scholarly journals Echocardiographic assessment of arrhythmogenic right ventricular cardiomyopathy

Heart ◽  
2001 ◽  
Vol 86 (1) ◽  
pp. 31-38
Author(s):  
L Lindström ◽  
U M Wilkenshoff ◽  
H Larsson ◽  
B Wranne
Keyword(s):  
Right Ventricular ◽  
Wall Motion ◽  
Arrhythmogenic Right Ventricular Cardiomyopathy ◽  
Velocity Ratio ◽  
Tissue Doppler ◽  
Doppler Velocity ◽  
Control Group ◽  
Ventricular Cardiomyopathy ◽  
Velocity Pattern ◽  
Ventricular Dimensions

OBJECTIVETo evaluate new echocardiographic modes in the diagnosis of arrhythmogenic right ventricular cardiomyopathy (ARVC).DESIGNProspective observational study.SETTINGUniversity Hospital.SUBJECTS15 patients with ARVC and a control group of 25 healthy subjects.METHODSTransthoracic echocardiography included cross sectional measurements of the right ventricular outflow tract, right ventricular inflow tract, and right ventricular body. Wall motion was analysed subjectively. M mode and pulsed tissue Doppler techniques were used for quantitative measurement of tricuspid annular motion at the lateral, septal, posterior, and anterior positions. Doppler assessment of tricuspid flow and systemic venous flow was also performed.RESULTSAssessed by M mode, the total amplitude of the tricuspid annular motion was significantly decreased in the lateral, septal, and posterior positions in the patients compared with the controls. The tissue Doppler velocity pattern showed decreased early diastolic peak annular (EA) velocity and an accompanying decrease in early (EA) to late diastolic (AA) velocity ratio in all positions; the systolic annular velocity was significantly decreased only in the lateral position. Four patients had normal right ventricular dimensions and three were judged to have normal right ventricular wall motion. The patient group had also a significantly decreased tricuspid flow E:A ratio.CONCLUSIONSTricuspid annular measurements are valuable, easy to obtain, and allow quantitative assessment of right ventricular function. ARVC patients showed an abnormal velocity pattern that may be an early but non-specific sign of the disease. Normal right ventricular dimensions do not exclude ARVC, and subjective detection of early changes in wall motion may be difficult.

Abstract 16584: Abnormal Right Ventricular Strain by Cardiac Magnetic Resonance in Preclinical Arrhythmogenic Right Ventricular Cardiomyopathy

Circulation ◽  
2014 ◽  
Vol 130 (suppl_2) ◽  
Author(s):  
Davis Vigneault ◽  
Anneline S te Riele ◽  
Cynthia A James ◽  
Stefan L Zimmerman ◽  
Hugh Calkins ◽  
...  
Keyword(s):  
Cardiac Magnetic Resonance ◽  
Magnetic Resonance ◽  
Right Ventricular ◽  
Feature Tracking ◽  
Longitudinal Strain ◽  
Wall Motion ◽  
Arrhythmogenic Right Ventricular Cardiomyopathy ◽  
Statistical Significance ◽  
Ventricular Cardiomyopathy ◽  
Wall Motion Abnormalities

Introduction: Arrhythmogenic right ventricular cardiomyopathy (ARVC) is an inherited cardiomyopathy characterized by regional wall motion abnormalities of the right ventricle (RV) that have not previously been quantified, resulting in challenges / errors in diagnosis of the disease. RV strain is poorly assessed with tagged cardiac magnetic resonance (CMR) due to the thin RV wall. We applied novel feature tracking analysis to assess RV strain in patients with ARVC. Methods: 106 subjects (30 controls, 37 preclinical ARVC [mutation+], and 39 overt ARVC [mutation+, Task Force+] patients) underwent 4 chamber and axial cine imaging using SSFP sequences. The RV was divided into subtricuspid (ST), anterior wall (AW), and apical (Ap) regions. Each region was analyzed to determine peak longitudinal strain and strain-rate using Multimodality Tissue Tracking (MTT) software (MTT Version 6.0.4725, Toshiba Medical Systems Corporation, Tokyo, Japan). Results: Average age was 33.6 ± 16.1 years (48.2% women); there were no differences between groups. In the 4 chamber view, mean global and segmental strain and strain rates decreased in magnitude from control (-37.7% ± 11.2) to preclinical (-32.2% ± 11.5) to overt ARVC (mean -22.2% ± 11.9). Differences between groups most pronounced in the subtricuspid segment, and reached statistical significance between overt ARVC and both control and preclinical ARVC (p < 0.01). A similar trend was observed in longitudinal strain measured in the axial view, but these trends were inconsistent; statistical significance was met globally, but most individual segments did not reach statistical significance. Conclusions: Longitudinal RV strain as measured by CMR feature tracking in ARVC appears able to quantify wall motion abnormalities in overt ARVC, as well as define subtle abnormalities in patients with preclinical ARVC.

