Perioperative morbidity in children with elastin arteriopathy

Area of Science:

• Cardiology
• Pediatric Anesthesiology
• Genetics

Background:

• Elastin arteriopathy (EA), often associated with Williams-Beuren syndrome, poses a significant risk of sudden death in children.
• Previous reports indicate a high risk of perioperative cardiac arrest and death in these patients, but cohort data on anesthesia-related morbidity and mortality are lacking.

Purpose of the Study:

• To determine the institutional incidence of morbidity and mortality in pediatric patients with EA undergoing anesthesia.
• To identify patient-specific risk factors associated with adverse perioperative outcomes in this population.

Main Methods:

• Retrospective review of medical records for children with EA who received anesthesia or sedation between 1990 and 2013.
• Analysis of cardiovascular hemodynamic indices from recent cardiac catheterization or echocardiography.
• Examination of intraoperative and early postoperative (up to 48 hours) complications, including cardiac arrest and cardiovascular events.

Main Results:

• A total of 141 anesthetics were administered to 48 patients with EA.
• Seven cardiac arrests (5% of anesthetics) and nine additional intraoperative cardiovascular complications (6% of anesthetics) occurred.
• All patients experiencing cardiac arrest or complications were under 3 years old and had biventricular outflow tract obstruction (BVOTO).
• Subgroup analysis revealed high cardiac arrest rates in children with BVOTO (44%) and those younger than 3 years (21%).
• No perioperative deaths were recorded; extracorporeal life support was used in five cases.

Conclusions:

• The study confirms a significantly elevated rate of cardiac arrest and complications in children with EA during anesthesia.
• Younger age (<3 years) and the presence of biventricular outflow tract obstruction (BVOTO) are identified as the primary risk factors for adverse events in this cohort.