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This review article seeks to discuss the salient information about the known genetic variants associated with congenital long QT syndrome (LQTS) and describe the current anesthetic considerations. LQTS describes a heterogeneous group of patients with varying genetic mutations that cause an increased risk of torsade de pointe and sudden death. Research continues to identify more genetic mutations associated with LQTS subtypes. Publications of clinical experience with LQTS patients under general anesthesia provide new insights on the risk of medication inducing arrhythmias in these patients. Many medications used during general anesthesia are known to prolong the QTc interval. Patients with LQTS are at increased risk of arrhythmias under general anesthesia.

Background The goal of preoperative fasting is to prevent pulmonary aspiration during general anesthesia. Fasting times are often prolonged leading to patient discomfort and risk for adverse events. This retrospective quality improvement survey evaluated effective nil-per-os (NPO) times and causes for prolonged NPO times with the aim to suggest improvement strategies by a newly founded fasting task force. Methods Data from all electronic anesthesia records from 2019 at our institution were reviewed for fasting times. Our NPO instructions follow American Society of Anesthesiology guidelines and are calculated based on the patient's arrival time (90 min before operating room [OR] time). Primary outcome was the effective NPO time for clear liquids, secondary outcomes were incidence of delays and the parental compliance with the NPO instructions. Data are presented as median (interquartile range). Results In total 9,625 cases were included in the analysis. NPO time was documented in 72.1% with a median effective NPO time of 7:13 h (7:36). OR in room times were documented in 72.8%, 2,075 (29.5%; median time 0:10 h [0:21]) were earlier and 4,939 (70.5%; median time 0:29 h [0:54]) were later than scheduled. Parental NPO compliance showed a median deviation for clear liquid intake of 0:55 h (8:30). Conclusions This study revealed that effective NPO times were longer than current ASA guidelines. Contributing causes include case delays and parental non-compliance to NPO instructions. Thus, task force recommendations include change NPO instruction calculations to scheduled OR time versus arrival time, and encourage parents to give their child clear liquids at the instructed time.

Introduction Transfusion protocols are not well-studied for pediatric patients with acute liver failure (ALF). This study evaluates the utility of an international normalized ratio (INR)-based transfusion threshold for these patients. Methods Forty-four ALF pediatric patients from 2009 to 2018 were reviewed and divided into two groups: (a) a threshold group including patients between 2009 and 2015 who were transfused for an INR above 3.0, per institutional policy (n = 30), and (b) a post-threshold group including patients after 2015 through 2018 who were transfused based on clinical judgment (n = 14). Preoperative INRs, preoperative transfusions, intraoperative transfusions, early reoperation, renal function, graft function and deaths were compared. Results Liver failure severity was similar between threshold and post-threshold groups. Threshold patients had a lower average INR prior to transplantation, 2.8 (range 1.8-3.8) vs 4.4 (range 2.1-9.0), respectively (P = .01). Twenty-six threshold patients (87%) received preoperative FFP compared with seven post-threshold patients (50%, P = .0088). Two threshold patients (7%) received preoperative cryoprecipitate compared with five post-threshold patients (36%, P = .014). The incidence of pre-transplant bleeding, operative transfusions, and 1-year patient and graft survival did not differ significantly. Conclusion Clinical judgment vs an INR-based threshold for transfusions did not increase perioperative complications in children with ALF.

SummaryBackground Combined heart and liver transplantation (CHLT) in the pediatric population involves a complex group of patients, many of whom have palliated congenital heart disease (CHD) involving single ventricle physiology. Objective The purpose of this study was to describe the perioperative management of pediatric patients undergoing CHLT at a single institution and to identify management strategies that may be used to optimize perioperative care. Methods We did a retrospective database review of all patients receiving CHLT at a children's hospital between 2006 and 2014. Information collected included preoperative characteristics, intraoperative management, blood transfusions, and postoperative morbidity and mortality. Results Five pediatric CHLTs were performed over an 8-year period. All patients had a history of complex CHD with multiple sternotomies, three of whom had failing Fontan physiology. Patient age ranged from 7 to 23 years and weight from 29.5 to 68.5 kg. All CHLTs were performed using an en-bloc technique where both the donor heart and liver were implanted together on cardiopulmonary bypass (CPB). The median operating room time was 14.25 h, median CPB time was 3.58 h, and median donor ischemia time was 4.13 h. Patients separated from CPB on dopamine, epinephrine, and milrinone infusions and two required inhaled nitric oxide. All patients received a massive intraoperative blood transfusion post CPB with amounts ranging from one to three times the patient's estimated blood volume. The patient who required the most transfusions was in decompensated heart and liver failure preoperatively. Four of the five patients received an antifibrinolytic agent as well as a procoagulant (prothrombin complex concentrate or recombinant activated Factor VII) to assist with hemostasis. There were no 30-day thromboembolic events detected. Postoperatively the median length of mechanical ventilation, ICU stay and stay to hospital discharge was 4, 8, and 37 days, respectively. All patients are alive and free from allograft rejection at this time. Conclusion Combined heart and liver transplantation in the pediatric population involves a complex group of patients with unique perioperative challenges. Successful management starts with thorough preoperative planning and communication and involves strategies to deal with massive intraoperative hemorrhage and coagulopathy in addition to protecting and supporting the transplanted heart and liver and meticulous surgical technique. An integrated multidisciplinary team approach is the cornerstone for successful outcomes.

