scholarly journals Anatomical-MRI Correlations in Adults and Children with Arrhythmogenic Right Ventricular Cardiomyopathy

Diagnostics ◽  
2021 ◽  
Vol 11 (8) ◽  
pp. 1388
Author(s):  
Simona-Sorana Cainap ◽  
Ilana Kovalenko ◽  
Edoardo Bonamano ◽  
Niclas Crousen ◽  
Alexandru Tirpe ◽  
...  
Keyword(s):  
Magnetic Resonance ◽  
Right Ventricular ◽  
Rare Disease ◽  
Task Force ◽  
Arrhythmogenic Right Ventricular Cardiomyopathy ◽  
Ventricular Myocardium ◽  
The United States ◽  
Fatty Tissue ◽  
Ventricular Cardiomyopathy ◽  
The Right

Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a rare disease in which the right ventricular myocardium is replaced by islands of fibro-adipose tissue. Therefore, ventricular re-entry circuits can occur, predisposing the patient to ventricular tachyarrhythmias, as well as dilation of the right ventricle that eventually leads to heart failure. Although it is a rare disease with low prevalence in Europe and the United States, many patients are addressed disproportionately for cardiac magnetic resonance imaging (MRI). The most severe consequence of this condition is sudden cardiac death at a young age due to untreated cardiac arrhythmias. The purpose of this paper is to revise the magnetic resonance characteristics of ARVC, including the segmental contraction abnormalities, fatty tissue replacement, decrease of the ejection fraction, and the global RV dilation. Herein, we also present several recent improvements of the 2010 Task Force criteria that are not included within the ARVC diagnosis guidelines. In our opinion, these features will be considered in a future Task Force Consensus.

Myocyte Transdifferentiation

2000 ◽  
Vol 124 (2) ◽  
pp. 287-290 ◽  
Author(s):  
Giulia d'Amati ◽  
Cira R. T. di Gioia ◽  
Carla Giordano ◽  
Pietro Gallo
Keyword(s):  
Right Ventricular ◽  
Transplant Patient ◽  
Arrhythmogenic Right Ventricular Cardiomyopathy ◽  
Ventricular Myocardium ◽  
Ventricular Cardiomyopathy ◽  
Familial Dilated Cardiomyopathy ◽  
Familial Cardiomyopathy ◽  
New Type ◽  
The Right ◽  
Gross Examination

Abstract Adipose substitution of ventricular myocardium is characteristic of arrhythmogenic right ventricular cardiomyopathy, but is also found in other heart conditions. It is thought to be a consequence of myocyte loss due to myocarditis or other noxious stimuli. We describe a unique case of cardiomyopathy with a morphologic pattern suggestive of transdifferentiation from myocytes to mature adipocytes. Gross, histologic, and ultrastructural examination were performed on the heart of a female transplant patient with a clinical diagnosis of familial dilated cardiomyopathy. Gross examination showed fibroadipose substitution of the left ventricle and adipose replacement of the right. Histology, immunohistochemistry, and ultrastructure were highly suggestive of transdifferentiation from cardiac muscle to adipose tissue. Myocyte transdifferentiation could represent an alternative pathogenetic pathway to the myocyte-loss and adipose-replacement mechanism in arrhythmogenic right ventricular cardiomyopathy, or it could be the basis of a new type of familial cardiomyopathy.

scholarly journals Possibly Late-Onset Arrhythmogenic Right Ventricular Cardiomyopathy: Unique Triglyceride Deposition by Analysis of Lipid Contents

2019 ◽  
Vol 12 ◽  
pp. 117954761982871
Author(s):  
Kentaro Yamamoto ◽  
Xin Guo ◽  
Ken-ichi Mizutani ◽  
Nozomu Kurose ◽  
Motona Kumagai ◽  
...  
Keyword(s):  
Right Ventricular ◽  
Late Onset ◽  
Arrhythmogenic Right Ventricular Cardiomyopathy ◽  
Ventricular Myocardium ◽  
Cholesterol Content ◽  
Left Ventricular ◽  
Left Ventricular Myocardium ◽  
Ventricular Cardiomyopathy ◽  
Lipid Contents ◽  
The Right

