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

Abstract

Objective To investigate the prevalence, severity and associated clinical factors of mitral and aortic valvular involvement in patients with systemic sclerosis (SSc). Methods This case–control study included 172 patients with SSc and 172 non-SSc adults without known cardiac disease matched by age, gender, and prevalence of cardiovascular risk factors. The screening of mitral and aortic valvular involvement was performed by transthoracic Doppler echocardiogram. The prevalence of aortic stenosis (AS) was also compared with that reported in a population-based study performed in our community during the same period. Results SSc patients showed an almost 5-fold increased prevalence of moderate-to-severe mitroaortic valve dysfunction compared to non-SSc controls (OR: 4.60, 95% CI 1.51–13.98; p=0.003). The most common lesion was mitral regurgitation (MR), which was observed in 5.2% of patients, followed by AS in 3.5%, and aortic regurgitation (AR) in 1.7%. Analyzing the different types of valvular lesion separately, we observed a significantly higher frequency of MR compared to controls (OR:4.69, 95% CI:1.12–22.04; p= 0.032), as well as a higher frequency of AS in the 65-75 (OR: 7.51, 95% CI:1.22–46.23, p=0.01) and 76-85 year-old age groups (OR:3.53, 95% CI:1.03–12.22, p=0.043) when compared to the general population in our community. Conclusion We found an increased prevalence of moderate-to-severe MR and AS in SSc compared to age-matched non-SSc controls with similar cardiovascular comorbidities. While results from this study do not allow to establish a direct causal relationship, they strongly support

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.…”

“…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.…”

Aortic stenosis is independently associated with male gender, obesity renal failure, COPD, Caucasians and numerous inflammatory diseases in addition to known cardiovascular risk factors

“…No meaningful associations were found between AS and psoriatic arthritis, Buerger's disease, gout, ankylosing spondylitis, or antiphospholipid antibody syndrome. Some of the inflammatory conditions in this study have been suggested to have associations with AS in very small studies or case reports, including rheumatoid arthritis [34], mixed connective tissue disease [35], and scleroderma (systemic sclerosis) [36] . For others, such as polyarteritis nodosa and Raynaud's syndrome, this is the first known identification of a relationship.…”

A Rare Presentation of Systemic Sclerosis

“…Another case-control study investigating the prevalence of MAVD in patients with SSc revealed that SSc patients have a significantly higher chance of experiencing moderate to severe MAVD compared to those without SSc, with a prevalence almost five times greater (OR 4.60) with MR being the most common lesion followed by AS and AR. On the other hand, the development of mitral stenosis is less frequent [ 6 ]. Aortic incompetence was documented with lesser frequency in the available literature.…”

“…Aortic incompetence was documented with lesser frequency in the available literature. Narváez et al observed a notably higher occurrence of AR in individuals with SSc compared to control subjects [ 6 ]. Additionally, Fernández-Codina et al established a significant connection between AR and the limited skin subset of SSc [ 7 ].…”