Abstract 15754: The Prevalence of Electronic Health Record-Based Clinical Phenotypes in Patients With Pathogenetic Variants Associated With Arrhythmogenic Right Ventricular Cardiomyopathy

Circulation ◽  
2015 ◽  
Vol 132 (suppl_3) ◽  
Author(s):  
Christopher M Haggerty ◽  
Sarah A Pendergrass ◽  
Marci Barr ◽  
Joseph B Leader ◽  
Dustin N Hartzel ◽  
...  
Keyword(s):  
Right Ventricular ◽  
Arrhythmogenic Right Ventricular Cardiomyopathy ◽  
Group Versus ◽  
Primary Treatment ◽  
Control Group ◽  
Implantable Cardioverter Defibrillators ◽  
Ventricular Cardiomyopathy ◽  
Pathogenic Variants ◽  
Increased Risk ◽  
Electronic Health

Objective Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a rare myocardial disorder. Genetic etiology is ascribed to variants in 8 genes ( PKP2 , DSP , DSC2 , DSG2 , JUP , TMEM43 , TGFβ2 , RYR2 ). We aimed to establish the prevalence and clinical phenotype of individuals with pathogenic variants (PV) in these genes from an unselected cohort. Methods Whole-exome sequences for 31,036 patients in the MyCode™ Biorepository were evaluated for pathogenic or likely pathogenic (P/LP) ARVC variants reported in ClinVar or the ARVC database. Composite ICD-9 data from the electronic health records of PV individuals vs a non-PV control group were analyzed. Results 57 P/LP variants were identified in 240 individuals (0.77%), aged 61 ± 19 years. Characteristics of this PV group versus a non-PV control group are summarized in the table. Zero patients in the PV group had a documented diagnosis of ARVC. Use of implantable cardioverter defibrillators—the primary treatment for ARVC—was rare. Of individuals ≥55 years, 24% with PV had no history of cardiac disease (vs. 22% for non-PV). However, there was a modest increase in the prevalence of cardiac electrical abnormalities (e.g., tachycardia, fibrillation) in PV vs. non-PV. There was also a trend towards increased diagnoses of 'other primary cardiomyopathy'. Conclusions The prevalence of reported pathogenic ARVC variants (0.77%) greatly exceeds the disease prevalence (0.02 - 0.10%). In this cohort of 240 genotype-positive subjects, there was little evidence of overt ARVC disease, suggesting that the positive-predictive value for classic ARVC in individuals with an incidental positive genetic finding is low. The increased risk of certain clinical findings, not specific to ARVC, supports the notion that the spectrum of phenotypes associated with pathogenic variants in these genes is broader than classically described. Targeted “deep phenotyping” is needed to assess subclinical phenotypes in genomically screened populations.

P365 Echocardiographic deformation imaging improves detection of arrhythmogenic right ventricular cardiomyopathy; a head-to-head comparison of deformation imaging and conventional assessment

2020 ◽  
Vol 21 (Supplement_1) ◽  
Author(s):  
F P Kirkels ◽  
L P Bosman ◽  
K Taha ◽  
M J Cramer ◽  
F W Asselbergs ◽  
...  
Keyword(s):  
Right Ventricular ◽  
Wall Motion ◽  
Arrhythmogenic Right Ventricular Cardiomyopathy ◽  
Type I ◽  
Ventricular Cardiomyopathy ◽  
Deformation Imaging ◽  
Increased Sensitivity ◽  
Wall Motion Abnormalities ◽  
Motion Assessment ◽  
Deformation Patterns

Abstract Background Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC) is an inherited cardiomyopathy diagnosed by a complex set of tests defined in the 2010 Task Force Criteria (TFC). For echocardiography, right ventricular (RV) dilatation and function are combined with visual wall motion assessment to obtain diagnostic criteria. However, subtle wall motion abnormalities can be missed by visual assessment, thereby limiting detection of disease. Recent studies have shown that echocardiographic deformation imaging has high sensitivity for detection of wall motion abnormalities. However, the performance of deformation imaging within the 2010 TFC for ARVC diagnosis remains unknown. Objectives To perform a head-to-head comparison of the diagnostic value of visual wall motion assessment versus deformation imaging in a real-world cohort of consecutive patients evaluated for ARVC. Methods We included a consecutive cohort of 163 patients who were referred for ARVC evaluation between 2009-2011, of whom 59 patients underwent an echocardiogram with images available for deformation analysis. Patients were diagnosed by consensus of 3 independent ARVC experts with access to all patient data including a median follow-up of 5.9 years IQR[2.7-7.6 yrs]. The original clinical assessment of RV outflow tract (RVOT) dimensions, fractional area change and wall motion was used. In addition, deformation patterns of the subtricuspid area were scored as normal (type I) or abnormal (type II/III), according to the presence of regional mechanical dysfunction (see figure). We evaluated the effect of replacing visual wall motion assessment with deformation imaging on the sensitivity, specificity and balanced accuracy of the echocardiographic TFC. Results Of 59 patients (age 38 ± 17 yrs, 49% male), the expert panel diagnosed 15 (25%) with ARVC. Conventional TFC, either minor or major, were observed in 10 patients; replacing visual wall motion assessment with deformation imaging led to 5 additional detections of ARVC patients, whereas 0 were lost. Consequently, deformation imaging increased sensitivity from 67% to 100%, whereas specificity decreased from 89% to 73%. The balanced accuracy increased from 0.78 to 0.86. Of the 12 patients with false positive TFC by deformation imaging, half were asymptomatic mutation carriers at risk for developing ARVC. Of the other 6 false positives, 3 were diagnosed with ventricular arrhythmia from the RVOT. There were no false negative diagnoses using deformation imaging. Conclusion All definite ARVC patients were detected when deformation imaging patterns were used to evaluate wall motion abnormalities. This increased sensitivity was accompanied by a slight decrease in specificity. Deformation imaging on its own was not able to reliably distinguish ARVC from other RV related disease. Since no ARVC diagnoses were missed, echocardiographic deformation imaging could be of great value to exclude ARVC in patients referred for ARVC evaluation. Abstract P365 Figure. Deformation patterns and % TFC per group

