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arrhythmogenic
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Book Chapter
Series: Atlases of Tumor and Non-Tumor Pathology, Series 5
Volume: 17
Published: 15 November 2023
10.55418/9781933477299-ch17
ISBN-10: 1-933477-29-6
ISBN: 978-1-933477-29-9
...Table 17-1 GENES IMPLICATED IN ARRHYTHMOGENIC CARDIOMYOPATHY Gene Protein Frequency Identified Reference PKP2 Plakophilin-2 34%-74% ( 47 ) DSP Desmoplakin 2%-39% ( 47 ) DSG2 Desmoglein-2 5%-26% ( 47 ) DSC2 Desmocollin-2 1%-2...
Images
Published: 15 November 2023
ARRHYTHMOGENIC CARDIOMYOPATHY: MOLECULAR MECHANISMS Figure 17-1 ARRHYTHMOGENIC CARDIOMYOPATHY: MOLECULAR MECHANISMS Arrhythmogenic cardiomyopathy most commonly results from mutations in the genes encoding for desmosomal proteins and Ca2+ handling proteins in the sarcoplasmic reticulum. More about this image found in ARRHYTHMOGENIC CARDIOMYOPATHY: MOLECULAR MECHANISMS Arrhythmogenic cardiom...
Images
Published: 15 November 2023
ARRHYTHMOGENIC CARDIOMYOPATHY: INFLAMMATION Figure 17-12 ARRHYTHMOGENIC CARDIOMYOPATHY: INFLAMMATION Inflammation is frequently seen throughout the myocardium in arrhythmogenic cardiomyopathy and may be one of the inciting events for fibrofatty replacement. More about this image found in ARRHYTHMOGENIC CARDIOMYOPATHY: INFLAMMATION Inflammation is frequently see...
Images
Published: 15 November 2023
ARRHYTHMOGENIC CARDIOMYOPATHY Figure 17-8 ARRHYTHMOGENIC CARDIOMYOPATHY The fatty replacement is nearly transmural in this example. More about this image found in ARRHYTHMOGENIC CARDIOMYOPATHY The fatty replacement is nearly transmural i...
Images
Published: 15 November 2023
ARRHYTHMOGENIC CARDIOMYOPATHY Figure 17-9 ARRHYTHMOGENIC CARDIOMYOPATHY The admixture of fat and fibrosis throughout the wall is evident at high-power magnification. More about this image found in ARRHYTHMOGENIC CARDIOMYOPATHY The admixture of fat and fibrosis throughout...
Images
Published: 15 November 2023
ARRHYTHMOGENIC CARDIOMYOPATHY Figure 17-2 ARRHYTHMOGENIC CARDIOMYOPATHY Grossly, the anterior surface demonstrates prominent right ventricular (RV) dilatation with a fatty appearance. More about this image found in ARRHYTHMOGENIC CARDIOMYOPATHY Grossly, the anterior surface demonstrates p...
Images
Published: 15 November 2023
ARRHYTHMOGENIC CARDIOMYOPATHY Figure 17-7 ARRHYTHMOGENIC CARDIOMYOPATHY Prominent fatty replacement is seen at low-power magnification, with little remaining myocardium in this section. More about this image found in ARRHYTHMOGENIC CARDIOMYOPATHY Prominent fatty replacement is seen at low-p...
Images
Published: 15 November 2023
ARRHYTHMOGENIC CARDIOMYOPATHY Figure 17-5 ARRHYTHMOGENIC CARDIOMYOPATHY The fatty replacement of the right ventricular wall is transmural, with no visible muscle outside of the papillary muscles in this example. More about this image found in ARRHYTHMOGENIC CARDIOMYOPATHY The fatty replacement of the right ventricul...
Images
Published: 15 November 2023
ARRHYTHMOGENIC CARDIOMYOPATHY Figure 17-10 ARRHYTHMOGENIC CARDIOMYOPATHY A Masson trichrome stain highlights the degree to which fat and fibrosis play a role in replacing the involved myocardium. More about this image found in ARRHYTHMOGENIC CARDIOMYOPATHY A Masson trichrome stain highlights the degr...
Images
Published: 15 November 2023
ARRHYTHMOGENIC CARDIOMYOPATHY Figure 17-3 ARRHYTHMOGENIC CARDIOMYOPATHY In short-axis view, the right ventricle is markedly dilated, with extensive fatty replacement of the wall and focal aneurysm (yellow arrow) formation. More about this image found in ARRHYTHMOGENIC CARDIOMYOPATHY In short-axis view, the right ventricle is m...
Images
Published: 15 November 2023
ARRHYTHMOGENIC CARDIOMYOPATHY Figure 17-4 ARRHYTHMOGENIC CARDIOMYOPATHY In short-axis view, the right vetricle (RV) is replaced by fat, with just a small amount of grossly identifiable RV myocardium in the inferoseptal region. More about this image found in ARRHYTHMOGENIC CARDIOMYOPATHY In short-axis view, the right vetricle (RV) ...