We presented an unusual arrhythmogenic right ventricular cardiomyopathy (ARVC) case of a late-60s elderly man’s death, due to severe pericardial/pleural effusion and ascites, and arrhythmic events, with unique pathological features. The hypertrophic heart grossly displayed yellowish to yellow-whitish predominantly in the variably thinned wall of the dilated right ventricle. Microscopic findings showed diffuse fatty/fibrofatty replacement in not only the right but left ventricular myocardium, together with an outer lymphoplasmacytic infiltrate. According to the lipid contents analysis, the triglyceride content, but not the cholesterol content, in our patient’s right and left ventricular cardiac muscle was much higher than that in the control subject. We propose that this unique triglyceride deposition in our possibly late-onset ARVC case might be one of new clues to understand its enigmatic cause. Further prospective studies are needed to validate the presence and significance of a greater volume of triglyceride deposit, after collecting and investigating a larger number of early and late-onset ARVC cases examined.

Myocardial transcriptome analysis of human arrhythmogenic right ventricular cardiomyopathy

2012 ◽  
Vol 44 (1) ◽  
pp. 99-109 ◽  
Author(s):  
Anna Gaertner ◽  
Patrick Schwientek ◽  
Peter Ellinghaus ◽  
Holger Summer ◽  
Stefan Golz ◽  
...  
Keyword(s):  
Gene Expression ◽  
Right Ventricular ◽  
Molecular Mechanisms ◽  
Arrhythmogenic Right Ventricular Cardiomyopathy ◽  
Expression Patterns ◽  
Ventricular Myocardium ◽  
Idiopathic Dilated Cardiomyopathy ◽  
Orthotopic Heart Transplantation ◽  
Ventricular Cardiomyopathy ◽  
The Right

Arrhythmogenic right ventricular cardiomyopathy (ARVC) is an inherited cardiomyopathy primarily of the right ventricle characterized through fibrofatty replacement of cardiomyocytes. The genetic etiology in ARVC patients is most commonly caused by dominant inheritance and high genetic heterogeneity. Though histological examinations of ARVC-affected human myocardium reveals fibrolipomatous replacement, the molecular mechanisms leading to loss of cardiomyocytes are largely unknown. We therefore analyzed the transcriptomes of six ARVC hearts and compared our findings to six nonfailing donor hearts (NF). To characterize the ARVC-specific transcriptome, we compared our findings to samples from seven patients with idiopathic dilated cardiomyopathy (DCM). The myocardial DCM and ARVC samples were prepared from hearts explanted during an orthotopic heart transplantation representing myocardium from end-stage heart failure patients (NYHA IV). From each heart, left (LV) and right ventricular (RV) myocardial samples were analyzed by Affymetrix HG-U133 Plus 2.0 arrays, adding up to six sample groups. Unsupervised cluster analyses of the groups revealed a clear separation of NF and cardiomyopathy samples. However, in contrast to the other samples, the analyses revealed no distinct expression pattern in LV and RV of myocardial ARVC samples. We further identified differentially expressed transcripts using t-tests and found transcripts separating diseased and NF ventricular myocardium. Of note, in failing myocardium only ∼15–16% of the genes are commonly regulated compared with NF samples. In addition both cardiomyopathies are clearly distinct on the transcriptome level. Comparison of the expression patterns between the failing RV and LV using a paired t-test revealed a lack of major differences between LV and RV gene expression in ARVC hearts. Our study is the first analysis of specific ARVC-related RV and LV gene expression patterns in terminal failing human hearts.

scholarly journals Utility of Cardiac Magnetic Resonance in the Evaluation of Unselected Patients with Possible Arrhythmogenic Right Ventricular Cardiomyopathy

