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Spectrum of Restrictive and Infiltrative Cardiomyopathies: Part 2 of a 2-Part Series
Naveen L Pereira1, Martha Grogan1, G William Dec2
1Department of Cardiovascular Diseases, Mayo Clinic, Rochester, Minnesota.
Journal of the American College of Cardiology
|March 10, 2018
Summary
Restrictive cardiomyopathies, a rare heart muscle disease, are increasingly diagnosed in elderly patients with preserved ejection fraction, especially cardiac amyloidosis. Early diagnosis through noninvasive methods improves outcomes with targeted therapies.
Area of Science:
- Cardiology
- Cardiovascular Medicine
- Heart Muscle Diseases
Background:
- Restrictive cardiomyopathies represent the least common category of heart muscle diseases.
- These conditions are classified as infiltrative, noninfiltrative, storage diseases, or endomyocardial disorders.
- While genetic forms often manifest in childhood, elderly individuals with heart failure with preserved ejection fraction are increasingly diagnosed with restrictive cardiomyopathy, notably cardiac amyloidosis.
Purpose of the Study:
- To review the diagnostic approaches and therapeutic implications of restrictive cardiomyopathies.
- To highlight the evolving recognition of restrictive cardiomyopathies in the elderly population.
- To emphasize the shift towards noninvasive diagnostic modalities.
Main Methods:
- Review of current literature on restrictive cardiomyopathies.
- Analysis of diagnostic criteria and imaging techniques.
- Evaluation of treatment strategies for various etiologies.
Main Results:
- Noninvasive evaluation is now the primary diagnostic tool, largely supplanting endomyocardial biopsy.
- Cardiac amyloidosis is a significant cause of restrictive cardiomyopathy in older adults with preserved ejection fraction.
- Early detection of infiltrative cardiomyopathies, including iron overload and sarcoidosis, enables disease-specific treatments.
Conclusions:
- Restrictive cardiomyopathies, though rare, require careful diagnosis, particularly in the elderly.
- Noninvasive imaging plays a crucial role in identifying infiltrative cardiomyopathies.
- Timely, etiology-specific therapies can lead to improved patient outcomes.
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