Abstract 1966: Comparison of Right Ventricular Strain and Strain Rate in Patients with Arrhythmogenic Right Ventricular Cardiomyopathy, Right Ventricular Outflow Tract Tachycardia, and Matched Controls

Circulation ◽  
2007 ◽  
Vol 116 (suppl_16) ◽  
Author(s):  
Omid Salehian ◽  
Andrew P Klug ◽  
Jeffrey Healey ◽  
Ian G Burwash
Keyword(s):  
Right Ventricular ◽  
Strain Rate ◽  
Task Force ◽  
Arrhythmogenic Right Ventricular Cardiomyopathy ◽  
Myocardial Strain ◽  
Strain Rates ◽  
Systolic Strain ◽  
Ventricular Cardiomyopathy ◽  
Strain And Strain Rate ◽  
Fatty Replacement

Background: Arrhythmogenic right ventricular cardiomyopathy (ARVC) is characterized by fibro-fatty replacement of RV myocardium leading to ventricular tachycardia and sudden death in otherwise healthy individuals. Diagnosis is challenging as there are no diagnostic tests with adequate accuracy. Current diagnosis of ARVC is made by the task force criteria. One of the main diagnostic dilemmas continues to be distinguishing ARVC from a relatively benign entity of right ventricular out flow tract tachycardia (RVOT VT). Assessment of myocardial strain and strain rate has been used to study patients with ischemic heart disease and has shown utility in identifying subtle myocardial dysfunction not detected by routine wall motion assessment. The goal of this study was to compare myocardial strain and strain rates in patients with ARVC, RVOT VT and matched controls. Methods: We prospectively enrolled 12 patients with ARVC (based on task force criteria), 10 with RVOT VT, and 22 age and gender matched controls. Echo studies (including tissue Doppler derived myocardial strain echocardiography) were performed with commercially available systems (GE, Vivid 7). Peak systolic RV myocardial strain and strain rate were measured at base, mid, and apical RV free wall and compared. Results: ARVC patients had significantly lower peak systolic RV strain compared to both RVOT VT patients and matched controls (Table ). There was also significantly lower peak systolic strain rate at the apical RV myocardium in patients with ARVC compared to both those with RVOT VT (p=0.02) and controls (p=0.01). Conclusions: Patients with ARVC have abnormally low RV systolic strain and strain rates when compared to matched controls as well as those with RVOT VT. This modality can be routinely used in assessing patients suspected of ARVC as it might assist in earlier diagnosis as well as help in distinguishing patients with ARVC from those with RVOT VT. P<0.05 compared to controls, † P<0.05 compared to RVOT VT group

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 Arrhythmogenic Right Ventricular Cardiomyopathy

2021 ◽  
Author(s):  
Sukanya Ghosh
Keyword(s):  
Right Ventricular ◽  
Task Force ◽  
Arrhythmogenic Right Ventricular Cardiomyopathy ◽  
Disease Process ◽  
Ventricular Cardiomyopathy ◽  
Molecular Genetic Study ◽  
Icd Implantation ◽  
Fatty Replacement ◽  
Genes Encoding ◽  
Long Term Prognosis

Arrythmogenic right ventricular cardiomyopathy (ARVC) is a genetic form of cardiomyopathy causing fibro-fatty replacement of the myocardium. Although usually transmission is autosomal dominant, 12 genes encoding cardiac desmosomes have been found to be closely linked to this disease process shifting the congenital disease theory to a genetic one. The categorisation of ARVC as a myocyte adhesion disorder was first suggested by a molecular genetic study involving patients with Naxos disease. Misnomeric to only affect the right ventricle, ARVC also affects the left ventricle - culminating into biventricular failure as a long term prognosis. Epidemiology is well established with a male to female preponderance. It is currently the second most common cause of sudden cardiac death (SCD) in population < 35 yrs. Pathological basis of the varied clinical presentation is explained at the molecular level with myocardial atrophy, fibro-fatty replacement and chamber dilatation. Diagnosing the condition by ruling out the pitfall differentials is an enormous challenge due to the broad phenotypic spectrum including syncope on one end and SCD on the other. Task Force Criteria combines electrocardiography (ECG), echocardiography (ECHO), cardiac magnetic resonance imaging (CMRI), myocardial biopsy for diagnosis; early detection, family screening and risk stratification being the cornerstones. Therapeutic options although limited due to the progressive nature of the disease is based on preventing life threatening arrhythmias encompassing primary and secondary prevention - Implantable cardioverter -defibrillator (ICD) implantation, radiofrequency ablation and heart transplantation are the main ones.

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.

scholarly journals Regional right ventricular function in rats: a novel magnetic resonance imaging method for measurement of right ventricular strain

2020 ◽  
Vol 318 (1) ◽  
pp. H143-H153
Author(s):  
Emil K. S. Espe ◽  
Jan M. Aronsen ◽  
Einar S. Nordén ◽  
Lili Zhang ◽  
Ivar Sjaastad
Keyword(s):  
Molecular Markers ◽  
Right Ventricular ◽  
Strain Rate ◽  
Longitudinal Strain ◽  
Myocardial Strain ◽  
Area Under The Curve ◽  
Strain Rates ◽  
Pulmonary Congestion ◽  
Right Ventricular Strain ◽  
Rv Function