2012 ◽  
Vol 6 ◽  
pp. CMC.S9996 ◽  
Author(s):  
Khang Li Looi ◽  
Colin Edwards ◽  
Hamish Hart ◽  
Jonathan P. Christiansen
Keyword(s):  
Cardiac Magnetic Resonance ◽  
Magnetic Resonance ◽  
Right Ventricular ◽  
Task Force ◽  
Arrhythmogenic Right Ventricular Cardiomyopathy ◽  
Fatty Infiltration ◽  
Venous Drainage ◽  
Ventricular Cardiomyopathy ◽  
Diagnostic Role ◽  
Characteristic Imaging

Introduction Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a rare but important cause of sudden cardiac death. We investigated the role of cardiac magnetic resonance imaging (CMR) in the evaluation of patients with suspected ARVC referred by a general cardiology service. Methods Ninety-two patients (mean age 48 ± 15, 49% female), referred for CMR assessment of possible ARVC, were reviewed. CMR included both functional and tissue characteristic imaging. Results No patients had ARVC based on the 1994 Task Force Criteria (TFC) prior to CMR, but 4 met proposed Modified TFC; 15% met one major (±1 minor) TFC, 71% 1 or 2 minor TFC, and 14% no TFC. Reasons for CMR referral included symptomatic arrhythmia of likely RV origin (28%), Electrocardiogram/Holter abnormalities (28%), echocardiographic features suspicious of ARVC (19%), and family history of ARVC (8%). CMR findings strongly suggestive of ARVC were found in nine patients (10%), although only three were considered typical. Of these patients two met 1 major TFC and seven met 1 or 2 minor TFC. CMR findings included RV thinning, aneurysm, and diastolic out-pouching, but only 1 patient had definite fatty infiltration of the RV. Incidentally, CMR detected important, previously undiagnosed pathology, including anomalous pulmonary venous drainage (2 patients) and non-ischaemic cardiomyopathy (6%). CMR was normal in 63%, with minor abnormalities in 29%. Conclusions CMR may play an important diagnostic role in the evaluation of possible ARVC. Patients who do not meet TFC for diagnosis may have CMR features typical of ARVC. Additionally CMR may detect other hitherto undiagnosed structural or functional abnormalities that alter patient management. However the majority of patients referred have a low pretest probability of ARVC, and the rate of normal CMR scans is high.

scholarly journals Arrhythmogenic left ventricular cardiomyopathy

2020 ◽  
Vol 6 (1) ◽  
pp. 20190079
Author(s):  
Seyedeh Mojdeh Mirmomen ◽  
Andrew Jay Bradley ◽  
Andrew Ernest Arai ◽  
Arlene Sirajuddin
Keyword(s):  
Right Ventricular ◽  
Cardiac Death ◽  
Arrhythmogenic Right Ventricular Cardiomyopathy ◽  
Ventricular Myocardium ◽  
Left Ventricular ◽  
Ventricular Cardiomyopathy ◽  
Cardiotoxic Effect ◽  
Life Threatening ◽  
Muscle Disorder ◽  
The Right

Arrhythmogenic ventricular cardiomyopathy (AVC) is a heritable heart muscle disorder characterized by fibrofatty infiltration of the myocardium. Intramyocardial fat deposition is considered arrhythmogenic and predisposes patients to life-threatening arrhythmias and sudden cardiac death. The classic subtype of AVC is characterized by fibrofatty replacement of the right ventricular myocardium (i.e. arrhythmogenic right ventricular cardiomyopathy). In advanced cases of arrhythmogenic right ventricular cardiomyopathy, the left ventricle may be involved as well. Predominantly left ventricular involvement by AVC is exceedingly rare and lack of specific diagnostic criteria as well as its potential cardiotoxic effect make its diagnosis challenging and of high importance.

scholarly journals Sporadic and rapidly progressive arrhythmogenic right ventricular cardiomyopathy in a 12-year-old boy who was diagnosed with epilepsy

2021 ◽  
Vol 2021 (6) ◽  
Author(s):  
Alaa Al-Khleaf ◽  
Amal Babi ◽  
Mulham Jarjanazi ◽  
Walid Haddad
Keyword(s):  
Right Ventricular ◽  
Genetic Disease ◽  
Arrhythmogenic Right Ventricular Cardiomyopathy ◽  
Ventricular Myocardium ◽  
Pediatric Emergency ◽  
Rapid Progression ◽  
Ventricular Cardiomyopathy ◽  
Primary And Secondary Prevention ◽  
Potential Risks ◽  
The Right

