ARVC. Methods: For this retrospective study, patients with a work-up for ARVC were classified into ‘no’, ‘possible’, ‘borderline’ or ‘definite’ ARVC according to the revised Task Force Criteria (rTFC). Ventricular size and function as well as LV myocardial strain and torsion were measured by cardiac magnetic resonance (CMR). Results: A total of 142 patients were enrolled, of whom 58 (41%) had no, 32 (23%) possible, 29 (20%) borderline and 23 (16%) definite ARVC. The groups were similar in age at CMR. With higher rTFC score, z scores (Z) of right ventricular (RV) ejection fraction (EF) were lower (p<0.001) while z-RV end diastolic volume (EDV) and z-LV EDV were larger (p=0.002 and 0.013, respectively). LV EF did not differ between rTFC categories. Global circumferential strain (GCS) of the LV was lower in patients in higher rTFC categories (p=0.018). Z-LVEDV correlated with z-RVEDV (r2 = 0.69, p<0.001) and z- LVEF correlated with z-RVEF (r2 = 0.55, p <0.001). Z-LVEF and z-RVEF correlated with LV GCS (r2 = 0.48, p<0.001 and r2 = 0.46, p<0.001, respectively) and torsion (r2 = 0.21, p=0.032 for both). Forty-two patients had a follow-up CMR, after a median interval of 2.6 years (0.4- 8.4). The rate of deterioration of LV or RV EF or EDV did not differ between rTFC categories. A more rapid increase of z-RVEDV was associated with a faster decline in z-RVEF (r2 = -0.383, p=0.004) and z-LVEF (r2 = -0.45, p=0.001). A decline of z-LVEF over time correlated with that of z-RVEF (r2 = 0.60, p<0.001) and z-LVEDV increase correlated with z-RVEDV increase (r2 = 0.84, p<0.001). Conclusion: LV myocardial dysfunction is present in young patients with suspected or confirmed ARVC. Quantification of myocardial mechanics with CMR may be a useful tool to detect early LV involvement in ARVC. Progressive LV dysfunction and enlargement appear to parallel those of the RV.

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.

scholarly journals Assessment of right ventricular sympathetic dysfunction in patients with arrhythmogenic right ventricular cardiomyopathy: An 123I-metaiodobenzylguanidine SPECT/CT study

2018 ◽  
Vol 27 (6) ◽  
pp. 2402-2409 ◽  
Author(s):  
Andrei Todica ◽  
Johannes Siebermair ◽  
Julia Schiller ◽  
Mathias J. Zacherl ◽  
Wolfgang P. Fendler ◽  
...  
Keyword(s):  
Right Ventricular ◽  
Hybrid Imaging ◽  
State Of The Art ◽  
Task Force ◽  
Arrhythmogenic Right Ventricular Cardiomyopathy ◽  
Sympathetic Innervation ◽  
Left Ventricular ◽  
Sympathetic Dysfunction ◽  
Novel Approach ◽  
Significant Difference

Abstract Purpose The purpose of the study was to evaluate a novel approach for the quantification of right ventricular sympathetic dysfunction in patients diagnosed with ARVC/D through state-of-the-art functional SPECT/CT hybrid imaging. Methods Sympathetic innervation of the heart was assessed using 123I-MIBG-SPECT/CT in 17 patients diagnosed with ARVC according to the modified task force criteria, and in 10 patients diagnosed with idiopathic ventricular fibrillation (IVF). The 123I-MIBG-uptake in the left (LV) and right ventricle (RV) was evaluated separately based on anatomic information derived from the CT scan, and compared to the uptake in the mediastinum (M). Results There was a significant difference in the LV/M ratio between the ARVC/D and the IVF groups (3.2 ± 0.5 vs. 3.9 ± 0.8, P = 0.014), with a cut-off value of 3.41 (77% sensitivity, 80% specificity, AUC 0.78). There was a highly significant difference in the mean RV/M ratios between both groups (1.6 ± 0.3 vs. 2.0 ± 0.2, P = 0.001), with optimal cut-off for discrimination at 1.86 (88% sensitivity, 90% specificity, AUC 0.93). Conclusion Employing state-of-the-art functional SPECT/CT hybrid imaging, we could reliably assess and quantify right and left ventricular sympathetic innervation. The RV/M ratio was significantly lower in patients diagnosed with ARVC/D and provided sensitive and specific discrimination between patients with ARVC/D and IVF patients.

scholarly journals The utility of the 1994 versus the revised 2010 arrhythmogenic right ventricular cardiomyopathy (ARVC) task force diagnostic criteria for identifying mutation-positive probands with ARVC

2020 ◽  
Vol 41 (Supplement_2) ◽  
Author(s):  
K Lukhna ◽  
S Kraus ◽  
N Ntusi
Keyword(s):  
Right Ventricular ◽  
Task Force ◽  
Diagnostic Yield ◽  
Arrhythmogenic Right Ventricular Cardiomyopathy ◽  
Left Ventricular ◽  
Ventricular Cardiomyopathy ◽  
Significant Difference ◽  
Diagnostic Panel ◽  
The Mean ◽  
The Right

