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Article Abstract
Background: Calcific aortic stenosis is increasingly recognized as a major determinant of mortality in the aging subset of patients with the ultra-rare Hutchinson-Gilford progeria syndrome (HGPS), particularly as survival improves with lonafarnib therapy. However, the optimal treatment strategy for severe aortic stenosis in this population remains undefined.
Case Summary: We report the first successful case of combined surgical aortic valve replacement and coronary artery bypass grafting in a severely symptomatic 21-year-old man with HGPS, severe calcific aortic stenosis, and coronary artery disease.
Discussion: Despite anatomical and procedural challenges, surgical valve replacement represents a reasonable treatment option in patients with HGPS when transcatheter or alternative approaches are not feasible. A multidisciplinary approach is essential to optimize outcomes in this high-risk population.
Take-home Message: Surgical aortic valve replacement may be considered among the potential treatment options for selected patients with HGPS.
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| http://www.ncbi.nlm.nih.gov/pmc/articles/PMC12402367 | PMC |
| http://dx.doi.org/10.1016/j.jaccas.2025.104823 | DOI Listing |
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