Intracardiac Echocardiography-Derived Right Ventricular Myocardial Strain Deformation Patterns with Segmental Dyssynchrony in Arrhythmogenic Cardiomyopathy

2021 ◽  
Vol 34 (1) ◽  
pp. 100-102
Author(s):  
Shiquan Chen ◽  
Jian-Fang Ren ◽  
Chenyang Jiang ◽  
Francis E. Marchlinski
Keyword(s):  
Right Ventricular ◽  
Myocardial Strain ◽  
Intracardiac Echocardiography ◽  
Arrhythmogenic Cardiomyopathy ◽  
Deformation Patterns

scholarly journals Parameter subset reduction for patient-specific modelling of arrhythmogenic cardiomyopathy-related mutation carriers in the CircAdapt model

2020 ◽  
Vol 378 (2173) ◽  
pp. 20190347 ◽  
Author(s):  
Nick van Osta ◽  
Aurore Lyon ◽  
Feddo Kirkels ◽  
Tijmen Koopsen ◽  
Tim van Loon ◽  
...  
Keyword(s):  
Right Ventricular ◽  
Computational Models ◽  
Clinical Decision Making ◽  
Early Stage ◽  
Patient Specific ◽  
Model Parameters ◽  
Arrhythmogenic Cardiomyopathy ◽  
Tissue Properties ◽  
Myocardial Disease ◽  
Deformation Patterns

Arrhythmogenic cardiomyopathy (AC) is an inherited cardiac disease, clinically characterized by life-threatening ventricular arrhythmias and progressive cardiac dysfunction. Patient-specific computational models could help understand the disease progression and may help in clinical decision-making. We propose an inverse modelling approach using the CircAdapt model to estimate patient-specific regional abnormalities in tissue properties in AC subjects. However, the number of parameters ( n  = 110) and their complex interactions make personalized parameter estimation challenging. The goal of this study is to develop a framework for parameter reduction and estimation combining Morris screening, quasi-Monte Carlo (qMC) simulations and particle swarm optimization (PSO). This framework identifies the best subset of tissue properties based on clinical measurements allowing patient-specific identification of right ventricular tissue abnormalities. We applied this framework on 15 AC genotype-positive subjects with varying degrees of myocardial disease. Cohort studies have shown that atypical regional right ventricular (RV) deformation patterns reveal an early-stage AC disease. The CircAdapt model of cardiovascular mechanics and haemodynamics has already demonstrated its ability to capture typical deformation patterns of AC subjects. We, therefore, use clinically measured cardiac deformation patterns to estimate model parameters describing myocardial disease substrates underlying these AC-related RV deformation abnormalities. Morris screening reduced the subset to 48 parameters. qMC and PSO further reduced the subset to a final selection of 16 parameters, including regional tissue contractility, passive stiffness, activation delay and wall reference area. This article is part of the theme issue ‘Uncertainty quantification in cardiac and cardiovascular modelling and simulation’.

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.

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 P1344 Right ventricular mass: multimodality diagnostic approach to an incidental finding

2020 ◽  
Vol 21 (Supplement_1) ◽  
Author(s):  
J Ambrozic ◽  
A Kovac ◽  
R Zbacnik ◽  
S Ponorac ◽  
P Kogoj ◽  
...  
Keyword(s):  
Right Ventricular ◽  
Incidental Finding ◽  
Contrast Echocardiography ◽  
Interventricular Septum ◽  
Percutaneous Biopsy ◽  
Intracardiac Echocardiography ◽  
Diagnostic Approach ◽  
Right Ventricular Mass ◽  
History Of ◽  
The Right

