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.

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.

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

Impact of revised Task Force Criteria: distinguishing the athlete’s heart from ARVC/D using cardiac magnetic resonance imaging

2011 ◽  
Vol 19 (4) ◽  
pp. 885-891 ◽  
Author(s):  
Tim Luijkx ◽  
Birgitta K Velthuis ◽  
Niek HJ Prakken ◽  
Moniek GPJ Cox ◽  
Michiel L Bots ◽  
...  
Keyword(s):  
Cardiac Magnetic Resonance ◽  
Magnetic Resonance ◽  
Right Ventricular ◽  
Task Force ◽  
Arrhythmogenic Right Ventricular Cardiomyopathy ◽  
Good Alternative ◽  
Ventricular Cardiomyopathy ◽  
Volume Increase ◽  
End Diastolic Volume ◽  
Cardiac Adaptation

Background: Cardiac magnetic resonance (CMR) evaluation of athletes for arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D) is complicated by overlapping features such as right ventricular (RV) volume increase. The revised ARVC/D diagnostic Task Force Criteria (TFC) incorporate cut-off values for RV ejection fraction (EF) and RV end-diastolic volume (EDV) on CMR. Design: To distinguish ARVC/D patients from athletes we compared CMR ventricular volumes, function, TFC cut-off values, and LV/RV ratios since athletes show proportionate, and ARVC/D patients disproportionate, changes in LV and RV. Methods: Quantitative CMR parameters of 33 ARVC/D patients (64% male, mean age 45.4 years, diagnosed by revised TFC), 66 healthy athletes and 66 healthy non-athletes (sex and age matched) were compared using revised TFC and new cut-off values representing LV/RV balance. Results and conclusions: Absolute values for ARVC/D patients/athletes/non-athletes were: in males, RV EDV 149/133/106 ml/m2, ratio EDV LV/RV 0.70/0.91/0.93, RV EF 34/52/54%, LV EF 48/57/58%, ratio EF LV/RV 1.49/1.10/1.09; and in females, RV EDV 115/115/91 ml/m2, ratio EDV LV/RV 0.86/0.94/0.97, RV EF 43/54/58%, LV EF 52/57/61%, ratio EF LV/RV 1.23/1.08/1.04 ( p-values < 0.05). Areas under the ROC-curve are 0.68 (RV EDV index), 0.84 (LV/RV EDV ratio) and 0.93 (RV EF), demonstrating significantly ( p < 0.001) better performance of RV EF and LV/RV EDV ratio. If a wall motion abnormality is present (observed in 30 ARVC/D patients and not in healthy subjects), RV EF can help distinguish ARVC/D from physiological cardiac adaptation in athletes on CMR whereas RV EDV index cannot. A good alternative in athletes is the LV/RV EDV ratio, representing normal proportionate adaptation of both ventricles.

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

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.

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.

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