Cardiovascular Magnetic Resonance Demonstrates Myocardial Inflammation of Differing Etiologies and Acuities in
George Markousis-Mavrogenis1,2, Antonios Belegrinos3, Aikaterini Giannakopoulou4
1Olympic Diagnostic/Research Center, 17674 Athens, Greece.
Insights
Cardiovascular magnetic resonance (CMR) reveals acute myocardial inflammation in most symptomatic inflammatory myopathy patients with normal echocardiography. Genetic myopathies show rare acute inflammation but evidence of chronic changes.
Area of Science:
- Cardiology
- Neurology
- Medical Imaging
Background:
- Myopathies are complex neuromuscular disorders affecting cardiac and skeletal muscles.
- Cardiac involvement is a concern in myopathies, necessitating advanced diagnostic tools.
- Echocardiography may not detect subtle cardiac changes in myopathy patients.
Purpose of the Study:
- To investigate the prevalence of cardiac inflammation in myopathy patients with cardiovascular symptoms and normal echocardiography.
- To utilize cardiovascular magnetic resonance (CMR) for detailed cardiac assessment.
- To differentiate cardiac inflammation patterns between genetic and inflammatory myopathies.
Main Methods:
- Prospective evaluation of 51 patients with genetic (n=23) and inflammatory (n=28) myopathies.
- Comparison of CMR findings (LGE, T1/T2 mapping, ECV) with age- and sex-matched controls.
- Application of updated Lake Louise criteria for myocardial inflammation assessment.
Main Results:
- Genetic myopathy patients showed higher LGE, native T1, ECV, and T2 mapping values than controls, indicating chronic inflammation.
- Inflammatory myopathy patients exhibited significantly elevated CMR tissue characterization indices, suggesting acute myocardial inflammation.
- CMR effectively differentiated between genetic and inflammatory myopathies with high sensitivity and specificity (AUC=0.9557).
Conclusions:
- Symptomatic inflammatory myopathy patients with normal echocardiography frequently present with acute myocardial inflammation.
- Genetic myopathies are characterized by chronic, low-grade myocardial inflammation rather than acute inflammatory processes.
- CMR is a valuable tool for detecting and characterizing cardiac involvement in various myopathies.
Introduction:
Myopathies are heterogeneous neuromuscular diseases of genetic and/or inflammatory etiology that affect both cardiac and skeletal muscle. We investigated the prevalence of cardiac inflammation in patients with myopathies, cardiovascular symptoms, and normal echocardiography using cardiovascular magnetic resonance (CMR).
Methods:
We prospectively evaluated 51 patients with various genetic (n = 23) and inflammatory (n = 28) myopathies (median age, IQR: 12 (11-15) years, 22% girls; 61 (55-65) years, 46% women, respectively) and compared their CMR findings to corresponding age- and sex-matched controls (n = 21 and 20, respectively) and to each other.
Results:
Patients with genetic myopathy had similar biventricular morphology and function to healthy controls but showed higher late gadolinium enhancement (LGE), native T1 mapping, extracellular volume fraction (ECV), and T2 mapping values. Collectively, 22 (95.7%) patients with genetic myopathy had a positive T1-criterion and 3 (13.0%) had a positive T2-criterion according to the updated Lake Louise criteria. Compared with healthy controls, patients with inflammatory myopathy showed preserved left ventricular (LV) function and reduced LV mass, while all CMR-derived tissue characterization indices were significantly higher (p < 0.001 for all). All patients had a positive T1-criterion, and 27 (96.4%) had a positive T2-criterion. A positive T2-criterion or T2-mapping > 50 ms could discriminate between patients with genetic and inflammatory myopathies with a sensitivity of 96.4% and a specificity of 91.3% (AUC = 0.9557).
Conclusions:
The vast majority of symptomatic patients with inflammatory myopathies and normal echocardiography show evidence of acute myocardial inflammation. In contrast, acute inflammation is rare in patients with genetic myopathies, who show evidence of chronic low-grade inflammation.
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