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Updated: Aug 22, 2025
Cardiac Magnetic Resonance for the Evaluation of Suspected Cardiac Thrombus: Conventional and Emerging Techniques
Published on: June 11, 2019
Cardiovascular Magnetic Resonance Imaging Patterns in Rare Cardiovascular Diseases
George Markousis-Mavrogenis1, Aikaterini Giannakopoulou2, Antonios Belegrinos2
1Onassis Cardiac Surgery Center, 17674 Athens, Greece.
Insights
Cardiovascular Magnetic Resonance (CMR) aids in diagnosing rare cardiovascular diseases (RCDs) by assessing function, perfusion, and tissue characteristics. Advanced imaging techniques reveal specific tissue changes indicative of various RCDs.
Area of Science:
- Cardiology
- Radiology
- Medical Imaging
Background:
- Rare cardiovascular diseases (RCDs) present diagnostic challenges due to low incidence but significant clinical impact.
- A comprehensive classification system categorizes RCDs into eight classes based on affected circulation, pathology, or specific conditions.
- Cardiovascular Magnetic Resonance (CMR) offers a versatile, non-invasive imaging modality for RCD evaluation.
Purpose of the Study:
- To highlight the utility of Cardiovascular Magnetic Resonance (CMR) in the diagnosis and management of various rare cardiovascular diseases (RCDs).
- To correlate specific CMR findings, including tissue characterization techniques, with distinct RCDs.
Main Methods:
- Utilizing CMR for angiography, functional assessment, perfusion imaging, and advanced tissue characterization (T2 mapping, Late Gadolinium Enhancement - LGE, native T1 mapping).
- Analyzing patterns of edema, fibrosis (diffuse subendocardial, replacement), hypertrophy, and aortopathies specific to different RCDs.
- Correlating parametric imaging indices with specific disease entities.
Main Results:
- Edema (high T2 signal/mapping) indicates acute inflammation; diffuse subendocardial LGE suggests microvascular disease (e.g., systemic sclerosis, amyloidosis).
- Replacement LGE in the inferolateral wall points to neuromuscular disorders; patchy LGE with edema is typical of myocarditis.
- Specific LGE patterns, hypertrophy, and T1 mapping changes differentiate hypertrophic cardiomyopathy (HCM), cardiac amyloidosis (CA), and Anderson-Fabry Disease (AFD).
- Magnetic resonance angiography detects aortopathies (e.g., Marfan syndrome); RV LGE is characteristic of arrhythmogenic cardiomyopathy (ARVC).
Conclusions:
- CMR, through its comprehensive imaging capabilities, is crucial for diagnosing and managing a spectrum of rare cardiovascular diseases.
- Parametric imaging indices derived from CMR are essential for detecting subtle tissue changes and enabling accurate RCD diagnosis.
- CMR facilitates the differentiation of various RCDs by identifying characteristic patterns of edema, fibrosis, and hypertrophy.
Abstract:
Rare cardiovascular diseases (RCDs) have low incidence but major clinical impact. RCDs' classification includes Class I-systemic circulation, Class II-pulmonary circulation, Class III-cardiomyopathies, Class IV-congenital cardiovascular diseases (CVD), Class V-cardiac tumors and CVD in malignancy, Class VI-cardiac arrhythmogenic disorders, Class VII-CVD in pregnancy, Class VIII-unclassified rare CVD. Cardiovascular Magnetic Resonance (CMR) is useful in the diagnosis/management of RCDs, as it performs angiography, function, perfusion, and tissue characterization in the same examination. Edema expressed as a high signal in STIRT2 or increased T2 mapping is common in acute/active inflammatory states. Diffuse subendocardial fibrosis, expressed as diffuse late gadolinium enhancement (LGE), is characteristic of microvascular disease as in systemic sclerosis, small vessel vasculitis, cardiac amyloidosis, and metabolic disorders. Replacement fibrosis, expressed as LGE, in the inferolateral wall of the left ventricle (LV) is typical of neuromuscular disorders. Patchy LGE with concurrent edema is typical of myocarditis, irrespective of the cause. Cardiac hypertrophy is characteristic in hypertrophic cardiomyopathy (HCM), cardiac amyloidosis (CA) and Anderson-Fabry Disease (AFD), but LGE is located in the IVS, subendocardium and lateral wall in HCM, CA and AFD, respectively. Native T1 mapping is increased in HCM and CA and reduced in AFD. Magnetic resonance angiography provides information on aortopathies, such as Marfan, Turner syndrome and Takayasu vasculitis. LGE in the right ventricle is the typical finding of ARVC, but it may involve LV, leading to the diagnosis of arrhythmogenic cardiomyopathy. Tissue changes in RCDs may be detected only through parametric imaging indices.
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