AI Article Synopsis

  • Hutchinson-Gilford progeria syndrome is a rare genetic disorder that causes premature aging in children and leads to severe health issues, primarily cardiovascular problems.
  • Most patients are managed conservatively when they have severe aortic valve stenosis, a common complication.
  • This study presents the first successful case of a 23-year-old patient with the syndrome who received a transcatheter aortic valve replacement, showing a new treatment approach.

Article Abstract

Hutchinson-Gilford progeria syndrome is an autosomal dominant, rare, fatal pediatric segmental premature aging disease. Cardiovascular and cerebrovascular diseases constitute the major cause of morbidity and mortality. Patients with the syndrome and severe aortic valve stenosis have been described in the literature, and for all of them a strategy of conservative management has been followed. We describe the first successful treatment of a 23-year-old Hutchinson-Gilford progeria syndrome patient with severe aortic stenosis who underwent transapical transcatheter aortic valve replacement.

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http://dx.doi.org/10.1016/j.athoracsur.2020.03.067DOI Listing

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