• Jessica A Berger, Dana D Huh, Teresa Lee, Rajeev S Wadia, Melania M Bembea, and Dheeraj K Goswami.
• Department of Anesthesiology and Critical Care Medicine, Children's Hospital of Philadelphia, Philadelphia, PA, USA.
• Paediatr Anaesth. 2021 Jul 1; 31 (7): 820-826.

BackgroundMarfan 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.AimsOur 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.MethodsWe 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.ResultsIn 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.ConclusionsDespite 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.© 2021 The Authors. Pediatric Anesthesia published by John Wiley & Sons Ltd.

13 keywords...

hide…