Increased Risk of Valvular Heart Disease in Systemic Sclerosis: An Underrecognized Cardiac Complication

Abstract

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 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 years (± 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: 0.7-6.3%) in non-SSc subjects (HR: 4.23; 95% CI: 2.03-8.83). Coronary heart disease was the only significant risk factor for VHD. Conclusion SSc patients 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 SSc patients, besides secondary tricuspid regurgitation. Underlying mechanisms for this association require further elucidation.

Increased Prevalence of Moderate to Severe Mitral and Aortic Valve Dysfunction in Systemic Sclerosis: A Case-control Study

“…Our results agree with the data provided by other population-based studies that have also analyzed the prevalence and/ or incidence of any degree of MAVD in patients with SSc 8,19,[21][22][23][24][25][26][27][28] . The main results of these studies are summarized in Table 5; three studies have not yet been published 25,26,27 .…”

“…Our results agree with the data provided by other population-based studies that have also analyzed the prevalence and/ or incidence of any degree of MAVD in patients with SSc 8,19,[21][22][23][24][25][26][27][28] . The main results of these studies are summarized in Table 5; three studies have not yet been published 25,26,27 . The great heterogeneity between the studies (discrepancies in study design; lack of control groups in some cases; selection biases, including only patients without clinical manifestations of heart failure; and great variability regarding disease duration) make their results difficult to compare.…”

“…The great heterogeneity between the studies (discrepancies in study design; lack of control groups in some cases; selection biases, including only patients without clinical manifestations of heart failure; and great variability regarding disease duration) make their results difficult to compare. However, although the prevalence rate of MAVD in SSc varies widely, in all cases in which it was compared to a control group, the rate was statistically higher in patients with SSc, with a 2-to 6-fold increase in prevalence compared to non-SSc subjects 8,19,[21][22][23][24][25][26][27] . MR was also by far the most common VHD found, followed by AS or AR 8,19,[21][22][23][24][25][26][27][28] .…”

“…However, although the prevalence rate of MAVD in SSc varies widely, in all cases in which it was compared to a control group, the rate was statistically higher in patients with SSc, with a 2-to 6-fold increase in prevalence compared to non-SSc subjects 8,19,[21][22][23][24][25][26][27] . MR was also by far the most common VHD found, followed by AS or AR 8,19,[21][22][23][24][25][26][27][28] . Most cases of AS presented with severe disease, whereas MR and AR cases were usually mild or moderate in severity.…”

Sudden Cardiac Death in Systemic Sclerosis: Diagnostics to Assess Risk and Inform Management

“…It is hypothesised that there is an increased frequency of valvular heart disease due to the effects of SSc itself; however, it remains unclear whether the observed increased rates of aortic and mitral valve disease seen in population-based studies [86] are attributable to the increased frequency of screening echocardiograms performed as part of routine SSc care. Case-control studies have suggested an increased frequency of moderate to severe aortic and mitral valvular disease in SSc [87,88]. However, valvular heart disease has not been linked with increased mortality [87] and how valvular pathology may impact SCD risk in SSc is unstudied.…”