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Increased Risk of Valvular Heart Disease in Systemic Sclerosis: An Underrecognized Cardiac Complication
Reto D Kurmann1, Edward A El-Am2, Yasser A Radwan3
1R.D. Kurmann, MD, Department of Cardiovascular Diseases, Mayo Clinic, Rochester, Minnesota, USA, and Department of Cardiology, Heart Center Lucerne Hospital, Lucerne, Switzerland.
Insights
Systemic sclerosis (SSc) patients show a fourfold higher prevalence and incidence of moderate/severe valvular heart disease (VHD) compared to non-SSc individuals. This highlights a significant association between SSc and VHD development.
Area of Science:
- Cardiology
- Rheumatology
- Systemic Autoimmune Diseases
Background:
- Cardiac involvement is a critical prognostic factor in systemic sclerosis (SSc).
- While myocardial fibrosis and arrhythmias are recognized, valvular heart disease (VHD) incidence in SSc is underreported.
- Understanding VHD prevalence is crucial for comprehensive SSc patient care.
Purpose of the Study:
- To determine the prevalence of VHD at SSc diagnosis.
- To assess the incidence of VHD during follow-up in SSc patients compared to non-SSc subjects.
- To investigate the association between SSc and VHD development.
Main Methods:
- Retrospective review of medical records for incident SSc cases.
- Matching of SSc subjects 1:2 by age and sex to non-SSc control subjects.
- Comparison of VHD prevalence at diagnosis and incidence over time.
Main Results:
- A nearly fourfold increase in moderate/severe VHD prevalence was observed in SSc patients at diagnosis (6% vs 0%).
- The cumulative incidence of VHD at 10 years was significantly higher in SSc patients (17.9%) versus non-SSc subjects (2.3%).
- Aortic stenosis and mitral regurgitation were more prevalent in SSc, alongside secondary tricuspid regurgitation.
Conclusions:
- SSc patients exhibit a fourfold higher prevalence of moderate/severe VHD at diagnosis.
- SSc patients face a fourfold increased risk of developing moderate/severe VHD post-diagnosis.
- Further research is needed to elucidate the underlying mechanisms connecting SSc and VHD.
Objective:
Cardiac involvement is a poor prognostic marker in systemic sclerosis (SSc). While diastolic dysfunction, myocardial fibrosis, and arrhythmias are traditionally considered features of primary cardiac involvement in SSc, the incidence of valvular heart disease (VHD) is not well reported. Our objective was to examine the prevalence of VHD at the time of SSc diagnosis and incidence of VHD during follow-up compared to non-SSc subjects.
Methods:
Medical records of patients with suspicion of SSc were reviewed to identify incident cases. SSc subjects were matched 1:2 by age and sex to non-SSc subjects.
Results:
The study included 78 incident SSc cases and 156 non-SSc comparators (56 yrs [± 15.7], 91% female). A nearly 4-fold increase in the prevalence of moderate/severe VHD prior to SSc diagnosis compared to non-SSc subjects (6% vs 0%; P = 0.004) was identified. During follow-up, 18 SSc and 12 non-SSc patients developed moderate/severe VHD. The cumulative incidence of VHD at 10 years after SSc incidence/index was 17.9% (95% CI 10.7-29.9) in patients with SSc compared with 2.3% (95% CI 0.7-7.0) in non-SSc subjects (HR 4.23, 95% CI 2.03-8.83). Coronary artery disease was the only significant risk factor for VHD.
Conclusion:
Patients with SSc have a 4-fold increase in the prevalence of moderate/severe VHD at diagnosis compared to non-SSc patients. They also have a 4-fold increased risk of developing moderate/severe VHD after diagnosis of SSc. Aortic stenosis and mitral regurgitation have a much higher prevalence in patients with SSc, besides secondary tricuspid regurgitation. Underlying mechanisms for this association require further elucidation.
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