Intensive Care Unit Analgosedation after Cardiac Surgery in Children with Williams Syndrome – a Matched Case-Control Study

Abstract

Abstract Objective: Cardiovascular abnormalities are common in patients with Williams syndrome (WS) and frequently require surgical intervention necessitating analgesia and sedation in a population with a unique neuropsychiatric profile, potentially increasing the risk of adverse cardiac events (ACE) during the perioperative period. Despite this risk, the overall postoperative analgosedative requirements in patients with WS in the cardiac intensive care unit (CICU) have not yet been investigated. Our primary aim was to examine the analgosedative requirement in patients with WS after cardiac surgery compared to a control group. Our secondary aim was to compare the risk of major ACE and mortality between the two groups. Design: Matched case-control study. Setting: Pediatric CICU at a Tertiary Children’s Hospital. Patients: Patients with WS and age-matched controls who underwent cardiac surgery and were admitted to the CICU after cardiac surgery between July 2014 and January 2021. Interventions: None Measurements and Main Results: Postoperative outcomes and total doses of analgosedative medications were collected in the first six days after surgery for the study groups. Median age was 29.8 (12.4-70.8) months for WS and 23.5 (11.2-42.3) months for controls. Across all study intervals (48h and first 6 post-operative days), there were no differences between groups in total doses of morphine equivalents (5.0 mg/kg vs 5.6mg/kg, p=0.7 and 8.2mg/kg vs 10.0 mg/kg, p=0.7), midazolam equivalents (1.8mg/kg vs 1.5mg/kg, p=0.4 and 3.4 mg/kg vs 3.8mg/kg, p=0.4), or dexmedetomidine (20.5 mcg/kg vs 24.4 mcg/kg, p=0.5 and 42.3 mcg/kg vs 39.1 mcg/kg, p=0.3). There was no difference in major ACE or mortality. Conclusions: Patients with WS received similar analgosedative medication doses compared with controls. There was no difference in the frequency of major ACE (including cardiac arrest, extracorporeal membrane oxygenation, and surgical reintervention) or mortality between the two groups. Patients with WS can receive similar analgosedation to controls without untoward cardiovascular events.