scholarly journals Capturing complex right ventricular wall motion abnormalities in arrhythmogenic right ventricular cardiomyopathy by combining longitudinal and radial myocardial dynamics in feature-tracking MRI

2021 ◽  
Vol 22 (Supplement_1) ◽  
Author(s):  
M Laredo ◽  
J Lamy ◽  
K Bouazizi-Verdier ◽  
A Giron ◽  
A Diallo ◽  
...  
Keyword(s):  
Right Ventricular ◽  
Feature Tracking ◽  
Wall Motion ◽  
Arrhythmogenic Right Ventricular Cardiomyopathy ◽  
Interquartile Range ◽  
Public Institution ◽  
Short Axis ◽  
Ventricular Cardiomyopathy ◽  
Wall Deformation ◽  
Wall Motion Abnormalities

Abstract Funding Acknowledgements Type of funding sources: Public Institution(s). Main funding source(s): INSERM Liliane Bettencourt doctoral grant Background Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC) is ass­­ociated with complex spatial and temporal right ventricular (RV) wall motion abnormalities. While cardiac magnetic resonance (CMR) is the gold-standard imaging technique, its diagnosic performance remains suboptimal and additional CMR biomarkers reflecting ARVC pathophysiology are needed. Purpose To evaluate the performance of a CMR feature-tracking (FT)-derived parameter combining both longitudinal and radial RV deformation and motion for the characterization of RV wall motion abnormalities in ARVC. Methods Thirty-nine patients with definite or borderline ARVC (median age 45 years, interquartile range 31–51, 56% males) were compared to 20 healthy controls with comparable age, sex and weight distributions. All subjects had 1.5T CMR including short axis and 4-chamber views steady-state free precession acquisitions. A custom FT software adapted to RV wall segmentation and tracking was used to assess RV wall deformation and motion in the 3 space directions resulting in: 1) global longitudinal strain (GLS) estimated on the 4 chamber view from the RV free wall, 2) basal circumferential strain (BCS) and radial motion fraction (BRMF) estimated as an average of short-axis slices comprised in the RV third basal portion. To capture the complex RV motion in ARCV, a longitudinal to radial strain loop (LRSL) was displayed and its area was calculated. Results The ARVC group comprised 28 (72%) patients with definite and 11 (28%) with borderline diagnosis . As compared to controls, LVEF and RVEF were significantly lower in ARVC patients (61(interquartile range (IQR) 52-71) vs. 71%(IQR 55-88) , p = 0.03 and 47%(IQR 16-63) vs. 57%(IQR 49-63) , p = 0.02, respectively), LVEF remaining within normal range limits. While there was no significant difference in RV GLS between ARVC patients and controls (median -17.7%(IQR -24–15) vs. -17.5%(IQR -20.1–15.2), p = 0.67) , BCS and BRMF were significantly lower in ARVC patients vs. controls [-7.5%(IQR -12.3–8.4.) vs. -9.8%(IQR -13.8–8.6.), p = 0.004 and -12.2(IQR -14.4–8.7.) vs. -14.9%(IQR -16.6–13.2) p = 0.0007, respectively] . The LRSL area was significantly and markedly lower in ARVC patients vs. controls [70.6 (IQR 16.3-63.1) vs. 144.1 (IQR 110.4-251.3), p = 0.0002] . LRSL area outperformed RVEF, BCS and BRS in separating ARVC from controls (area under receiving operator characteristics curve 0.82 vs. 0.78, 0.73 and 0.78, respectively). Conclusion In ARVC, a FT-derived parameter combining longitudinal and radial RV wall deformation and motion provided better discrimination of ARVC patients from controls than conventional FT measurements. Its implementation in clinical practice may bolster CMR performance to characterize ARVC wall motion abnormalities. Abstract Figure

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