Perioperative management and considerations in pediatric patients with connective tissue disorders undergoing cardiac surgery

Abstract

Background 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. Aims 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. Methods 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. Results 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. Conclusions 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.

The Mystery of Ehlers-Danlos Syndrome: An Autobiographical Case Report

“…There are cases of patients with MFS and LDS who undergo evaluation for GERD, but these are secondary to a structural abnormality (such as a hiatal hernia which is more commonly seen in these disorders) [ 7 , 8 ]. GERD has been a well-characterized manifestation of hypermobility EDS (hEDS), upwards of 50% in some studies [ 9 ].…”