A Case of Arrhythmogenic Right Ventricular Cardiomyopathy in Sinus Rhythm Associated with Thrombus in the Right Atrium

2000 ◽  
Vol 13 (2) ◽  
pp. 0154-0156
Author(s):  
Mehmet Andr[eacute]s Bilge ◽  
Beyhan Andr[eacute]s Eryonucu ◽  
Niyazi Andr[eacute]s G[uuml ]ler
Keyword(s):  
Sinus Rhythm ◽  
Right Ventricular ◽  
Right Atrium ◽  
Arrhythmogenic Right Ventricular Cardiomyopathy ◽  
Ventricular Cardiomyopathy ◽  
The Right

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.

Sinus rhythm detection of conducting channels and ventricular tachycardia isthmus in arrhythmogenic right ventricular cardiomyopathy

Heart Rhythm ◽  
2014 ◽  
Vol 11 (5) ◽  
pp. 747-754 ◽  
Author(s):  
Juan Fernández-Armenta ◽  
David Andreu ◽  
Diego Penela ◽  
Emilce Trucco ◽  
Laura Cipolletta ◽  
...  
Keyword(s):  
Ventricular Tachycardia ◽  
Sinus Rhythm ◽  
Right Ventricular ◽  
Arrhythmogenic Right Ventricular Cardiomyopathy ◽  
Ventricular Cardiomyopathy

scholarly journals Asymptomatic Presentation in Arrhythmogenic Right Ventricular Cardiomyopathy: a Case Report.

2021 ◽  
Vol 18 (1) ◽  
pp. 65-66
Author(s):  
Anish Hirachan ◽  
Ranjit Sharma ◽  
Prabesh Neupane ◽  
Milan Gautam ◽  
Ram Kumar Ghimire
Keyword(s):  
Right Ventricular ◽  
Cardiac Death ◽  
Multimodality Imaging ◽  
Arrhythmogenic Right Ventricular Cardiomyopathy ◽  
Specific Treatment ◽  
Ventricular Cardiomyopathy ◽  
Delay In Diagnosis ◽  
Cardiac Condition ◽  
The Common ◽  
The Right

Arrythmogenic right ventricular cardiomyopathy (ARVC) is an inherited cardiac condition leading to one of the common cause for sudden cardiac death in young individuals. It is characterized by fibrofatty replacement of the cardiac myocytes predominantly the right ventricle. As the presentation can be varied and very nonspecific, it often leads to delay in diagnosis and specific treatment. Here, we present a 30 year male who was asymptomatic and detected to have electrocardiograph (ECG) and echocardiographic abnormalities suggestive of ARVC. Further, a cardiac imaging with cardiac MRI confirmed the diagnosis of ARVC highlighting the importance of multimodality imaging.

Arrhythmogenic Right Ventricular Cardiomyopathy with Left Ventricular Involvement: A Novel Splice Site Mutation in the DSG2 Gene

Cardiology ◽  
2015 ◽  
Vol 130 (3) ◽  
pp. 159-161 ◽  
Author(s):  
Marina Pereira Fernandes ◽  
Olga Azevedo ◽  
Vitor Pereira ◽  
Lucy Calvo ◽  
António Lourenço
Keyword(s):  
Right Ventricular ◽  
Arrhythmogenic Right Ventricular Cardiomyopathy ◽  
Splice Site Mutation ◽  
Left Ventricular ◽  
Transthoracic Echocardiogram ◽  
Ventricular Cardiomyopathy ◽  
Site Mutation ◽  
Electrocardiographic Monitoring ◽  
History Of ◽  
The Right

We report the case of a 37-year-old male patient admitted to the cardiac intensive care unit for acute pulmonary edema. He had a history of excessive alcoholic consumption and had had a viral syndrome in the preceding 10 days. A transthoracic echocardiogram revealed severe biventricular dysfunction, mild dilatation of the left heart chambers, and severe dilatation of the right chambers. Nonsustained ventricular tachycardia with a left bundle branch block morphology was detected during electrocardiographic monitoring. In the follow-up, he underwent a contrast-enhanced transthoracic echocardiogram and a cardiac resonance which were compatible with the diagnosis of arrhythmogenic right ventricular cardiomyopathy with biventricular involvement. Molecular analysis detected the mutation c.1423+2T>G (IVS10 ds +2T>G) in intron 10 of the gene DSG2 (desmoglein-2) in heterozygosity. To our knowledge, this mutation has not been previously described in arrhythmogenic right ventricular cardiomyopathy.

Sudden Unexpected Death in a 31-Year-Old Man Caused by Arrhythmogenic Right Ventricular Cardiomyopathy

2005 ◽  
Vol 129 (10) ◽  
pp. 1330-1333
Author(s):  
Dongjiu Ye ◽  
William D. Edwards ◽  
Waheeb Rizkalla
Keyword(s):  
Right Ventricular ◽  
Ventricular Myocytes ◽  
Interstitial Fibrosis ◽  
Arrhythmogenic Right Ventricular Cardiomyopathy ◽  
Free Wall ◽  
Ventricular Cardiomyopathy ◽  
Right Ventricular Free Wall ◽  
Unexpected Death ◽  
Negative Results ◽  
The Right

Abstract A 31-year-old white man collapsed suddenly at a graduation ceremony and was pronounced dead after attempted resuscitation. He had no pertinent medical or familial history. Postmortem toxicologic studies showed negative results. A complete autopsy revealed a cardiac cause of death. Grossly, the right ventricular chamber was moderately to markedly dilated, and its free wall showed extensive myocardial adiposity. Microscopically, the right ventricular free wall consisted predominantly of adipose tissue, with only small subendocardial islands of hypertrophied myocytes and interstitial fibrosis. These features are characteristic of arrhythmogenic right ventricular cardiomyopathy. Moreover, Purkinje-like cells were observed among right ventricular myocytes and may have increased the likelihood of developing an arrhythmia. To our knowledge, this finding has not been previously emphasized. Because arrhythmogenic right ventricular cardiomyopathy accounts for 10% of cases of sudden unexpected cardiac death, recognition of this disease by pathologists is important, especially in cases of otherwise unexplained death in young persons.

Arrhythmogenic right ventricular cardiomyopathy: contemporary challenges of diagnosis and management

2019 ◽  
Vol 14 (12) ◽  
pp. 1-14
Author(s):  
Liam Driver
Keyword(s):  
Right Ventricular ◽  
Cardiac Death ◽  
Ventricular Arrhythmias ◽  
Structural Changes ◽  
Arrhythmogenic Right Ventricular Cardiomyopathy ◽  
Ventricular Cardiomyopathy ◽  
Device Therapy ◽  
Score System ◽  
To Come ◽  
The Right

Arrhythmogenic right ventricular cardiomyopathy is a rare but potentially lethal condition that predominantly affects the right ventricle although a left or biventricular form is now recognised. The condition can present in a variety of ways from palpitations to aborted sudden cardiac death. It is characterised by the replacement of healthy myocardium with fatty infiltrates and is progressive in nature. There is no single test for this condition and diagnosis can be difficult – relying on a diagnostic criteria to aid diagnosis. This score system uses criteria based on ECG findings, evidence of arrhythmia, structural changes detected on echocardiogram and MRI, histopathology and family history. Treatment is initially medicine based, to suppress ventricular arrhythmias; however, patients may require device therapy, ablation or cardiac transplantation. There are recognised psychological implications for patients with arrhythmogenic right ventricular tachycardia and they may require support to come to terms with the changes that a diagnosis can bring.

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