Abstract Background Arrhythmogenic right ventricular cardiomyopathy (ARVC) is an inherited cardiac disorder characterised by structural and functional abnormalities of the right ventricle with or without left ventricular involvement. In 1994, Task Force criteria (TFC) were proposed for the diagnosis of ARVC and were found to be highly specific but lacked sensitivity. In 2010, revised TFC were proposed to increase sensitivity and facilitate diagnosis in those with subtle phenotypes. Purpose To compare the utility of the 1994 vs the 2010 TFC for the diagnosis of mutation-positive probands with ARVC in the IMHOTEP (The African Cardiomyopathy and Myocarditis Registry Program) study. Method 162 participants with the suspicion of ARVC were referred between May 2003 and May 2018. After the exclusion of 12 participants lacking sufficient clinical data, 150 cases were reviewed and classified using both 1994 and 2010 TFC by a diagnostic panel in an hospital. Results 82 participants were found to have an alternative diagnosis or insufficient criteria and were excluded. 68 participants were diagnosed with ARVC by the diagnostic panel and included; 14/68 participants with ARVC were found to be mutation-positive. Mutation-positive probands presented at a significantly younger age compared to the mutation-negative group (29±14 years vs 39±13 years, p=0.009), suggesting an earlier onset of ARVC. Common reasons for presentation in the mutation-positive cohort included palpitations (79%) and presyncope (64%), with twice the number of participants presenting with sustained ventricular tachycardia compared to mutation-negative participants (79% vs 47%, p=0.036). The diagnostic yield of the 2010 vs 1994 TFC in participants with ARVC (n=68) revealed more participants with a definite diagnosis (77% vs 69%, p=0.267). A 67% change in diagnosis from 1994 borderline to 2010 definite was observed. Mutation-positive participants had a higher yield for definite ARVC compared to mutation-negative participants (100% vs 86%). When comparing the mean number of task force (TF) major and minor criteria according to mutation status, we found a significant difference in the mean number of 2010 TF major criteria between mutation-positive and mutation-negative groups, even with the exclusion of gene mutation as a criterion (2.50±0.86 vs 1.74±0.85, p=0.005). We assessed each diagnostic modality's contribution to the 2010 TF major criteria in mutation-positive definite participants and found cardiac magnetic resonance contribution statistically significant, p=0.021. Conclusion Mutation-positive ARVC probands were found to be younger, more likely to present with sustained VT, and fulfilled a significantly higher number of 2010 TF major criteria than mutation-negative probands. The evolution in classification between the 2010 and 1994 TFC suggests that reclassifying participants recruited in traditional ARVC registries according to updated criteria is worthwhile. Funding Acknowledgement Type of funding source: None

scholarly journals Role of Provocable Brugada ECG Pattern in The Correct Risk Stratification for Major Arrhythmic Events

2021 ◽  
Vol 10 (5) ◽  
pp. 1025
Author(s):  
Nicolò Martini ◽  
Martina Testolina ◽  
Gian Luca Toffanin ◽  
Rocco Arancio ◽  
Luca De Mattia ◽  
...  
Keyword(s):  
Sudden Death ◽  
Risk Stratification ◽  
Right Ventricular ◽  
Arrhythmogenic Right Ventricular Cardiomyopathy ◽  
Right Ventricular Outflow Tract ◽  
Ventricular Outflow Tract ◽  
Conduction Delay ◽  
St Segment Elevation ◽  
Additional Information ◽  
The Right

The so-called Brugada syndrome (BS), first called precordial early repolarization syndrome (PERS), is characterized by the association of a fascinating electrocardiographic pattern, namely an aspect resembling right bundle branch block with a coved and sometime upsloping ST segment elevation in the precordial leads, and major ventricular arrhythmic events that could rarely lead to sudden death. Its electrogenesis has been related to a conduction delay mostly, but not only, located on the right ventricular outflow tract (RVOT), probably due to a progressive fibrosis of the conduction system. Many tests have been proposed to identify people at risk of sudden death and, among all, ajmaline challenge, thanks to its ability to enhance latent conduction defects, became so popular, even if its role is still controversial as it is neither specific nor sensitive enough to guide further invasive investigations and managements. Interestingly, a type 1 pattern has also been induced in many other cardiac diseases or systemic diseases with a cardiac involvement, such as long QT syndrome (LQTS), arrhythmogenic right ventricular cardiomyopathy (ARVC), hypertrophic cardiomyopathy (HCM) and myotonic dystrophy, without any clear arrhythmic risk profile. Evidence-based studies clearly showed that a positive ajmaline test does not provide any additional information on the risk stratification for major ventricular arrhythmic events on asymptomatic individuals with a non-diagnostic Brugada ECG pattern.

Left Side Right Ventricular Cardiomyopathy

2002 ◽  
Vol 42 (4) ◽  
pp. 313-317 ◽  
Author(s):  
M Michalodimitrakis ◽  
A Papadomanolakis ◽  
J Stiakakis ◽  
K Kanaki
Keyword(s):  
Right Ventricular ◽  
Arrhythmogenic Right Ventricular Cardiomyopathy ◽  
Left Ventricular ◽  
Muscle Disease ◽  
Pathologic Examination ◽  
Arrhythmogenic Cardiomyopathy ◽  
Ventricular Cardiomyopathy ◽  
First Case ◽  
Fibrofatty Tissue ◽  
Myocardial Muscle

Arrhythmogenic right ventricular cardiomyopathy or dysplasia, a heart muscle disease of unknown cause, is anatomically characterized by variable replacement of myocardial muscle with adipose or fibroadipose tissue. It is usually considered a selective disorder whereas concomitant left ventricular involvement has been noted in a few cases. Two cases of the disease with evidence of extensive left ventricular involvement at pathologic examination are described. Hearts from two patients who died suddenly showed extensive biventricular infiltration by fibrofatty tissue in the first case and exclusively in the wall of the left ventricle the localization of the fatty and fibrotic lesions. These findings might suggest that the various localizations of the fibroadipose tissue are rather different expressions of the same disease and it is preferable to be termed ‘arrhythmogenic cardiomyopathy’ as other studies also indicate.

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