Abstract Background Cardiac masses are rare entities and often diagnostically challenging. When incidentally found multimodality diagnostic approach is warranted for further characterization and evaluation of its contribution to the patient’s symptoms. Case report 62-year-old male, smoker, with a history of non-Hodgkin lymphoma treated with radiation and chemotherapy 10 years ago, was admitted due to subacute myocardial infarction. Coronary angiogram showed subacute occlusion of partly collateralized RCA, subsequently treated with dilatation and stenting. Echocardiography revealed signs of dilated cardiomyopathy with severely reduced left ventricular ejection fraction (EF 20%), that could be due to chemotherapy-related cardiotoxicity. On admission CTA was performed to exclude aortic dissection since the patient presented with very severe chest pain radiating to the back. It showed a heterogeneous formation within the right ventricle (RV) extending along the interventricular septum from the apex towards the tricuspid annulus, appearing to involve interventricular septum as well. According to the imaging characteristics the mass was suspected to be a liposarcoma (Figure A). With contrast echocardiography hypoechogenic formation in the RV on parasternal long-axis view could be visualized (Figure B). Due to previous history of malignancy PET scan was performed that revealed hypometabolic RV mass, suggesting a benign tumor, without any sign of metastasis. We decided for percutaneous biopsy that was done under intracardiac echocardiography (ICE) guidance using the view from the RV towards RV outflow tract (Figure C). Histology revealed proliferation of mature adipocytes, either orthotopic or multiplied (Figure D). To exclude well-differentiated liposarcoma FISH (fluorescent in situ hybridization) with amplification of MDM2 gene was undertaken, confirming benign lesion, most probably cardiac lipoma. It was concluded that the mass was an incidental finding, not related to the patient’s symptoms and not causing any functional disturbances; therefore surgical therapy could be avoided. Conclusion While echocardiography remains the first-line imaging modality, multimodality diagnostic approach is mandatory for evaluation and treatment decision of a newly-discovered cardiac mass. Histology provides definitive diagnosis and ICE could be helpful for guiding percutaneous biopsy, thus avoiding invasive open-heart procedures. Figure. Cardiac CTA (A), contrast echocardiography (B), intracardiac echocardiography (C) and histologic specimen (D) of the right ventricular mass (arrows). Abstract P1344 Figure.

scholarly journals Knockout of SORBS2 Protein Disrupts the Structural Integrity of Intercalated Disc and Manifests Features of Arrhythmogenic Cardiomyopathy

2020 ◽  
Vol 9 (17) ◽  
Author(s):  
Yonghe Ding ◽  
Jingchun Yang ◽  
Peng Chen ◽  
Tong Lu ◽  
Kunli Jiao ◽  
...  
Keyword(s):  
Right Ventricular ◽  
Structural Integrity ◽  
Human Genetics ◽  
Susceptibility Gene ◽  
Right Ventricular Dysfunction ◽  
Premature Death ◽  
Phenotypic Characterization ◽  
Mouse Heart ◽  
Arrhythmogenic Cardiomyopathy ◽  
Intercalated Disc

Background Sorbs2b (sorbin and SH3 domain‐containing 2b) was recently identified as a cardiomyopathy gene from a zebrafish mutagenesis screen. However, cardiac functions of its mammalian ortholog remain elusive. Methods and Results We conducted a detailed expression and subcellular localization analysis of Sorbs2 ortholog in mice and a phenotypic characterization in Sorbs2 knockout mice. Sorbs2 is highly expressed in the mouse heart and encodes an adhesion junction/desmosome protein that is mainly localized to the intercalated disc. A mutation with near complete depletion of the Sorbs2 protein in mice results in phenotypes characteristic of human arrhythmogenic cardiomyopathy (ACM), including right ventricular dilation, right ventricular dysfunction, spontaneous ventricular tachycardia, and premature death. Sorbs2 is required to maintain the structural integrity of intercalated disc. Its absence resulted in profound cardiac electrical remodeling with impaired impulse conduction and action potential derangements. Targeted sequencing of human patients with ACM identified 2 rare splicing variants classified as likely pathogenic were in 2 unrelated individuals with ACM from a cohort of 59 patients with ACM. Conclusions The Sorbs2 knockout mouse manifests several key features reminiscent of human ACM. Although the candidacy of SORBS2 as a new ACM‐susceptibility gene is supported by preliminary human genetics study, future validation in larger cohorts with ACM is needed.

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.

Right ventricular remodeling in athletes and in arrhythmogenic cardiomyopathy

2017 ◽  
Vol 52 (1) ◽  
pp. 13-19 ◽  
Author(s):  
Monica Chivulescu ◽  
Kristina Haugaa ◽  
Øyvind H. Lie ◽  
Thor Edvardsen ◽  
Carmen Ginghină ◽  
...  
Keyword(s):  
Right Ventricular ◽  
Ventricular Remodeling ◽  
Arrhythmogenic Cardiomyopathy ◽  
Right Ventricular Remodeling
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