Leslie B Gordon, Sammy Basso, Justine Maestranzi, Elena Aikawa, Cassandra L Clift, Antonio Giovanni Cammardella, Tommaso Hinna Danesi, Pedro J Del Nido, Elazer R Edelman, Abeer Hamdy, Sheila M Hegde, Monica E Kleinman, Nicola Maschietto, Mandeep R Mehra, Srinivasan Mukundan, Francesco Musumeci, Marco Russo, Frank J Rybicki, Pinak Bipin Shah, William A Suarez, Kelsey Tuminelli, Katherine Zaleski, Ashwin Prakash, Marie Gerhard-Herman
Hutchinson-Gilford Progeria Syndrome (HGPS) is an ultra-rare genetic premature aging disease that is historically fatal in teenage years, secondary to severe accelerated atherosclerosis. The only approved treatment is the farnesyltransferase inhibitor lonafarnib, which improves vascular structure and function, extending average untreated lifespan of 14.5 years by 4.3 years (30%). With this longer lifespan, calcific aortic stenosis (AS) was identified as an emerging critical risk factor for cardiac death in older patients...
2024: Frontiers in Cardiovascular Medicine