Systemic sclerosis linked to four-fold increase in valvular heart disease prevalence
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Patients with systemic sclerosis demonstrate a four-fold increased prevalence of moderate-to-severe valvular heart disease at diagnosis compared with those without systemic sclerosis, according to data published in The Journal of Rheumatology.
“Cardiopulmonary complications are the leading cause of mortality in systemic sclerosis, with up to 70% mortality at 5 years reported with heart involvement,” Ashima Makol, MD, director of the scleroderma/Raynaud’s and nailfold capillaroscopy clinic at the Mayo Clinic, in Rochester, Minnesota, told Healio Rheumatology. “In a recently published study from my group, we showed that patients with SSc are at a greater than two-fold increased risk for experiencing a cardiovascular event compared with persons without SSc.”
According to Makol, this risk is underestimated four- to five-fold by traditional risk scoring systems, such as the Framingham Risk Score and American College of Cardiology/American Heart Association score.
“Currently, however, there is no gold standard definition of heart involvement in scleroderma, and this is an area of high interest among SSc experts,” she added. “In general, myocardial fibrosis has been considered to be the hallmark of cardiac involvement in SSc, clinically characterized by myocarditis, congestive heart failure, diastolic dysfunction, conduction defects and arrythmias. Valvular heart disease, while reported in autopsy and small echocardiographic studies, has not been considered a major primary cardiac manifestation of SSc and data on the incidence and prevalence of VHD in SSc is scarce.”
To analyze the prevalence of valvular heart disease at SSc diagnosis, as well as its incidence during follow-up, compared with individuals without SSc, Makol and colleagues conducted a retrospective study of residents of Olmsted County, Minnesota. The researchers reviewed the medical records of all adults with a diagnosis, or suspicion, of SSc in the county from Jan. 1, 1980, to Dec. 31, 2016.
Clinical data were collected from the Rochester Epidemiology Project, a medical record system that links together all medical records for Olmsted County residents from multiple health care providers. Patients with other rheumatologic diseases were excluded from the study. For the comparator cohort, the researchers selected a random collection from the same population of Olmsted County residents. The included patients with SSc and comparator individuals were then matched 1:2 based on age and sex. In all, the study included 78 patients with incident SSc and 156 individuals without SSc.
According to the researchers, there was a nearly four-fold increase in the prevalence of moderate-to-severe valvular heart disease prior to SSc diagnosis, compared with those without SSc — or 6% versus 0% (P = .004). Later, during follow-up, 18 patients with SSc and 12 individuals from the comparator cohort developed moderate-to-severe valvular heart disease.
The cumulative incidence of valvular heart disease at 10 years after SSc incidence or index date was 17.9% (95% CI, 10.7-29.9) in patients with SSc, compared with 2.3% (95% CI, 0.7–7.0) among those without SSc (HR = 4.23; 95% CI, 2.03-8.83). Coronary artery disease was the sole significant risk factor for valvular heart disease, the researchers wrote.
“In accordance with recently published data from a Danish nationwide cohort and a Swedish study, our findings illustrate that valvular heart disease develops at high frequency during the natural history of disease and should be regarded as a primary SSc‐related cardiac complication,” Makol said.
“Although the mechanisms of this complication warrant further study, given the high prevalence of valvular heart disease at baseline, we recommend all patients with SSc undergo echocardiography at diagnosis, not only to screen for pulmonary arterial hypertension but also to assess for valvular dysfunction,” she added. “Given the high cardiovascular morbidity in SSc, early detection can allow us to improve quality of life and longevity in patients with cardiac involvement in scleroderma.”
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