Immunologic regulator and effector functions in perimyocarditis, postmyocarditic heart muscle disease and dilated cardiomyopathy

Author(s):  
Bernhard Maisch
2000 ◽  
Vol 48 (5) ◽  
pp. 579-584 ◽  
Author(s):  
Hongyi Zhang ◽  
Yanwen Li ◽  
Tianqing Peng ◽  
Malen Aasa ◽  
Li Zhang ◽  
...  

SUMMARY The association of enterovirus infection and heart muscle diseases has been investigated extensively by detection of viral genomic RNA using nucleic acid hybridization and the reverse transcription-polymerase chain reaction. To further understand the role of enterovirus and its persistence in these diseases, an immunohistochemical technique was optimized to investigate the expression of viral capsid proteins in situ. A monoclonal antibody (5-D8/1) against an epitope in the N-terminus of capsid protein VP1, conserved in the enterovirus genus, was employed. To enhance sensitivity, the EnVison system was used to detect antigen-antibody complex. VP1 was detected in formalin-fixed, paraffin-embedded endomyocardial biopsy or postmortem myocardial tissues and in liver, spleen, lung, kidney, and pancreas from patients with myocarditis or dilated cardiomyopathy, but not from controls. VP1 was localized in cytoplasm of myofibers, often adjacent to necrosis and infiltrate in myocarditis, and was clustered or scattered in dilated cardiomyopathy. This technique can be used for a definitive laboratory diagnosis of enterovirus-associated diseases and for studying the mechanisms of virus persistence in chronic myocardial disease.


2020 ◽  
Vol 41 (Supplement_2) ◽  
Author(s):  
A Protonotarios ◽  
D Cannie ◽  
M Akhtar ◽  
K Savvatis ◽  
N Sekhri ◽  
...  

Abstract Introduction Desmoplakin (DSP) gene mutations are reported to cause arrhythmogenic right ventricular cardiomyopathy (ARVC), but recent evidence suggests that they manifest as a much broader range of phenotypes. Purpose To describe the phenotypic characteristics of DSP mutation carriers in a consecutive cohort of patients with heart muscle disease. Methods A retrospective analysis of consecutive patients with heart muscle disease and their relatives that underwent diagnostic or research genetic testing. Only DSP variants classified as pathogenic by the American College of Medical Genetics and Genomics criteria were considered. Dilated cardiomyopathy (DCM), hypokinetic non-dilated cardiomyopathy (HNDC) and ARVC were diagnosed in accordance with current criteria. Results 109 mutation carriers were identified: 34 (31%) had DCM and 23 (21%) HNDC. ARVC diagnosis was borderline and definite in 39 (36%) and 39 (36%) individuals, respectively. Sixteen (15%) presented with clinically suspected myocarditis. 23 patients met both definite AC and DCM criteria and 13 patients met definite AC and HNDC criteria. In 87 patients with cardiac magnetic resonance imaging, a spectrum of disease was observed (Table) including an intermediate phenotype characterised by subepicardial LV fibrosis in the absence of ventricular dilatation or dysfunction. Ventricular dysfunction was manifest as left dominant disease (n=22, 48%) and biventricular disease (n=24, 52%); none had right dominant disease. Conclusions DSP mutations are associated with left and biventricular phenotypes and can present as myocarditis. Current ARVC and DCM diagnostic criteria inadequately describe the clinical subtypes of DSP related disease. A novel approach to disease classification is proposed. Funding Acknowledgement Type of funding source: Foundation. Main funding source(s): British Heart Foundation


2020 ◽  
Vol 9 (11) ◽  
pp. 3385
Author(s):  
Marco Merlo ◽  
Antonio Cannatà ◽  
Gianfranco Sinagra

Dilated Cardiomyopathy (DCM) has a straightforward and apparently “simple” definition: a heart muscle disease characterized by left ventricular (LV) or biventricular dilation and systolic dysfunction in the absence of either pressure or volume overload or coronary artery disease sufficient enough to explain the dysfunction [...]


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