Altered patterns of cardiac intercellular junction distribution in hypertrophic cardiomyopathy.
Key result
Hypertrophic cardiomyopathy hearts with myofibre disarray showed marked disorganisation of intercalated discs and random gap junction distribution compared to normal and diseased control hearts.
Authors
Discussion
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Implication
Disorganised intercalated discs may promote arrhythmias in HCM; hypothesis-generating and should not yet change practice.
Key Points
- To investigate the spatial distribution of desmosomes and gap junctions in hypertrophic cardiomyopathy heart tissue exhibiting myofibre disarray.
- Examined tissue sections from six necropsied hypertrophic cardiomyopathy hearts across the interventricular septum and left and right ventricular free walls, compared against normal and disease controls.
- Performed immunohistochemical staining targeting desmoplakin for desmosomes and connexin43 for gap junctions, evaluated via confocal laser scanning microscopy.
- Areas with myofibre disarray displayed severe desmosome disorganization, including abnormally enlarged megadiscs, intersecting disc configurations, and aberrant side-to-side connections.
- Connexin43 gap junctions were displaced from intercalated discs, distributing randomly across myocyte surfaces and forming side-to-side links as well as continuous circular cell-to-cell loops.
- Structural junction abnormalities were absent in normal control hearts, disease controls, and regions of hypertrophic cardiomyopathy hearts lacking myofibre disarray.
Study Design
Type
Observational (n=6)
PICO
P
Population
Hypertrophic cardiomyopathy (n=6)
O
Primary Outcome
Distribution pattern of intercellular junctions (desmosomes and gap junctions)
Cite This Study
Sepp et al. (1996) conducted an observational in Hypertrophic cardiomyopathy (n=6). Hypertrophic cardiomyopathy hearts with myofibre disarray showed marked disorganisation of intercalated discs and random gap junction distribution compared to normal and diseased control hearts.