From the Departments of Anesthesiology, Critical Care and Pain Medicine.
Cardiovascular Surgery.
A A Pract. 2024 Aug 23;18(8):e01842. doi: 10.1213/XAA.0000000000001842. eCollection 2024 Aug 1.
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.
哈钦森-吉尔福德早衰综合征(HGPS)是一种罕见的节段性早发性衰老疾病,与动脉粥样硬化的发展以及严重的心血管和脑血管疾病有关。使用 lonafarnib 治疗可改善 HGPS 患者的生存率;然而,在长期随访中,进展性主动脉瓣钙化狭窄的发病率增加。HGPS 的不断发展促使人们重新考虑保守治疗,并制定了解剖治疗策略。在本病例报告中,我们描述了接受体外循环下主动脉瓣狭窄手术治疗的 HGPS 患者的麻醉管理。