Abstract
BACKGROUND: Pediatric cardiomyopathy is rare with an estimated annualized incidence of just over 1 per 100 000 children. However, many of these children have systemic disorders underlying their cardiomyopathy, including RASopathies, muscular dystrophies, and inborn errors of metabolism, and thus may require noncardiac procedural interventions or diagnostic studies. There is a paucity of data about the rate and nature of periprocedural complications and outcomes in this patient population. AIMS: This study aims to describe children with cardiomyopathy undergoing procedures or diagnostic studies requiring anesthesia, and their perioperative outcomes. METHODS: We performed a single-center retrospective cohort study using each procedural event as the unit of analysis. Perioperative (intraoperative and 72 h postoperatively) data and outcomes were recorded. Logistic regression was used to identify risk factors for adverse events. RESULTS: We identified 98 patients who underwent a total of 231 procedures. There were 26 total adverse events (11%), including four major adverse events (involving three distinct patients). ASA-PS class 4 status was associated with an increased likelihood of any adverse event, and all the patients who experienced major adverse events had moderate or greater left ventricular dysfunction. CONCLUSIONS: Anesthesia in children with cardiomyopathy is associated with a low (< 2%), but notable, rate of major adverse events. The risk of major adverse events appears to be highest in children with marked left ventricular dysfunction and was often associated with respiratory events in our cohort.