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

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.

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Identification of Arrhythmogenic Right Ventricular Cardiomyopathy-Causing Gene Mutations in Young Sudden Unexpected Death Autopsy Cases

Journal of Forensic Sciences ◽

10.1111/1556-4029.12657 ◽

2015 ◽

Vol 60 (2) ◽

pp. 457-461 ◽

Cited By ~ 8

Author(s):

Takako Sato ◽

Hajime Nishio ◽

Koichi Suzuki

Keyword(s):

Right Ventricular ◽

Arrhythmogenic Right Ventricular Cardiomyopathy ◽

Gene Mutations ◽

Sudden Unexpected Death ◽

Ventricular Cardiomyopathy ◽

Unexpected Death

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Association of epicardial fat thickness with left ventricular diastolic function parameters in a community population

10.21203/rs.3.rs-171598/v2 ◽

2021 ◽

Author(s):

Wei Ma ◽

Baowei Zhang ◽

Ying Yang ◽

Litong Qi ◽

Jin Zhou ◽

...

Keyword(s):

Confidence Interval ◽

Right Ventricular ◽

Diastolic Function ◽

Ventricular Free Wall ◽

Free Wall ◽

Right Ventricular Free Wall ◽

Community Population ◽

The Mean ◽

The Right ◽

The Relationship

Abstract Purpose We examined the relationship between EFT measured by echocardiography and LV diastolic function parameters in a Beijing community population. Methods We included 1004 participants in this study. Echocardiographic parameters including E and A peak velocity, the early diastolic velocities (e’) of the septal and lateral of mitral annulus using tissue doppler imaging, E/e’, and EFT, were measured. EFT1 was measured perpendicularly on the right ventricular free wall at end-diastole in the extension line of the aortic root. EFT2 was the maximum thickness measured perpendicularly on the right ventricular free wall at end-diastole. Multivariate linear regression was used to analyze the relationship between EFT and the mean e’ and E/e’. Results The mean age of the participants was 63.91 ± 9.02 years old (51.4% men). EFT1 and EFT2 were negatively correlated with e’ lat, e’ sep, and e’ mean (p < 0.05), and positively correlated with E/e’ lat, E/e’ sep, and E/e’ mean. Multivariate regression analysis showed that EFT1 and EFT2 were independently and negatively correlated with e’ mean (EFT1: β = −0.089 [95% confidence interval = − 0.177, − 0.000, p = 0.050]; EFT2: β = −0.078 [95% confidence interval = − 0.143, − 0.012, p = 0.020]). There were no interactions between EFT and any covariates, including age or heart groups, sex, BMI, or presence of hypertension, diabetes, or coronary heart disease in relation to LV diastolic dysfunction. Conclusions EFT was negatively and independently correlated with e’ mean, suggesting that more attention to this type of adipose fat is required for cardiovascular disease therapy.

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Myocyte Transdifferentiation

Archives of Pathology & Laboratory Medicine ◽

10.5858/2000-124-0287-mt ◽

2000 ◽

Vol 124 (2) ◽

pp. 287-290 ◽

Cited By ~ 3

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.

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Progressive right ventricular outflow tract fibrosis after repair of tetralogy of Fallot

Cardiology in the Young ◽

10.1017/s1047951120002164 ◽

2020 ◽

Vol 30 (9) ◽

pp. 1366-1367

Author(s):

Gauri R. Karur ◽

Wadi Mawad ◽

Lars Grosse-Wortmann

Keyword(s):

Cardiac Magnetic Resonance ◽

Right Ventricular ◽

Tetralogy Of Fallot ◽

T1 Mapping ◽

Right Ventricular Outflow Tract ◽

Ventricular Outflow Tract ◽

Ventricular Free Wall ◽

Free Wall ◽

Right Ventricular Free Wall ◽

The Right

AbstractObjectives:The objective of this study was to determine the evolution of fibrosis over time and its association with clinical status.Methods:Children with repaired tetralogy of Fallot who had undergone at least two cardiac magnetic resonance examinations including T1 mapping at least 1 year apart were included.Results:Thirty-seven patients (12.7 ± 2.6 years, 61% male) were included. Right ventricular free wall T1 increased (913 ± 208 versus 1023 ± 220 ms; p = 0.02). Baseline cardiac magnetic resonance parameters did not predict a change in imaging markers or exercise tolerance. The right ventricular free wall per cent change correlated with left ventricular T1% change (r = 0.51, p = 0.001) and right ventricular mass Z-score change (r = 0.51, p = 0.001). T1 in patients with late gadolinium enhancement did not differ from the rest.Conclusion:Increasing right ventricular free wall T1 indicates possible progressive fibrotic remodelling in the right ventricular outflow tract in this pilot study in children and adolescents with repaired tetralogy of Fallot. The value of T1 mapping both at baseline and during serial assessments will need to be investigated in larger cohorts with longer follow-up.

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