Clinical Characteristics of Patients with a Right Ventricular Thrombus in Arrhythmogenic Right Ventricular Cardiomyopathy

2019 ◽  
Vol 119 (08) ◽  
pp. 1373-1378 ◽  
Author(s):  
Deniz Akdis ◽  
Kai Chen ◽  
Ardan M. Saguner ◽  
Simon F. Stämpfli ◽  
Xiao Chen ◽  
...  
Keyword(s):  
Ejection Fraction ◽  
Right Ventricular ◽  
Clinical Characteristics ◽  
Task Force ◽  
Thrombus Formation ◽  
Rare Complication ◽  
Arrhythmogenic Right Ventricular Cardiomyopathy ◽  
The Mean ◽  
The University ◽  
Rv Function

Background Thrombus formation within the left ventricle (LV) is a well-known clinical entity and is often associated with underlying myocardial disease, whereas right ventricular (RV) thrombi are rarely observed. This study aimed to investigate the clinical characteristics of patients with arrhythmogenic RV cardiomyopathy (ARVC) who developed an RV thrombus. Methods and Results This study included patients with an RV thrombus from the ARVC databases of the University Heart Center in Zurich, Switzerland, and the Fuwai Hospital in Beijing, China. In total, there were 13 ARVC patients who had an RV thrombus detected. The mean age was 33 ± 15 (range: 11–51) years. Eight patients (62%) were male. The mean Task Force score was 6 ± 1. Nine of these patients (69%) had an RV thrombus only whereas four patients had biventricular thrombi. All 13 ARVC patients had a severely impaired RV function (RV fractional area change 16 ± 9% and RV ejection fraction 15 ± 4%); LV ejection fraction (LVEF) was 40 ± 15%. ARVC patients with an additional LV thrombus had a lower LVEF than the others (24 ± 11 vs. 47 ± 11, p = 0.02). Under therapeutic anticoagulation, complete thrombus resolution was observed in 9/13 patients (69%). Conclusion RV thrombus formation is a potential complication of ARVC with impaired RV function. In patients with biventricular involvement, thrombi may also occur within the LV. Anticoagulation is generally effective to dissolve RV thrombi. This study highlights the need for awareness during cardiac imaging to detect this rare complication of ARVC.

P310Differentiation between arrhythmogenic right ventricular cardiomyopathy and athlete's heart using cardiac magnetic resonance based derived parameters and strain analysis

2019 ◽  
Vol 40 (Supplement_1) ◽  
Author(s):  
C Czimbalmos ◽  
I Csecs ◽  
Z Dohy ◽  
A Toth ◽  
F I Suhai ◽  
...  
Keyword(s):  
Ejection Fraction ◽  
Diagnostic Accuracy ◽  
Right Ventricular ◽  
Task Force ◽  
Arrhythmogenic Right Ventricular Cardiomyopathy ◽  
Strain Analysis ◽  
Volume Index ◽  
Ventricular Cardiomyopathy ◽  
End Diastolic Volume ◽  
Regional Strain

Abstract Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a major cause of sudden cardiac death in young athletes. However diagnosing ARVC in highly trained athletes may be complicated because of overlapping features such as elevated right ventricular (RV) end-diastolic volume index or T-wave inversion in precordial leads. The revised Task Force criteria contain no specific cut-off value for professional athletes. Additional CMR parameters and CMR deformation imaging may have an added diagnostic value in this special patient population. Our goal was to determine novel CMR parameters which can help to distinguish between ARVC and athlete's heart. CMR examination of ARVC patients with definite diagnosis based on the revised Task Force criteria (n=34; 41±13 y, 22 male) and healthy professional athletes (members of the Hungarian national water polo, canoing or rowing team performing minimum of 15 hours of training per week, n=34, 32±6 y, 22 male) was performed. We evaluated left and right ventricular end-systolic, end-diastolic (EDVi) and stroke volume index, ejection fraction (EF) and mass. We established derived parameters such as ejection fraction ratio (LVEF/RVEF) and end-diastolic volume ratio (LVEDV/RVEDV). Global and regional strain analysis for the right ventricle was performed using feature tracking technique. Area under the ROC curves (AUC) of conventional and derived CMR parameters and CMR based strain values were analysed. There was no significant difference between RVEDVi of ARVC patients and athletes (124±17 vs 142±47), RVEF was lower in ARVC patients compared to athletes (56±5 vs 41±14%; p<0.001). Significant differences were found between athletes and ARVC patients in LVEDV/RVEDV (0.96±0.08 vs 0.82±0.23), LVEF/RVEF (1.04±0.06 vs 1.41±0.56), global circumferential strain (−34.8±5.9 vs −25.2±12.2) and regional strain values such as midventricular RV strain (−31.5±10.2 vs −20.0±13.4) or midventricular RV strain rate (−1.37±0.56 vs −1.04±0.68), respectively. RVEF and LVEF/RVEF showed excellent (AUC of 0.9–1.0), RV global strain and RV midventricular strain values showed good diagnostic accuracy (AUC of 0.8–0.9), while RVEDVi showed poor diagnostic accuracy (AUC of 0.59). Consequently, in highly trained healthy athletes RVEDVi is in the range of major Task Force criteria, while CMR based derived parameters such as LVEDV/RVEDV or LVEF/RVEF and both global and regional RV strain parameters can be useful parameters in the differential diagnosis. Acknowledgement/Funding National Research, Development and Innovation Office (NKFIH) of Hungary (K 120277), ÚNKP-18-3-IV New National Excellence Program of Human Capacities.