The function of the right ventricle (RV) is linked to clinical outcome in many cardiovascular diseases, but its role in experimental heart failure remains largely unexplored due to difficulties in measuring RV function in vivo. We aimed to advance RV imaging by establishing phase-contrast MRI (PC-MRI) as a robust method for measuring RV function in rodents. A total of 46 Wistar-Hannover rats with left ventricular (LV) myocardial infarction and 10 control rats (sham) were examined 6 wk after surgery. Using a 9.4-T preclinical MRI system, we utilized PC-MRI to measure strain/strain rate in the RV free wall under isoflurane anesthesia. Cine MRI was used to measure RV volumes. LV end-diastolic pressure (LVEDP) was measured and used to identify pulmonary congestion. The infarct rats were divided into two groups: those with signs of pulmonary congestion (PC), with LVEDP ≥ 15 mmHg ( n = 26) and those without signs of pulmonary congestion (NPC), with LVEDP < 15 mmHg ( n = 20). The NPC rats exhibited preserved RV strains/strain rates, whereas the PC rats exhibited reduced strains/strain rates (26–48% lower than sham). Of the strain parameters, longitudinal strain and strain rate exhibited the highest correlations to LVEDP and lung weight (rho = 0.65–0.72, P < 0.001). Basal longitudinal strain was most closely associated with signs of pulmonary congestion and indexes of RV remodeling. Longitudinal RV strain had higher area under the curve than ejection fraction for detecting subtle RV dysfunction (area under the curve = 0.85 vs. 0.67). In conclusion, we show for the first time that global and regional RV myocardial strain can be measured robustly in rodents. Reduced RV strain was closely associated with indexes of pulmonary congestion and molecular markers of RV remodeling. NEW & NOTEWORTHY Global and regional right ventricular myocardial strain can be measured with high reproducibility and low interobserver variability in rodents using tissue phase mapping MRI. Reduced right ventricular strain was associated with indexes of pulmonary congestion and molecular markers of right ventricular remodeling. Regional strain in the basal myocardium was considerably higher than in the apical myocardium.

scholarly journals An International Evidence Based Reappraisal of Genes Associated with Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC) using the ClinGen Framework

2021 ◽  
Author(s):  
Cynthia A. James ◽  
Jan D.H. Jongbloed ◽  
Ray E. Hershberger ◽  
Ana Morales ◽  
Daniel P. Judge ◽  
...  
Keyword(s):  
Right Ventricular ◽  
Ventricular Arrhythmias ◽  
Task Force ◽  
Arrhythmogenic Right Ventricular Cardiomyopathy ◽  
Model Systems ◽  
Polymorphic Ventricular Tachycardia ◽  
Inherited Disease ◽  
Ventricular Cardiomyopathy ◽  
Moderate Evidence ◽  
Major Criterion

Background - Arrhythmogenic right ventricular cardiomyopathy (ARVC) is an inherited disease characterized by ventricular arrhythmias and progressive ventricular dysfunction. Genetic testing is recommended and a pathogenic variant in an ARVC-associated gene is a major criterion for diagnosis according to the 2010 Task Force Criteria (TFC). As incorrect attribution of a gene to ARVC can contribute to misdiagnosis, we assembled an international multidisciplinary ARVC ClinGen Gene Curation Expert Panel to reappraise all reported ARVC genes. Methods - Following a comprehensive literature search, six two-member teams conducted blinded independent curation of reported ARVC genes using the semi-quantitative ClinGen framework. Results - Of 26 reported ARVC genes, only six ( PKP2 , DSP , DSG2 , DSC2 , JUP , TMEM43 ) had strong evidence and were classified as definitive for ARVC causation. There was moderate evidence for two genes, DES and PLN . The remaining 18 genes had limited or no evidence. RYR2 was refuted as an ARVC gene since clinical data and model systems exhibited a catecholaminergic polymorphic ventricular tachycardia (CPVT) phenotype. In ClinVar, only 5 pathogenic / likely pathogenic (P/LP) variants (1.1%) in limited evidence genes had been reported in ARVC cases in contrast to 450 desmosome gene variants (97.4%). Conclusions - Using the ClinGen approach to gene-disease curation, only eight genes, ( PKP2 , DSP , DSG2 , DSC2 , JUP , TMEM43 , PLN , DES ) had definitive or moderate evidence for ARVC and these genes accounted for nearly all P/LP ARVC variants in ClinVar. Therefore, only P/LP variants in these eight genes should yield a major criterion for ARVC diagnosis. P/LP variants identified in other genes in a patient should prompt further phenotyping as variants in many of these genes are associated with other cardiovascular conditions.

scholarly journals Role of ventricular tachycardia ablation in arrhythmogenic right ventricular cardiomyopathy

2017 ◽  
Vol 7 (1) ◽  
Author(s):  
Alberto Cipriani ◽  
Riccardo Bariani ◽  
Manuel De Lazzari ◽  
Federico Migliore ◽  
Carlo Angheben ◽  
...  
Keyword(s):  
Ventricular Tachycardia ◽  
Catheter Ablation ◽  
Right Ventricular ◽  
Arrhythmogenic Right Ventricular Cardiomyopathy ◽  
Ventricular Cardiomyopathy ◽  
Ventricular Tachycardia Ablation ◽  
Fatty Replacement ◽  
Technical Issues ◽  
Long Term Prognosis

Arrhythmogenic right ventricular cardiomyopathy (ARVC) is characterized by progressive fibro-fatty replacement of the myocardium that represents the substrate for recurrent sustained ventricular tachycardia (VT). These arrhythmias characterize the clinical course of a sizeable proportion of patients and have significant implications for their quality of life and long-term prognosis. Antiarrhythmic drugs are often poorly tolerated and usually provide incomplete control of arrhythmia relapses. Catheter ablation is a potentially effective strategy to treat frequent VT episodes and ICD shocks in ARVC patients. The aims of this review are to discuss the electrophysiological and electroanatomic substrates of ventricular tachycardia in patients with ARVC and to analyze the role of catheter ablation in their management with particular reference to selection of patients, technical issues, potential complications and outcomes.

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