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Surgical Aortic Valve Replacement Combined With Coronary Artery Bypass Grafting in a Patient With Progeria
Simon Anquetil1, Mathieu Bignon2, Emré Belli3
1Department of Pediatric Cardiology, CHU de Caen, Caen, France.
Insights
Surgical aortic valve replacement is a viable option for Hutchinson-Gilford progeria syndrome patients with severe aortic stenosis. This multidisciplinary approach addresses complex cardiac conditions in this rare, aging population.
Area of Science:
- Cardiology
- Genetics
- Surgical Innovation
Background:
- Hutchinson-Gilford progeria syndrome (HGPS) patients face increased mortality from calcific aortic stenosis.
- Improved survival with lonafarnib therapy highlights the need for managing age-related comorbidities in HGPS.
Observation:
- A 21-year-old male with HGPS presented with severe symptomatic calcific aortic stenosis and coronary artery disease.
- This case represents the first successful combined surgical aortic valve replacement and coronary artery bypass grafting in an HGPS patient.
Findings:
- Surgical aortic valve replacement, despite anatomical and procedural challenges, proved to be a feasible treatment.
- A multidisciplinary team successfully managed this high-risk patient.
Implications:
- Surgical valve replacement should be considered for HGPS patients when less invasive options are unsuitable.
- Optimizing outcomes in this rare population necessitates a collaborative, specialized approach.
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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