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Background: Mortality of newborns with Hypoplastic Left Heart Syndrome (HLHS) is mainly concentrated after Norwood procedure (NP) stage 1 palliation (S1P) and between S1P and stage 2 palliation (S2P). Standardized management of these patients may help to control hospital mortality. Aim of the study was to evaluate the impact on hospital mortality of a standardized perioperative management (SPM) for newborns requiring S1P in a low volume center for NP., Methods: A consecutive series of patients undergoing S1P from January 1, 2002 to December 31, 2006 were retrospectively compared, by a "before and after" design, with those receiving a SPM (i.e. use of selective cerebral perfusion, near infrared spectroscopy, delayed sternal closure, modified ultrafiltration) from January 1 st , 2007 to December 31 st , 2018. Demographic, intraoperative and postoperative characteristics were collected. Univariate and multivariate analyses assessed differences before and after SPM., Results: Ninety-one newborns underwent S1P in the considered period; of 74 eligible patients, 25 did not receive SPM, while 49 received SPM. Hospital mortality after S1P was 31% (CI 21-44%). The introduction of a SPM did not affect hospital mortality both at the univariate-(28% vs. 29%, P=0.959) and at the multivariate analysis (HR 1.85, P=0.62). Mortality was 12% (CI 6-25%) between hospital discharge after S1P and S2P and 8% (CI 3-22%) between S2P and S3P., Conclusions: The use of a SPM for HLHS newborns requiring S1P was not effective in reducing hospital mortality in a low volume center. We suggest a collaboration between Italian Pediatric Cardiac Centers to manage HLHS patients.

Background: Ventilator-associated pneumonia (VAP) is a serious complication in children after cardiac surgery that may result from micro-aspiration. However, the current recommendation to use cuffed tracheal tubes (TTs) versus uncuffed TTs in children is still uncertain. Our main aim was to evaluate the incidence of VAP, ventilator-associated tracheobronchitis (VAT) and ventilator-associated conditions (VAC) in children up to five years old who underwent elective cardiac surgery., Methods: Single-center, prospective before-and-after study at a tertiary pediatric intensive care unit (PICU) in Italy. 242 patients (121 in each group) through the following periods: phase I (from Jan 2017 to 20 th Feb 2018), during which children were intubated with uncuffed TTs; phase II (from 21 th Feb 2018 to Feb 2019), during which children were intubated with cuffed TTs., Results: Data were collected using an electronic dedicated database. Median age was five months. The use of cuffed tubes reduced the risk of VAC and VAP respectively 15.8 times (95% CI 3.4-73.1, P=0.0008) and 14.8 times (95% CI 3.1-71.5, P=0.002). No major related airway complications were observed in the cuffed TTs group. Average treatment effect, calculated after propensity score matching, confirmed the significant effect of cuffed TTs on VAC and VAP., Conclusions: Our study suggests a marked reduction of VAP and VAC associated with use of a cuffed versus uncuffed TT in infants and children ≤5 years of age after elective cardiac surgery. A randomized clinical trial is needed to confirm these results and define the impact of use of a cuffed versus uncuffed TT across other relevant ICU outcomes and non-cardiac PICU patients.

Background: Despite significant improvements in surgical techniques and medical care, thrombotic complications still represent the primary cause of early graft failure and re-transplantation following paediatric liver transplantation. There is still no standardized approach for thrombosis prevention., Materials and Methods: The study aimed to evaluate the effectiveness of early intravenous unfractionated heparin started 12 hours postoperatively at 10 UI/kg per hour and used a retrospective "before and after" design to compare the incidence of early thrombotic complications prior to (2002-2010) and after (2011-2016) the introduction of heparin in our institute., Results: From 2002 to 2016, 479 paediatric patients received liver transplantation in our institution with an overall survival rate over one year of 0.91 (95% CI: 0.87-0.94). Of 365 eligible patients, 244 did not receive heparin while 121 did receive heparin. We reported a lower incidence of venous thrombosis (VT) in the group treated with heparin: 2.5% (3/121) vs 7.9% (19/244) (p=0.038). All clinical and laboratory variables considered potential risk factors for VT were studied. By multivariate stepwise Cox proportional hazards models, heparin prophylaxis resulted significantly associated to a reduction in VT (HR=0.29 [95% CI: 0.08-0.97], p=0.045), while age <1 year was found to be an independent risk factor for VT (HR=2.62 [95% CI: 1.11-6.21]; p=0.028)., Discussion: Early postoperative heparin could be considered a valid and safe strategy to prevent early VT after paediatric liver transplantation without a concomitant increase in bleeding. A future randomised control trial is mandatory in order to strengthen this conclusion.

Objectives: We sought to compare gas exchange, respiratory mechanics, and asynchronies during pressure support ventilation (PSV), sigh adjunct to PSV (PSV SIGH), and neurally adjusted ventilatory assist (NAVA) in hypoxemic infants after cardiac surgery., Design: Prospective, single-center, crossover, randomized physiologic study., Setting: Tertiary-care pediatric intensive care unit., Patients: Fourteen hypoxemic infants (median age 11.5 days [8.7-74])., Interventions: The protocol begins with a 1 hour step of PSV, followed by two consecutive steps in PSV SIGH and NAVA in random order, with a washout period of 30 minutes (PSV) between them., Main Results: Three infants presented an irregular Eadi signal because of diaphragmatic paralysis and were excluded from analysis. For the remaining 11 infants, PaO 2 /FiO 2 and oxygenation index improved in PSV SIGH compared with PSV (P < 0.05) but not in NAVA compared with PSV. PSV SIGH showed increased tidal volumes and lower respiratory rate than PSV (P < 0.05), as well as a significant improvement in compliance with respiratory system indexed to body weight when compared with both PSV and NAVA (P < 0.01). No changes in mean airway pressure was registered among steps. Inspiratory time resulted prolonged for both PSV SIGH and NAVA than PSV (P < 0.05). NAVA showed the higher coefficient of variability in respiratory parameters and a significative decrease in asynchrony index when compared with both PSV and PSV SIGH (P < 0.01)., Conclusions: The adjunct of one SIGH per minute to PSV improved oxygenation and lung mechanics while NAVA provided the best patient-ventilator synchrony in infants after cardiac surgery., (© 2019 Wiley Periodicals, Inc.)