Adipositas Cordis: A Rare and Poorly Understood Cardiomyopathy

Area of Science:

• Cardiology
• Pathology

Background:

• Adipositas cordis is a rare cardiomyopathy defined by diffuse fatty infiltration of the ventricular myocardium or interventricular septum.
• It is distinct from arrhythmogenic right ventricular cardiomyopathy as it does not involve myocardial cell destruction.

Observation:

• A 40-year-old obese woman presented with a II/VI systolic murmur, exacerbated by standing.
• Transthoracic echocardiography revealed interventricular septal hypertrophy with preserved left ventricular ejection fraction.
• Cardiac MRI identified a fatty mass within the interventricular septum.

Findings:

• Endomyocardial biopsy confirmed structurally normal myocytes with diffuse adipose cell infiltration, excluding malignancy.
• Comprehensive cardiac catheterization and stress echocardiography yielded normal results.

Implications:

• This case underscores the importance of a broad differential diagnosis for rare cardiac diseases.
• It demonstrates the significant role of noninvasive imaging modalities in guiding clinical decision-making for complex cases.