ABSTRACT Arrhythmogenic right ventricular cardiomyopathy (ARVC) is one of the leading causes of sudden cardiac death amongst young people and athletes. In this genetic disease, arrhythmia and fibro-fatty changes in the right ventricular myocardium are the main characteristics of the disease. Here, we report a case of ARVC in a 12-year-old boy who was previously diagnosed with epilepsy, the patient’s condition manifested sporadically and was complicated by rapid progression, and unfortunate fatal deterioration after admission into the pediatric emergency room due to fatigue, dizziness and palpitation. A diagnosis of ARVC was established, even though a family history was absent. Due to possible rapid deterioration, as described in this case, we recommend immediate primary and secondary prevention of arrhythmias in these patients, and to take in consideration of the potential risks of using sodium valproate in these patients.

P1077Late gadolinium enhancement with cardiac magnetic resonance imaging demonstrates left ventricular involvement is under-recognized in arrhythmogenic right ventricular cardiomyopathy

EP Europace ◽  
2020 ◽  
Vol 22 (Supplement_1) ◽  
Author(s):  
S Mohamed ◽  
M Kunz ◽  
S Casey ◽  
W Katsiyiannis ◽  
R Abdelhadi ◽  
...  
Keyword(s):  
Cardiac Magnetic Resonance ◽  
Late Gadolinium Enhancement ◽  
Magnetic Resonance ◽  
Right Ventricular ◽  
Arrhythmogenic Right Ventricular Cardiomyopathy ◽  
Left Ventricular ◽  
Fatty Tissue ◽  
Post Mortem ◽  
Ventricular Cardiomyopathy ◽  
Gadolinium Enhancement

Abstract Funding Acknowledgements The study is partially funded by Medtronic and the Minneapolis Heart Institute Foundation. Background/Introduction: Arrhythmogenic right ventricular cardiomyopathy (ARVC) is characterized by replacement of the myocardium with fibrous and fatty tissue that may lead to an increased risk of ventricular arrhythmias and heart failure.  Although left ventricular (LV) and biventricular forms have been identified post-mortem resulting in the increased use of the term arrhythmogenic cardiomyopathy, there is only inclusion of right ventricular wall motion abnormalities in the taskforce diagnostic criteria. Purpose The aim of our study was to examine the utility of cardiac magnetic resonance (CMR) imaging in characterizing LV or biventricular involvement with late gadolinium enhancement (LGE) in a large cohort of patients with suspected ARVC. Methods Retrospective, single-institution, chart review of 76 patients diagnosed with ARVC between January 2009 and July 2019. Data collection and analysis included baseline demographics and parameters specific to diagnosis (definite, borderline, or possible) and risk stratification of ARVC based on 2019 modified taskforce criteria, as well as detailed CMR evaluation. Results Of the 76 patients with ARVC, 66 (87%) had at least one CMR with gadolinium administered. In that subset of patients, 27 (41%) had LGE. Of those with LGE, LV involvement was identified in 23 (85%) patients. The pattern of LGE was not localized to one myocardial region but demonstrated variable LV enhancement patterns including anterior, inferior, lateral, septal, basal, mid, apical, and from the sub-epicardium into the mid-myocardium. Conclusions   Left ventricular involvement reflected by LGE was identified in a high percentage of patients with suspected ARVC, and there was significant variation in the pattern of distribution in terms of region and depth of myocardial involvement.  While post-mortem examination of patients with ARVC demonstrates a high prevalence of left ventricular involvement, this study shows that CMR can consistently detect late gadolinium enhancement, and ARVC should be considered in the differential diagnosis for biventricular cardiomyopathy.  The identification of variable locations of LGE within the LV suggests there is more than one phenotype, and this imaging modality may help to clarify the implications of left ventricular involvement in disease progression.

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