Arrhythmogenic Right Ventricular Cardiomyopathy Diagnosis

Sayed, Amer; Pal, Suman; Poplawska, Maria; Aronow, Wilbert S.; Frishman, William H.; Fuisz, Anthon; Jacobson, Jason T.

doi:10.1097/crd.0000000000000292

Cited by 9 publications

(8 citation statements)

References 59 publications

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“…A residual cardiomyocyte number < 60% evaluated by a morphometric analysis, with fibrous replacement of the right ventricular free wall myocardium are reported as the main diagnostic criteria [ 14 ]. The myocardiocyte replacement usually originates at the subepicardial level, and then tends to extend to the rest of the wall until it reaches the endocardium [ 21 , 22 , 23 , 24 , 25 ]. In the literature, cardiac wall fatty infiltration alone is not considered as a sufficient morphological hallmark of ARVC [ 10 , 15 , 20 ].…”

Section: Resultsmentioning

confidence: 99%

“…In the literature, cardiac wall fatty infiltration alone is not considered as a sufficient morphological hallmark of ARVC [ 10 , 15 , 20 ]. However, some authors highlighted that the mere replacement of myocytes with adipose tissue, in the absence of fibrotic phenomena, would seem to be associated with the thickening of the ventricular walls, the complete sparing of the left ventricular wall and the little or complete absence of an inflammatory infiltrate [ 21 , 22 , 23 ]. The left ventricle involvement is increasingly frequent, and it is usually associated with the sparing of the septal wall [ 11 ].…”

Section: Resultsmentioning

confidence: 99%

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Arrhythmogenic Right Ventricular Cardiomyopathy Post-Mortem Assessment: A Systematic Review

Cianci,

Forzese,

Sapienza

et al. 2024

IJMS

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Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a genetic disorder characterized by the progressive fibro-fatty replacement of the right ventricular myocardium, leading to myocardial atrophy. Although the structural changes usually affect the right ventricle, the pathology may also manifest with either isolated left ventricular myocardium or biventricular involvement. As ARVC shows an autosomal dominant pattern of inheritance with variable penetrance, the clinical presentation of the disease is highly heterogeneous, with different degrees of severity and patterns of myocardial involvement even in patients of the same familiar group with the same gene mutation: the pathology spectrum ranges from the absence of symptoms to sudden cardiac death (SCD) sustained by ventricular arrhythmias, which may, in some cases, be the first manifestation of an otherwise silent pathology. An evidence-based systematic review of the literature was conducted to evaluate the state of the art of the diagnostic techniques for the correct post-mortem identification of ARVC. The research was performed using the electronic databases PubMed and Scopus. A methodological approach to reach a correct post-mortem diagnosis of ARVC was described, analyzing the main post-mortem peculiar macroscopic, microscopic and radiological alterations. In addition, the importance of performing post-mortem genetic tests has been underlined, which may lead to the correct identification and characterization of the disease, especially in those ARVC forms where anatomopathological investigation does not show evident morphostructural damage. Furthermore, the usefulness of genetic testing is not exclusively limited to the correct diagnosis of the pathology, but is essential for promoting targeted screening programs to the deceased’s family members. Nowadays, the post-mortem diagnosis of ARVC performed by forensic pathologist remains very challenging: therefore, the identification of a clear methodological approach may lead to both a reduction in under-diagnoses and to the improvement of knowledge on the disease.

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Section: Resultsmentioning

confidence: 99%

Section: Resultsmentioning

confidence: 99%

Arrhythmogenic Right Ventricular Cardiomyopathy Post-Mortem Assessment: A Systematic Review

Cianci,

Forzese,

Sapienza

et al. 2024

IJMS

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“…About 39% of patients are mainly right ventricular lesions, 56% of patients have lesions involving bilateral ventricles, 5% of patients' lesions mainly involve the LV 40 . Although ARVC can involve the LV in some cases, the lesions are typically located in the posterolateral subepicardial region, whereas LVAH primarily affects the LV apex 41 . LVEMF is a restricted cardiomyopathy characterized by the deposition of fibrous tissue in the apex and ventricular wall 42 .…”

Section: Discussionmentioning

confidence: 99%

“…40 Although ARVC can involve the LV in some cases, the lesions are typically located in the posterolateral subepicardial region, whereas LVAH primarily affects the LV apex. 41 LVEMF is a restricted cardiomyopathy characterized by the deposition of fibrous tissue in the apex and ventricular wall. 42 While LVEMF shares some similarities with LVAH, such as a short and plump LV and LV apical occlusion, it does not involve the wrapping of the right ventricle around the LV as seen in LVAH.…”

Section: Differential Diagnosismentioning

confidence: 99%

Multimodality imaging for the diagnosis of left ventricular apical hypoplasia: A case presentation and review of the literatures

Liao,

Jin,

Miao

et al. 2024

Echocardiography

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Left ventricular apical hypoplasia is a rare malformation recently described congenital abnormality characterized by: (1) truncation of the left ventricle, with the septum projecting toward the right ventricle; (2) abnormal papillary muscle originating from the flattened left ventricular apex; (3) a narrow right ventricle encompassing the periapical area of the left ventricle; (4) fatty infiltration of the apex of the left ventricle. We reported a case of LVAH and reviewed the patient's clinical presentation. And its morphologic characteristics were revealed by multimodality imaging, including echocardiography and cardiac magnetic resonance imaging. Additionally, we reviewed 41 cases from 32 reports to summarize the pathogenesis and analyzed the imaging manifestations of LVAH in this study, aiming to provide new ideas for the diagnosis and clinical management of LVAH patients.

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“…Thus multidetector CT could be a reasonable alternative in patients in which the MRI is less likely to be performed 5 . PET-CT can be useful in differentiating ACM from sarcoidosis 30 .…”

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confidence: 99%

Multimodality imaging in arrhythmogenic cardiomyopathy

Țăpoi¹,

Clément²,

Radu³

et al. 2021

Romanian Journal of Cardiology

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Arrhythmogenic cardiomyopathy, as it has been recently redefi ned, is characterized by progressive myocyte loss with fibrosis and fat infiltration of the myocardium, which finally leads to a broad clinical spectrum ranging from heart failure symptoms to sudden cardiac death. The diagnosis of arrhythmogenic cardiomyopathy is challenging particularly because of its heterogeneity in presentation, which varies from focal right ventricular involvement to biventricular or prominent left ventricular phenotype. In the past decades, the development of new electrocardiographic and imaging diagnostic criteria for arrhythmogenic cardiomyopathy constituted an important area of research and resulted in the elaboration of the Padua criteria. However, even with the widespread availability of modern imaging techniques, there is still a lack of awareness in the health care community and this pathology persist in being under-or misdiagnosed. Given the limited indication of endomyocardial biopsy for the diagnosis of arrhythmogenic cardiomyopathy, one can conclude that the progress that has been made in the last few years in the multimodality imaging field is of utmost importance for the early detection and proper treatment of patients with arrhythmogenic cardiomyopathy, providing valuable prognostic information.

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Arrhythmogenic Right Ventricular Cardiomyopathy Diagnosis

Cited by 9 publications

References 59 publications

Arrhythmogenic Right Ventricular Cardiomyopathy Post-Mortem Assessment: A Systematic Review

Arrhythmogenic Right Ventricular Cardiomyopathy Post-Mortem Assessment: A Systematic Review

Multimodality imaging for the diagnosis of left ventricular apical hypoplasia: A case presentation and review of the literatures

Multimodality imaging in arrhythmogenic cardiomyopathy

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