Intramyocardial calcification in apical hypertrophic cardiomyopathy assessed using multimodality imaging: a case series.
ESC Heart Fail
; 11(4): 2415-2420, 2024 Aug.
Article
in En
| MEDLINE
| ID: mdl-38634252
ABSTRACT
Apical hypertrophic cardiomyopathy (ApHCM) is an HCM variant, affecting frequently males in midlife. It is characterized by apical obliteration and persistent diastolic contraction, often resulting in microvascular ischaemia. We report five cases of ApHCM, with evidence of intramyocardial calcification on echocardiogram. On cardiac magnetic imaging (MRI), a hypointense component at early gadolinium enhancement (EGE) sequences, compatible with calcium, and a deep layer, with hyperintensity at late gadolinium enhancement (LGE) sequences, referable to fibrosis, suggest an endomyocardial fibrosis (EMF) diagnosis. EMF pathologic hallmark is endocardium and myocardium scarring, evolving to dystrophic calcification. It is found only in few ApHCM patients. Our series is the largest one described until now. Analysing patients' history, coexistent inflammatory triggers were evident in all of them, so their co-morbidities could represent a further cause of small vessel disease, in the context of ischaemic microvascular stress due to hypertrophy, leading to fibrosis and dystrophic calcification. This series could demonstrate the relation between apical fibrosis/calcification and microvascular ischaemia due to hypertrophy and inflammatory triggers.
Key words
Full text:
1
Collection:
01-internacional
Database:
MEDLINE
Main subject:
Cardiomyopathy, Hypertrophic
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Calcinosis
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Magnetic Resonance Imaging, Cine
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Multimodal Imaging
Limits:
Adult
/
Aged
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Female
/
Humans
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Male
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Middle aged
Language:
En
Journal:
ESC Heart Fail
Year:
2024
Document type:
Article
Affiliation country: