Dysplasie arythmogène du ventricule droit et mort subite : étude autopsique et histologique

Salem, Nidhal Haj; Mesrati, Med Amin; Hadhri, Rym; Besbes, Souhail; Belhadj, M.; Aissaoui, Abir; Zakhama, A.; Chadly, Ali

doi:10.1016/j.ancard.2015.02.004

Cited by 4 publications

(7 citation statements)

References 17 publications

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“…The main findings of ARVC are adipose or fibro-adipose replacement of myocytes associated with ventricular atrophy [ 8 , 10 , 14 , 15 , 16 , 17 , 18 , 19 , 20 ]. These patterns are predominantly described in the right ventricle, where the most common affected regions are referred to the so-called “triangle of dysplasia”: it is formed by the right ventricular inflow tract, apex and outflow tract [ 14 ].…”

Section: Resultsmentioning

confidence: 99%

“…The affected chamber shows, typically, global or regional dilatation and/or a wall thinning due to both the reduction of myocytes and the increase in adipose and fibro-adipose tissue [ 14 , 17 , 18 ]. It is reported that wall thinning due to myocardial atrophy usually progress over the time, starting from the epicardium and, then, extending toward the endocardium [ 15 , 19 , 20 ]. The disease progression can lead to aneurysmal dilatation of the ventricular cavities, severe segmental dilatation and remodeling processes with formation of fibrotic areas [ 10 , 16 , 17 , 18 , 19 , 20 ].…”

Section: Resultsmentioning

confidence: 99%

“…It is reported that wall thinning due to myocardial atrophy usually progress over the time, starting from the epicardium and, then, extending toward the endocardium [ 15 , 19 , 20 ]. The disease progression can lead to aneurysmal dilatation of the ventricular cavities, severe segmental dilatation and remodeling processes with formation of fibrotic areas [ 10 , 16 , 17 , 18 , 19 , 20 ].…”

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%

Section: Resultsmentioning

confidence: 99%

See 1 more Smart Citation

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

Cianci,

Forzese,

Sapienza

et al. 2024

IJMS

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“…The latter is a more reliable feature for diagnosis [ 15 , 16 ]. The occurrence of myocyte atrophy with or without necrosis as well as the presence of lymphocyte infiltrates have also been reported [ 17 , 18 ]. Myocyte atrophy with mild lymphocyte infiltrate was observed in the index case ( Figure 4 ).…”

Section: Discussionmentioning

confidence: 99%

Right ventricular dysplasia in the elderly: a case report from autopsy

Mgbehoma¹,

Onayemi²,

Soyemi³

et al. 2021

Pan Afr Med J

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Right ventricular dysplasia (RVD) is a rare disease of the heart that primarily affects the right ventricle. It is a clinical and pathological entity that presents classically with palpitations, syncope, or even sudden death. It presents rarely in the elderly. Where sudden death is the first and only presentation, an autopsy is required to make the diagnosis. However, the pathomorphological features of RVD can easily be overlooked or missed at autopsy. We report the case of a 68-year-old male with the past medical history of hypertension, gout and inflammatory bowel syndrome. He was admitted on account of difficulty in breathing, abdominal swelling and reduced urination. Physical examination revealed hypertension with cardiac murmurs, widespread crepitations, distended abdomen and lower limb oedema. Provisional diagnoses of acute-on-chronic kidney disease and congestive cardiac failure secondary to hypertensive heart disease, precipitated by probable gastrointestinal infection were made. While on admission, he had an episode of syncope. Electrocardiogram revealed bigeminy and bradycardic sinus rhythm with unifocal ventricular premature contraction. He died on the 8 th week of admission. Autopsy revealed an enlarged heart weighing 600gm; there was thinning of the apical aspect of the right ventricular wall with subtotal fibrofatty replacement. Microscopic examination revealed a transmural replacement of cardiac myocytes by fibroadipose tissue extending inwards, in the most parts, from the epicardium to the endocardial surface. Our aim is to increase the awareness of these pathomorphological features among anatomic/forensic pathologists.

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“…No obstante, aunque las muestras se tomen de la pared libre, existe una gran variabilidad en la sensibilidad y especificidad en los parámetros morfométricos según la región de donde se tomó la muestra, debido a que la CAVD se distribuye en parches a lo largo del VD (20). Para sobreponerse a estos inconvenientes, se ideó un protocolo de toma de BEM cuando se sospecha CAVD, el cual establece que se deben tomar muestras de la pared libre del VD en la región inferior, antero-apical y del tracto de salida del VD, los cuales son los sitios en los que más comúnmente se presentan los cambios histopatológicos (21,22). Más recientemente se ha propuesto que la BEM se tome de las regiones en las cuales los estudios de imagen realizados previamente muestren defectos en la contractilidad, ya que existe una correlación positiva entre estas regiones y la atrofia miocárdica (23).…”

Section: Discussionunclassified

Cardiomiopatía arritmogénica del ventrículo derecho: Reporte de un caso y revisión del tema

Sanabria

González²

2020

RC_UCR-HSJD

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Dysplasie arythmogène du ventricule droit et mort subite : étude autopsique et histologique

Cited by 4 publications

References 17 publications

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

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

Right ventricular dysplasia in the elderly: a case report from autopsy

Cardiomiopatía arritmogénica del ventrículo derecho: Reporte de un caso y revisión del tema

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