Browsing by Author "Zhang, Wenying"

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    Exome sequencing identified a novel HIST1H1E heterozygous protein-truncating variant in a 6-month-old male patient with Rahman syndrome: A case report
    (Wiley, 2022-02-07) Indugula, Subba Rao; Ayala, Sofia Saenz; Vetrini, Francesco; Belonis, Alyce; Zhang, Wenying; Medical and Molecular Genetics, School of Medicine
    Rahman syndrome is a rare congenital anomaly syndrome recently described, which results from pathogenic variants in the HIST1H1E gene. The condition is characterized by variable somatic overgrowth, macrocephaly, distinctive facial features, intellectual disability, and behavioral problems. This report extends the genotype and clinical phenotype of HIST1H1E-associated Rahman syndrome.
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    Influence of Early Extubation Location on Outcomes Following Pediatric Cardiac Surgery
    (Wolters Kluwer, 2020-10) Rooney, Sydney R.; Mastropietro, Christopher M.; Benneyworth, Brian; Graham, Eric M.; Klugman, Darren; Costello, John; Ghanayem, Nancy; Zhang, Wenying; Banerjee, Mousumi; Gaies, Michael; Pediatrics, School of Medicine
    Objectives: Early extubation following pediatric cardiac surgery is common, but debate exists whether location affects outcome, with some centers performing routine early extubations in the operating room (odds ratio) and others in the cardiac ICU. We aimed to define early extubation practice variation across hospitals and assess impact of location on hospital length-of-stay and other outcomes. Design: Secondary analysis of the Pediatric Cardiac Critical Care Consortium registry. Setting: Twenty-eight Pediatric Cardiac Critical Care Consortium hospitals. Patients: Patients undergoing Society of Thoracic Surgeons-European Association for Cardio-Thoracic Surgery mortality category 1-3 operations between August 2014 and February 2018. Interventions: None. Measurements and main results: We defined early extubation as extubation less than 6 hours after postoperative admission. Hospitals were categorized based on the proportion of their early extubation patients who underwent an odds ratio extubation. Categories included low- (< 50% of early extubation, n = 12), medium- (50%-90%, n = 8), or high- (> 90%, n = 8) frequency odds ratio early extubation centers. The primary outcome of interest was postoperative hospital length-of-stay. We analyzed 16,594 operations (9,143 early extubation, 55%). Rates of early extubation ranged from 16% to 100% across hospitals. Odds ratio early extubation rates varied from 16% to 99%. Patient characteristics were similar across hospital odds ratio early extubation categories. Early extubation rates paralleled the hospital odds ratio early extubation rates-77% patients underwent early extubation at high-frequency odds ratio extubation centers compared with 39% at low-frequency odds ratio extubation centers (p < 0.001). High- and low-frequency odds ratio early extubation hospitals had similar length-of-stay, cardiac arrest rates, and low mortality. However, high-frequency odds ratio early extubation hospitals used more noninvasive ventilation than low-frequency hospitals (15% vs. 9%; p < 0.01), but had fewer extubation failures (3.6% vs. 4.5%; p = 0.02). Conclusions: Considerable variability exists in early extubation practices after low- and moderate-complexity pediatric cardiac surgery. In this patient population, hospital length-of-stay did not differ significantly between centers with different early extubation strategies based on location or frequency.
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    Variation in extubation failure rates after neonatal congenital heart surgery across Pediatric Cardiac Critical Care Consortium hospitals
    (Elsevier, 2017-06) Benneyworth, Brian D.; Mastropietro, Christopher W.; Graham, Eric M.; Klugman, Darren; Costello, John M.; Zhang, Wenying; Gaies, Michael; Pediatrics, School of Medicine
    OBJECTIVE: In a multicenter cohort of neonates recovering from cardiac surgery, we sought to describe the epidemiology of extubation failure and its variability across centers, identify risk factors, and determine its impact on outcomes. METHODS: We analyzed prospectively collected clinical registry data on all neonates undergoing cardiac surgery in the Pediatric Cardiac Critical Care Consortium database from October 2013 to July 2015. Extubation failure was defined as reintubation less than 72 hours after the first planned extubation. Risk factors were identified using multivariable logistic regression with generalized estimating equations to account for within-center correlation. RESULTS: The cohort included 899 neonates from 14 Pediatric Cardiac Critical Care Consortium centers; 14% were premature, 20% had genetic abnormalities, 18% had major extracardiac anomalies, and 74% underwent surgery with cardiopulmonary bypass. Extubation failure occurred in 103 neonates (11%), within 24 hours in 61%. Unadjusted rates of extubation failure ranged from 5% to 22% across centers; this variability was unchanged after adjusting for procedural complexity and airway anomaly. After multivariable analysis, only airway anomaly was identified as an independent risk factor for extubation failure (odds ratio, 3.1; 95% confidence interval, 1.4-6.7; P = .01). Neonates who failed extubation had a greater median postoperative length of stay (33 vs 23 days, P < .001) and in-hospital mortality (8% vs 2%, P = .002). CONCLUSIONS: This multicenter study showed that 11% of neonates recovering from cardiac surgery fail initial postoperative extubation. Only congenital airway anomaly was independently associated with extubation failure. We observed a 4-fold variation in extubation failure rates across hospitals, suggesting a role for collaborative quality improvement to optimize outcomes.