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Perioperative morbidity in children with elastin arteriopathy
Author(s) -
Latham Gregory J.,
Ross Faith J.,
Eisses Michael J.,
Richards Michael J.,
Geiduschek Jeremy M.,
Joffe Denise C.
Publication year - 2016
Publication title -
pediatric anesthesia
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.704
H-Index - 82
eISSN - 1460-9592
pISSN - 1155-5645
DOI - 10.1111/pan.12967
Subject(s) - medicine , perioperative , anesthesia , cardiac catheterization , complication , anesthesiology , extracorporeal membrane oxygenation , mortality rate , incidence (geometry) , sedation , cohort , surgery , physics , optics
Summary Background Children with elastin arteriopathy ( EA ), the majority of whom have Williams–Beuren syndrome, are at high risk for sudden death. Case reports suggest that the risk of perioperative cardiac arrest and death is high, but none have reported the frequency or risk factors for morbidity and mortality in an entire cohort of children with EA undergoing anesthesia. Aim The aim of this study was to present one institution's rate of morbidity and mortality in all children with EA undergoing anesthesia and to examine patient characteristics that pose the greatest risk. Methods We reviewed medical records of children with EA who underwent anesthesia or sedation for any procedure at our institution from 1990 to 2013. Cardiovascular hemodynamic indices from recent cardiac catheterization or echocardiography were tabulated for each child. The incidence, type, and associated factors of complications occurring intraoperatively through 48 h postoperatively were examined. Results Forty‐eight patients with confirmed EA underwent a total of 141 anesthetics. There were seven cardiac arrests (15% of patients, 5% of anesthetics) and nine additional intraoperative cardiovascular complications (15% of patients, 6% of anesthetics). Extracorporeal life support was initiated in five cases. There were no perioperative deaths. All children having a cardiac arrest or complication were <3 years old and had biventricular outflow tract obstruction ( BVOTO ). Subgroup analysis demonstrated high rates of cardiac arrest in two groups: children with BVOTO (44%) and age <3 years old (21%). Conclusions We have confirmed that the rate of cardiac arrest and complications is significantly elevated in children with EA undergoing anesthesia. Children <3 years old and with BVOTO were at the greatest risk in our population.