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Emerging Insights into Granulomatous and Amyloidogenic Cardiomyopathies
Syed Bukhari1, Adnan Younus2, Zubair Bashir3
1Department of Medicine, Division of Cardiology, Johns Hopkins University School of Medicine, Baltimore, MD 21287, USA.
Insights
Advanced imaging aids early diagnosis of fatal cardiac amyloidosis and cardiac sarcoidosis. Prompt, tailored treatments significantly improve outcomes for these infiltrative cardiomyopathies.
Area of Science:
- Cardiology
- Cardiovascular Imaging
- Infiltrative Cardiomyopathies
Background:
- Cardiac amyloidosis and cardiac sarcoidosis are underdiagnosed, potentially fatal infiltrative cardiomyopathies.
- These conditions are cardiac manifestations of systemic diseases, increasingly recognized with imaging advancements and new therapies.
- Focus on these conditions has intensified for clinicians and researchers.
Purpose of the Study:
- To review clinical features, diagnostic techniques, and treatment strategies for cardiac amyloidosis and cardiac sarcoidosis.
- To highlight the role of advanced imaging in differentiating and managing these cardiomyopathies.
- To emphasize the impact of early diagnosis and treatment on patient outcomes.
Main Methods:
- Comprehensive literature review of studies since 1990 from PubMed and Scopus.
- Focus on studies relevant to echocardiography, cardiac magnetic resonance imaging (CMR), positron emission tomography (PET), and technetium-labeled nuclear scintigraphy.
- Inclusion of studies on treatment modalities for both conditions.
Main Results:
- Multimodality imaging, especially CMR, technetium-labeled nuclear scan, and PET, is crucial for early identification and differentiation.
- Distinct CMR enhancement patterns and morphological differences aid diagnosis.
- Technetium-labeled nuclear scintigraphy distinguishes cardiac amyloidosis subtypes; early diagnosis and treatment improve outcomes.
Conclusions:
- Multimodality imaging facilitates early detection of cardiac amyloidosis and cardiac sarcoidosis.
- Treatment strategies vary significantly: cardiac amyloidosis (subtype-specific therapies) vs. cardiac sarcoidosis (corticosteroids/immunosuppressants).
- Early, accurate diagnosis via advanced imaging is critical for improving patient outcomes.
Abstract:
Background: Granulomatous and amyloidogenic cardiomyopathies are infiltrative conditions that can be fatal if left untreated. Among these, cardiac amyloidosis and cardiac sarcoidosis are significant but often underdiagnosed causes of heart failure, each serving as cardiac manifestations of broader systemic diseases. Advancements in imaging techniques and the emergence of novel therapies-particularly for cardiac amyloidosis-have brought these conditions into sharper focus for both clinicians and researchers. Methods: We conducted a comprehensive review of the literature by searching databases including PubMed and Scopus for studies published since 1990 regarding clinical features, diagnostic techniques, and treatment strategies for cardiac amyloidosis and cardiac sarcoidosis. Studies were selected based on relevance to imaging methods, including echocardiography, cardiac magnetic resonance imaging (CMR), positron emission tomography (PET), and technetium-labeled nuclear scintigraphy, as well as treatment modalities for both conditions. Results: Imaging techniques, particularly CMR, technetium-labeled nuclear scan, and PET, were found to be crucial for the early identification and differentiation of cardiac amyloidosis and cardiac sarcoidosis. Distinct late gadolinium enhancement patterns were observed in CMR along with morphological differences, aiding in diagnosis. Technetium-labeled nuclear scintigraphy can definitively distinguish between subtypes of cardiac amyloidosis in the absence of paraproteinemia. Early diagnosis has been shown to significantly improve patient outcomes. Early treatment can reduce morbidity in both cardiomyopathies. Conclusions: Multimodality imaging can help in the early detection of cardiac amyloidosis and cardiac sarcoidosis. Treatment strategies differ substantially: cardiac amyloidosis is primarily managed with disease-modifying therapies for the transthyretin subtype and chemotherapy/stem cell transplant for the AL subtype, while cardiac sarcoidosis is treated with corticosteroids and immunosuppressive drugs to reduce inflammation. Early and accurate diagnosis through advanced imaging techniques is critical to improving outcomes for patients with these conditions.
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