Cardiovascular Magnetic Resonance Imaging clarifies cardiac pathophysiology in early, asymptomatic diffuse systemic
Sophie I Mavrogeni1, Konstantinos Bratis, Georgia Karabela
1Onassis Cardiac Surgery Center, 50 Esperou Street, 175 61 Palaio Faliro, Athens, Greece. soma13@otenet.gr.
Insights
Cardiovascular magnetic resonance (CMR) detects early heart disease in asymptomatic scleroderma (SSc) patients, revealing inflammation or reduced perfusion and fibrosis. This imaging can identify severe cardiac involvement before clinical symptoms arise.
Area of Science:
- Cardiology
- Radiology
- Rheumatology
Background:
- Scleroderma (SSc) commonly causes cardiac disease through myopericardial inflammation, perfusion defects, and fibrosis.
- Early identification of cardiac involvement in asymptomatic diffuse SSc is crucial for management.
Purpose of the Study:
- To investigate the utility of inflammation and stress perfusion-fibrosis cardiovascular magnetic resonance (CMR) in identifying cardiac pathophysiology in asymptomatic diffuse SSc.
- To differentiate cardiac involvement patterns in SSc using advanced CMR techniques.
Main Methods:
- 46 asymptomatic diffuse SSc patients underwent CMR on a 1.5T system.
- Protocols included T2 imaging, stress perfusion, and late gadolinium enhancement (LGE) based on initial T2 ratio.
- Results were compared to age/sex-matched controls and patients with coronary artery disease (CAD).
Main Results:
- Two patients showed acute myocardial inflammation. The remaining 44/46 had reduced Myocardial Perfusion Reserve Index (MPRI) compared to controls (p<0.001), similar to CAD.
- Fibrosis was diffuse and greater than controls, comparable to CAD.
- Two-year follow-up revealed further MPRI deterioration and diffuse subendocardial LGE in 8/11 patients, without changes in ventricular volumes or ejection fractions.
Conclusions:
- CMR can detect significant cardiac involvement in early, asymptomatic diffuse SSc, even with normal routine evaluations.
- Findings include myocardial inflammation or significant MPRI reduction and diffuse fibrosis.
- Long-term follow-up indicates progressive cardiac damage in SSc patients.
Background:
Myopericardial inflammation, perfusion's defects and fibrosis are major causes of cardiac disease in scleroderma (SSc). We hypothesized that using inflammation and stress perfusion-fibrosis cardiovascular magnetic resonance (CMR), we can identify the pathophysiology of heart disease in asymptomatic diffuse SSc.
Patients-Methods:
46 recently diagnosed, asymptomatic patients with diffuse SSc had a CMR examination using a 1.5T system. ECG gated breath hold cine and short tau inversion recovery (STIR) T2 images were initially acquired. If T2 ratio<2 a stress perfusion-fibrosis protocol was applied. If T2>2 a myocarditis protocol including early (EGE) and late (LGE) gadolinium imaging was applied. SSc patients' results were compared with age and sex-matched controls and patients with coronary artery disease (CAD).
Results:
In 2/46 SSc with T2 ratio>2, the myocarditis protocol was positive for acute myocardial inflammation, who developed clinical signs of acute myocarditis shortly after the CMR evaluation. In the rest 44/46 with T2 ratio<2 the stress perfusion-fibrosis CMR identified a significant reduction in Myocardial Perfusion Reserve Index (MPRI) compared with matched controls (0.6±0.4 vs 3.2±0.8, p<0.001), but not with CAD (0.6±0.4 vs 0.86±0.46, p=NS) and correlated only with the presence of digital ulcers (p<0.05). The scar was diffused and greater compared to controls, but did not differ from that assessed in CAD. Two years follow up, available in 11/44 SSc, showed further asymptomatic MPRI deterioration in all and diffuse subendocardial LGE in 8/11, without any change in LV, RV volumes and ejection fractions.
Conclusion:
CMR may reveal severe cardiac involvement in early, asymptomatic diffuse SSc with normal routine cardiac evaluation, presenting either as myocardial inflammation or as severe reduction of MPRI and diffuse fibrosis with further deterioration in the long term follow up.
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