Myocarditic Appearance of Arrhythmogenic Right Ventricular Cardiomyopathy

Jordan, Andrew N.; Lyne, Jonathan; Silva, Ranil de; Wong, Tom

doi:10.1161/circulationaha.110.960096

Cited by 9 publications

(8 citation statements)

References 5 publications

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“…Although fibrofatty replacement of the myocardium is the hallmark feature of AC, the consistent observation of small lymphocytic foci in the disease 31– 33 further supports the involvement of cardiac-resident Sca1+ PDGFRα+ mesenchymal progenitors in its progression, as these progenitors have been recently shown to contribute in vivo to lymph node stroma 34 and follicular dendritic cells 35 . Thus, the milieu of chronic myocardial inflammation may trigger their differentiation not just into adipocytes and myofibroblasts, but also possibly in cells capable of attracting and supporting lymphocytes.…”

Section: Cellular Source Of Fibrofatty Infiltration In Arrhythmogenicmentioning

confidence: 71%

Tissue-resident Sca1+ PDGFRα+ mesenchymal progenitors are the cellular source of fibrofatty infiltration in arrhythmogenic cardiomyopathy

et al. 2013

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Arrhythmogenic cardiomyopathy (AC) is a disease of the heart involving myocardial dystrophy leading to fibrofatty scarring of the myocardium and is associated with an increased risk of both ventricular arrhythmias and sudden cardiac death. It often affects the right ventricle but may also involve the left. Although there has been significant progress in understanding the role of underlying desmosomal genetic defects in AC, there is still a lack of data regarding the cellular processes involved in its progression. The development of cardiac fibrofatty scarring is known to be a principal pathological process associated with ventricular arrhythmias, and it is vital that we elucidate the role of various cell populations involved in the disease if targeted therapeutics are to be developed. The known role of mesenchymal progenitor cells in the reparative process of both the heart and skeletal muscle has provided inspiration for the identification of the cellular basis of fibrofatty infiltration in AC. Here we hypothesize that reparative processes triggered by myocardial degeneration lead to the differentiation of tissue-resident Sca1+ PDGFRα+ mesenchymal progenitors into adipocytes and fibroblasts, which compose the fibrofatty lesions characteristic of AC.

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Section: Cellular Source Of Fibrofatty Infiltration In Arrhythmogenicmentioning

confidence: 71%

Tissue-resident Sca1+ PDGFRα+ mesenchymal progenitors are the cellular source of fibrofatty infiltration in arrhythmogenic cardiomyopathy

et al. 2013

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“…However, this hypothesis is controversial, because the inflammation could be a secondary event; infact, an already diseased myocardium could have an increased susceptibility to viral infection with resulting myocardial inflammation [6]. Therefore, the precise relationship between ARVD and acute myocarditis is not yet determined [7]. …”

Section: Discussionmentioning

confidence: 99%

A unique association of arrhythmogenic right ventricular dysplasia and acute myocarditis, as assessed by cardiac MRI: a case report

Ponsiglione

Puglia

Morisco

et al. 2016

BMC Cardiovasc Disord

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BackgroundArrhythmogenic right ventricular dysplasia (ARVD), is a genetic disorder of the heart, which mainly involves the right ventricle. It is characterized by hypokinetic areas at the free wall of the right ventricle (RV) or both ventricles, where myocardium is replaced by fibrous or fatty tissue. ARVD is an important cause of ventricular arrhythmias in children and young adults. Although the transmission of the disease is based on hereditary, in young adults it may not show any symptoms. The main differential diagnoses with other frequent etiological causes of sudden arrhythmia are: idiopathic outflow tract ventricular tachycardia of the RV, myocarditis, dilated cardiomyopathy and sarcoidosis.Case presentationWe describe an unusual case of a 44-year-old woman who was hospitalized for ventricular tachycardia, deep asthenia and dyspnoea with no previous history of cardiac disease. The patient had a ten-year history of palpitations, which started immediately after her last pregnancy. She was diagnosed with both acute/subacute viral myocarditis and arrhythmogenic right ventricular dysplasia, based on established clinical and cardiac MRI criteria. After the diagnosis the patient received an automatic implantable cardioverter defibrillator. Currently, she is on clinical follow-up with no apparent further complications.ConclusionAnalyzing this rare case, we have shown the link between myocarditis and arrhythmogenic right ventricular dysplasia, and how important is to perform a cardiac MRI, in the context of acute myocarditis and ventricular arrhythmia.

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“…During the last decades, a possible role for inflammation in the development and progression of myocardial damage in patients with DSP mutations has been postulated, supporting the hypothesis that inflammatory dysregulation could contribute to phenotypic disease progression [ 9 ]. In terms of pathophysiology, possible variable links between myocarditis and arrhythmogenic cardiomyopathy have been proposed, and clusters of familial-recurrent myocarditis associated with DSP mutations have been increasingly described [ 10 , 11 ]. However, whether myocarditis is a primary cause of the development of the cardiomyopathic phenotype or the result of cellular and humoral reaction to cardiomyocytes dysfunction and death is not clearly understood.…”

Section: Discussionmentioning

confidence: 99%

Imaging the “Hot Phase” of a Familiar Left-Dominant Arrhythmogenic Cardiomyopathy

Rubino

Scatteia²,

Frisso

et al. 2021

Genes

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Myocarditic Appearance of Arrhythmogenic Right Ventricular Cardiomyopathy

Cited by 9 publications

References 5 publications

Tissue-resident Sca1+ PDGFRα+ mesenchymal progenitors are the cellular source of fibrofatty infiltration in arrhythmogenic cardiomyopathy

Tissue-resident Sca1+ PDGFRα+ mesenchymal progenitors are the cellular source of fibrofatty infiltration in arrhythmogenic cardiomyopathy

A unique association of arrhythmogenic right ventricular dysplasia and acute myocarditis, as assessed by cardiac MRI: a case report

Imaging the “Hot Phase” of a Familiar Left-Dominant Arrhythmogenic Cardiomyopathy

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