Intensive Care Unit Analgosedation After Cardiac Surgery in Children with Williams Syndrome : a Matched Case-Control Study.

OBJECTIVE: Cardiovascular abnormalities are common in patients with Williams syndrome 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 during the perioperative period. Despite this risk, the overall postoperative analgosedative requirements in patients with WS in the cardiac intensive care unit 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 frequency 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 (48 h and first 6 postoperative days), there were no differences between groups in total doses of morphine equivalents (5.0 mg/kg vs 5.6 mg/kg, p = 0.7 and 8.2 mg/kg vs 10.0 mg/kg, p = 0.7), midazolam equivalents (1.8 mg/kg vs 1.5 mg/kg, p = 0.4 and 3.4 mg/kg vs 3.8 mg/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 frequency of major ACE or mortality. CONCLUSIONS: Patients with WS received similar analgosedative medication doses compared with controls. There was no significant difference in the frequency of major ACE (including cardiac arrest, extracorporeal membrane oxygenation, and surgical re-intervention) or mortality between the two groups, though these findings must be interpreted with caution. Further investigation is necessary to elucidate the adequacy of pain/sedation control, factors that might affect analgosedative needs in this unique population, and the impact on clinical outcomes.

Postoperative Acute Kidney Injury in Williams Syndrome Compared With Matched Controls.

OBJECTIVES: Cardiovascular manifestations occur in over 80% of Williams syndrome (WS) patients and are the leading cause of morbidity and mortality. One-third of patients require cardiovascular surgery. Renal artery stenosis (RAS) is common in WS. No studies have assessed postoperative cardiac surgery-related acute kidney injury (CS-AKI) in WS. Our objectives were to assess if WS patients have higher risk of CS-AKI postoperatively than matched controls and if RAS could contribute to CS-AKI. DESIGN: This was a retrospective study of all patients with WS who underwent cardiac surgery at our center from 2010 to 2020. The WS study cohort was compared with a group of controls matched for age, sex, weight, and surgical procedure. SETTING: Patients underwent cardiac surgery and postoperative care at Lucile Packard Children's Hospital Stanford. PATIENTS: There were 27 WS patients and 43 controls (31% vs 42% female; p = 0.36). Median age was 1.8 years (interquartile range [IQR], 0.7-3.8 yr) for WS and 1.7 years (IQR, 0.8-3.1 yr) for controls. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: Postoperative hemodynamics, vasopressor, total volume input, diuretic administration, and urine output were collected in the first 72 hours. Laboratory studies were collected at 8-hour intervals. Multivariable analysis identified predictors of CS-AKI.Controlled for renal perfusion pressure (RPP) and vasoactive inotrope score (VIS), compared with controls, the odds ratio (OR) of CS-AKI in WS was 4.2 (95% CI, 1.1-16; p = 0.034). Higher RPP at postoperative hours 9-16 was associated with decreased OR of CS-AKI (0.88 [0.8-0.96]; p = 0.004). Increased VIS at hour 6 was associated with an increased OR of CS-AKI (1.47 [1.14-1.9]; p = 0.003). Younger age was associated with an increased OR of CS-AKI (1.9 [1.13-3.17]; p = 0.015). CONCLUSIONS: The OR of CS-AKI is increased in pediatric patients with WS compared with controls. CS-AKI was associated with VIS at the sixth postoperative hour. Increases in RPP and mean arterial pressure were associated with decreased odds of CS-AKI.

The Impact of Massage and Reading on Children's Pain and Anxiety After Cardiovascular Surgery: A Pilot Study.

OBJECTIVES: The purpose of this pilot study was three-fold: 1) to evaluate the safety and feasibility of instituting massage therapy in the immediate postoperative period after congenital heart surgery, 2) to examine the preliminary results on effects of massage therapy versus standard of care plus three reading visits on postoperative pain and anxiety, and 3) to evaluate preliminary effects of opioid and benzodiazepine exposure in patients receiving massage therapy compared with reading controls. DESIGN: Prospective, randomized controlled trial. SETTING: An academic children's hospital. SUBJECTS: Sixty pediatric heart surgery patients between ages 6 and 18 years. INTERVENTIONS: Massage therapy and reading. MEASUREMENT AND MAIN RESULTS: There were no adverse events related to massage or reading interventions in either group. Our investigation found no statistically significant difference in Pain or State-Trait Anxiety scores in the initial 24 hours after heart surgery (T1) and within 48 hours of transfer to the acute care unit (T2) after controlling for age, gender, and Risk Adjustment for Congenital Heart Surgery 1 score. However, children receiving massage therapy had significantly lower State-Trait Anxiety scores after receiving massage therapy at time of discharge (T3; p = 0.0075) than children receiving standard of care plus three reading visits. We found no difference in total opioid exposure during the first 3 postoperative days between groups (median [interquartile range], 0.80 mg/kg morphine equivalents [0.29-10.60] vs 1.13 mg/kg morphine equivalents [0.72-6.14]). In contrast, children receiving massage therapy had significantly lower total benzodiazepine exposure in the immediate 3 days following heart surgery (median [interquartile range], 0.002 mg/kg lorazepam equivalents [0-0.03] vs 0.03 mg/kg lorazepam equivalents [0.02-0.09], p = 0.0253, Wilcoxon rank-sum) and number of benzodiazepine PRN doses (0.5 [0-2.5] PRN vs 2 PRNs (1-4); p = 0.00346, Wilcoxon rank-sum). CONCLUSIONS: Our pilot study demonstrated the safety and feasibility of implementing massage therapy in the immediate postoperative period in pediatric heart surgery patients. We found decreased State-Trait Anxiety scores at discharge and lower total exposure to benzodiazepines. Preventing postoperative complications such as delirium through nonpharmacologic interventions warrants further evaluation.