scholarly journals Long term CMR follow up of patients with right ventricular abnormality and clinically suspected arrhythmogenic right ventricular cardiomyopathy (ARVC)

2019 ◽  
Vol 21 (1) ◽  
Author(s):  
Giuseppe Femia ◽  
Christopher Semsarian ◽  
Mark McGuire ◽  
Raymond W. Sy ◽  
Rajesh Puranik
Keyword(s):  
Family History ◽  
Ejection Fraction ◽  
Right Ventricular ◽  
Task Force ◽  
Arrhythmogenic Right Ventricular Cardiomyopathy ◽  
Premature Death ◽  
Ventricular Cardiomyopathy ◽  
Degree Relative ◽  
History Of

Abstract Background The Task Force Criteria (TFC) for arrhythmogenic right ventricular cardiomyopathy (ARVC) was updated in 2010 to improve specificity. There was concern however that the revised cardiovascular magnetic resonance (CMR) criteria was too restrictive and not sensitive enough to detect early forms of the condition. We previously described patients with clinically suspected ARVC who satisfied criteria from non-imaging TFC categories and fulfilled parameters from the original but not the revised CMR criteria; as a result, these patients were not confirmed as definite ARVC but may represent an early phenotype. Methods Patients scanned between 2008 and 2015 who had either right ventricular (RV) dilatation or regional dyskinesia satisfying at least minor imaging parameters from the original criteria and without contra-indication underwent serial CMR scanning using a 1.5 T scanner. The aims were to assess the risk of progressive RV abnormalities, evaluate the accuracy of the revised CMR criteria and the need for guideline directed CMR surveillance in at-risk individuals. Results Overall, 48 patients were re-scanned; 24 had a first-degree relative diagnosed with ARVC using the revised TFC or a first-degree relative with premature sudden death from suspected ARVC and 24 patients had either left bundle branch morphology ventricular tachycardia or > 500 ventricular extra-systoles in 24-h. Mean follow up was 69+/− 25 months. The indexed RV end-diastolic, end-systolic volumes and ejection fraction were calculated for both scans. There was significant reduction in RV volumes and improvement in RV ejection fraction (EF) irrespective of changes to body surface area; − 11.7+/− 15.2 mls/m2, − 6.4+/− 10.5 mls/m2 and + 3.3 +/− 7.9% (p = 0.01, 0.01 and 0.04). Applying the RV parameters to the revised CMR criteria, two patients from the family history group (one with confirmed ARVC and one with a premature death) had progressive RV abnormalities satisfying major criteria. The remaining patients (n = 46) did not satisfy the criteria and either had normal RV parameters with regression of structural abnormalities (27,56.3%) or stable abnormalities (19,43.7%). Conclusion The revised CMR criteria represents a robust tool in the evaluation of patients with clinical suspicion of ARVC, especially for those with ventricular arrhythmias without a family history for ARVC. For patients with RV abnormalities that do not fulfill the revised criteria but have a family history of ARVC or an ARVC associated gene mutation, a surveillance CMR scan should be considered as part of the clinical follow up protocol.

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 Right ventricular ejection fraction as predictor of outcome in acute heart failure using RV ellipsoid model: A retrospective analysis of a prospective cross-sectional study

2021 ◽  
Vol 10 ◽  
pp. 204800402110027
Author(s):  
Eshan Ashcroft ◽  
Otar Lazariashvili ◽  
Jonathan Belsey ◽  
Max Berrill ◽  
Pankaj Sharma ◽  
...  
Keyword(s):  
Heart Failure ◽  
Ejection Fraction ◽  
Right Ventricular ◽  
Retrospective Analysis ◽  
Cross Sectional Study ◽  
Volume Estimation ◽  
Sectional Study ◽  
Cross Sectional ◽  
Ventricular Ejection Fraction ◽  
Rv Function

Objectives The right ventricular (RV) function is an important prognostic factor in acute and chronic heart failure (HF). Echocardiography is an essential imaging modality with established parameters for RV function which are useful and easy to perform. However, these fail to reflect global RV volumes due to reliability on one acoustic window. It is therefore attractive to calculate RV volumes and ejection fraction (RVEF/E) using an ellipsoid geometric model which has been validated against MRI in healthy adults but not in the HF patients. Design This is a retrospective analysis of a prospective cross-sectional study enrolling 418 consecutive patients with symptoms of HF according to a predefined study protocol. All patients underwent echocardiographic assessment of RV function using Tricuspid Annular Plane Systolic Excursion (TAPSE) and RV fractional area change (RVFAC) and RVEF/E. Setting Single centre study with multiple locations for acute in-patients including high dependency units. Participants Patients with acute or exacerbation of chronic HF older than 18 y.o. Main outcome measures Ability of RVEF/E to predict patient outcomes compared with two established parameters of RV function over two-year follow-up period. Primary outcome measure was all-cause mortality. Results RVEF/E is equal to TAPSE & RVFAC in predicting outcome (p ≤ 0.01 vs p ≤ 0.01) and provides additional benefit of RV volume estimation based on standard 2D echo measurements. Conclusions In this study we have shown that RVEF/E derived from ellipsoid model is not inferior to well established measures of RV function as a prognostic indicator of outcome in the acute HF.

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.

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.

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