Although cardiovascular involvement is a well-known complication correlated with a poor prognosis in patients with systemic sclerosis, there are few reports on valvular heart disease. Forty patients with systemic sclerosis were retrospectively analyzed. Valvular heart disease was found in six patients, five of whom had severe tri-leaflet aortic stenosis. Three of these 5 patients were ≤71 years old. Two frail elderly patients who underwent transcatheter aortic valve replacement died within two years.
Premature-onset aortic stenosis is not uncommon in patients with systemic sclerosis. When considering mechanical intervention, the evaluation of frailty is important.
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