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Hutchinson-Gilford Progeria Syndrome (HGPS) is an ultrarare disorder of segmental premature aging that is associated with the development of advanced atherosclerosis and significant cardiovascular and cerebrovascular disease. Treatment with lonafarnib has improved survival in patients with HGPS; however, in extended longitudinal follow-up, there has been an increase in the prevalence of rapidly progressive calcific aortic stenosis. The evolving course of HGPS has prompted reconsideration of conservative management and led to the development of strategies for anatomic treatment. In this case report, we describe the anesthetic management of patients with HGPS undergoing surgical management of aortic stenosis with cardiopulmonary bypass.
Zaleski et al. (Thu,) studied this question.