Images
Published: 15 November 2023
ARRHYTHMOGENIC CARDIOMYOPATHY: LEFT VENTRICULAR INVOLVEMENT Figure 17-11 ARRHYTHMOGENIC CARDIOMYOPATHY: LEFT VENTRICULAR INVOLVEMENT Involvement of the left ventricle by fibrofatty replacement is seen, frequently involving the epicardial half of the ventricle first. More about this image found in ARRHYTHMOGENIC CARDIOMYOPATHY: LEFT VENTRICULAR INVOLVEMENT Involvement of...
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Published: 15 November 2023
ARRHYTHMOGENIC CARDIOMYOPATHY: LEFT VENTRICULAR INVOLVEMENT Figure 17-6 ARRHYTHMOGENIC CARDIOMYOPATHY: LEFT VENTRICULAR INVOLVEMENT In addition to the right ventricular involvement, the left ventricle is also involved by fibrofatty replacement, which is nearly transmural along the lateral wall. More about this image found in ARRHYTHMOGENIC CARDIOMYOPATHY: LEFT VENTRICULAR INVOLVEMENT In addition to...
Images
Published: 15 November 2023
UHL ANOMALY Figure 17-13 UHL ANOMALY The ventricular wall is devoid of muscle, showing endocardium (black arrow) directly opposed to epicardial fat. This complete lack of muscle in the wall is different than the pathology seen in arrhythmogenic cardiomyopathy. More about this image found in UHL ANOMALY The ventricular wall is devoid of muscle, showing endocardium ...
Book Chapter
Series: Atlases of Tumor and Non-Tumor Pathology, Series 5
Volume: 17
Published: 15 November 2023
10.55418/9781933477299-ch13
ISBN-10: 1-933477-29-6
ISBN: 978-1-933477-29-9
... b , NYHA Functional Class (D) Dilated (H) Hypertrophic (R) Restrictive (A) Arrhythmogenic cardiomyopathy (NC) Left ventric-noncompaction (E) Early, with type in parenthetical (NS) Nonspecific phenotype (NA) Information not available (0) Unaffected (H&R), (D+A), (H+D) Overlapping (H) Heart...
Book Chapter
Series: Atlases of Tumor and Non-Tumor Pathology, Series 5
Volume: 17
Published: 15 November 2023
10.55418/9781933477299-ch25
ISBN-10: 1-933477-29-6
ISBN: 978-1-933477-29-9
..., for example, ion channel disease, or by an arrhythmogenic focus located in the ventricles caused by underlying myocardial disease. Metabolic factors (especially acidosis and alterations in serum electrolytes) and drugs may lower the arrhythmogenic threshold for arrhythmogenic foci. In virtually all cases...
Book Chapter
Series: Atlases of Tumor and Non-Tumor Pathology, Series 5
Volume: 17
Published: 15 November 2023
10.55418/9781933477299-ch14
ISBN-10: 1-933477-29-6
ISBN: 978-1-933477-29-9
... cause familial dilated cardiomyopathy . J Am Coll Cardiol 2009 ; 54 : 930 - 41 . 43. van den Hoogenhof MM , Beqqali A , Amin AS , et al . RBM20 mutations induce an arrhythmogenic dilated cardiomyopathy related to disturbed calcium handling . Circulation 2018 ; 138 : 1330...
Book Chapter
Series: Atlases of Tumor and Non-Tumor Pathology, Series 5
Volume: 17
Published: 15 November 2023
10.55418/9781933477299-ch03
ISBN-10: 1-933477-29-6
ISBN: 978-1-933477-29-9
..., more sections may be taken. Examples include 1) myocarditis, which can be nonuniform in distribution and severity, 2) acute myocardial infarction, which may not involve the mid-ventricular level, and 3) arrhythmogenic cardiomyopathy, which most commonly affects the right ventricular free wall. Taking...
Book Chapter
Series: Atlases of Tumor and Non-Tumor Pathology, Series 5
Volume: 17
Published: 15 November 2023
10.55418/9781933477299-ch15
ISBN-10: 1-933477-29-6
ISBN: 978-1-933477-29-9
... , Bonow RO et al . Eligibility and disqualification recommendations for competitive athletes with cardiovascular abnormalities: task force 3: hypertrophic cardiomyopathy, arrhythmogenic right ventricular cardiomyopathy and other cardiomyopathies, and myocarditis: a scientific statement from...
Book Chapter
Series: Atlases of Tumor and Non-Tumor Pathology, Series 5
Volume: 17
Published: 15 November 2023
10.55418/9781933477299-ch21
ISBN-10: 1-933477-29-6
ISBN: 978-1-933477-29-9
... replacement in some areas (particularly the anterior portion and the acute margin) ( fig. 21-8 ). Such fatty replacement of the wall may mimic arrhythmogenic cardiomyopathy (ACM) (see chapter 17 ). Unlike ACM, this type of fatty change in obesity is not typically associated with significant interstitial...
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