Intramyocardial calcification in apical hypertrophic cardiomyopathy assessed using multimodality imaging: a case...

Area of Science:

• Cardiology
• Cardiovascular Imaging
• Pathology

Background:

• Apical hypertrophic cardiomyopathy (ApHCM) is a variant of hypertrophic cardiomyopathy characterized by apical obliteration and diastolic dysfunction.
• It frequently affects middle-aged males and can lead to microvascular ischemia.

Observation:

• This study reports five cases of ApHCM with evidence of intramyocardial calcification on echocardiography.
• Cardiac MRI revealed hypointensity on early gadolinium enhancement (EGE) sequences (suggesting calcium) and hyperintensity on late gadolinium enhancement (LGE) sequences (suggesting fibrosis).

Findings:

• The imaging findings suggest a potential diagnosis of endomyocardial fibrosis (EMF), characterized by scarring and dystrophic calcification.
• This series represents the largest description of ApHCM with these features to date.
• Coexistent inflammatory triggers were identified in all patients, suggesting a role in the development of small vessel disease.

Implications:

• The findings suggest a link between apical fibrosis/calcification and microvascular ischemia in ApHCM.
• Inflammatory comorbidities may exacerbate microvascular stress in the context of cardiac hypertrophy.
• This research highlights a potential pathway involving inflammation, hypertrophy, fibrosis, and calcification in ApHCM.