Department of Anesthesiology and Critical Care Medicine, Children's Hospital of Philadelphia, Philadelphia, PA, USA.
Johns Hopkins University School of Medicine, Baltimore, MD, USA.
Paediatr Anaesth. 2021 Jul;31(7):820-826. doi: 10.1111/pan.14196. Epub 2021 May 13.
Marfan syndrome and Loeys-Dietz syndrome are connective tissue disorders associated with cardiac and vascular disease. Patients often require surgical repair, but limited data exist to describe their perioperative management.
Our goals were to review the perioperative features of patients with Marfan and Loeys-Dietz syndrome that may affect anesthesia care and to describe the differences in preoperative clinical characteristics and intra-operative anesthetic management.
We conducted a retrospective cohort study of patients with Marfan and Loeys-Dietz syndrome who underwent cardiac surgery at a single institution. We collected demographic and perioperative data from the electronic medical record and performed descriptive statistics to characterize the patient populations and describe their anesthetic management.
In 71 patients (40 Marfan, 31 Loeys-Dietz), we found significant differences between the Marfan and Loeys-Dietz patients in airway difficulty, preoperative weight, blood utilization, valvular disease, and age at first cardiac surgery. Patients with Loeys-Dietz syndrome had higher preoperative rates of severe noncardiac comorbidities, including gastroesophageal reflux and asthma that required chronic medical therapy.
Despite undergoing similar surgical procedures, patients with Marfan and Loeys-Dietz syndrome have different intrinsic patient characteristics and comorbidities that may affect their perioperative care. This retrospective cohort study identified some factors, but additional collection and reporting of patient data based on multicenter experience are essential for the ongoing optimization of perioperative care in these patient populations.
马凡综合征和洛伊茨-迪茨综合征是与心脏和血管疾病相关的结缔组织疾病。患者通常需要手术修复，但有关围手术期管理的数据有限。
我们的目标是回顾马凡综合征和洛伊茨-迪茨综合征患者的围手术期特征，这些特征可能会影响麻醉护理，并描述术前临床特征和术中麻醉管理的差异。
我们对在一家机构接受心脏手术的马凡综合征和洛伊茨-迪茨综合征患者进行了回顾性队列研究。我们从电子病历中收集了人口统计学和围手术期数据，并进行了描述性统计，以描述患者人群并描述其麻醉管理。
在 71 名患者（40 名马凡综合征，31 名洛伊茨-迪茨综合征）中，我们发现马凡综合征和洛伊茨-迪茨综合征患者在气道困难、术前体重、血液利用、瓣膜疾病和首次心脏手术年龄方面存在显著差异。洛伊茨-迪茨综合征患者术前严重非心脏合并症的发生率较高，包括需要慢性药物治疗的胃食管反流和哮喘。
尽管接受了类似的手术程序，但马凡综合征和洛伊茨-迪茨综合征患者具有不同的固有患者特征和合并症，这可能会影响他们的围手术期护理。这项回顾性队列研究确定了一些因素，但需要根据多中心经验进一步收集和报告患者数据，以持续优化这些患者群体的围手术期护理。