Background Accurate measurement of intraoperative blood loss is an important clinical variable in managing fluid resuscitation and avoiding unnecessary transfusion of blood products. In this study, we measured surgical blood loss using a tablet computer programmed with a unique algorithm modeled after facial recognition technology. The aim of the study was to assess the accuracy and performance of the system on surgical laparotomy sponges in vitro. Methods Whole blood samples of premeasured hemoglobin (Hb) and volume were reconstituted from units of human packed red blood cells and plasma and distributed across surgical laparotomy sponges. Normal saline was added to simulate the presence of varying levels of hemodilution and/or irrigation use. Soaked sponges from 4 different manufacturers were scanned using the Triton System with Feature Extraction Technology (Gauss Surgical, Inc., Palo Alto, CA) under 3 different ambient light conditions in an operating room. Accuracy of Hb loss measurement was evaluated relative to the premeasured values using linear regression and Bland-Altman analysis. Correlations between studied variables and measurement bias were analyzed using nonparametric tests. Results The overall mean percent error for measure of Hb loss for the Triton System was 12.3% (95% confidence interval [CI], 8.2%-16.4%). A strong positive linear correlation between the premeasured and actual Hb masses was noted across the full range of intraoperative lighting conditions, including (A) high (r = 0.95 [95% CI, 0.93-0.96]), (B) medium (r = 0.94 [95% CI, 0.93-0.96]), and (C) low (r = 0.90 [95% CI, 0.87-0.93]) mean ambient light intensity. Bland-Altman analysis revealed a bias of 0.01 g [95% CI, -0.03 to 0.06 g] of Hb per sponge between the 2 measures. The corresponding lower and upper limits of agreement were -1.16 g (95% CI, -1.21 to -1.12 g) per sponge and 1.19 g (95% CI, 1.15-1.24 g) per sponge, respectively. Measurement bias of estimated blood loss and Hb mass using the new system were not associated with the volume of saline used to reconstitute the samples (P = 0.506 and P = 0.469, respectively), suggesting that the system is robust under a wide range of sponge saturation conditions. Conclusions Mobile blood loss monitoring using the Triton system is accurate in assessing Hb mass on surgical sponges across a range of ambient light conditions, sponge saturation, saline contamination, and initial blood Hb. Utilization of this tool could significantly improve the accuracy of blood loss estimates.

Prolonged fetal tracheal occlusion (TO) accelerates lung growth but leads to loss of alveolar epithelial type II (AE2) cells. In contrast, temporary TO leads to recovery of AE2 cells and their ability to produce surfactant. The aim of this study was to determine the effects of temporary TO in fetal sheep with lung hypoplasia on postnatal lung function, structure, and surfactant protein mRNA expression. Diaphragmatic hernia (DH) was created in 22 fetal sheep at 65 days of gestation. TO was performed between 110 days of gestation and full term (DH/TO, n = 7) and between 110 and 130 days of gestation (DH/TO+R, n = 6). Sham-operated fetuses ( n = 11) served as controls. Lambs were delivered at ∼139 days of gestation, and blood gas tensions were monitored over a 2-h resuscitation period. Temporary TO increased growth of the hypoplastic lung and restored surfactant protein mRNA expression and AE2 cell density but did not improve respiratory function above that of animals that underwent prolonged TO; DH/TO and DH/TO+R lambs were hypoxic and hypercapnic compared with Sham animals. Lung compliance remained low in DH/TO+R lambs, most likely as a consequence of the persistent increase in alveolar wall thickness in these animals.

We examined the effect of accelerated lung growth, induced by in utero tracheal occlusion (TO), on lung liquid uptake in near-term fetal sheep. In utero TO was performed in five fetal sheep at 110 days of gestation (term, ∼145 days); six SHAM operated fetuses served as controls. The rate of liquid movement across the pulmonary epithelium was measured, using a previously established technique, in anesthetized fetal sheep between 133–137 days of gestation during a 2-hr adrenaline infusion (0.50 μg/min/kg, I.V.) and while lung luminal pressure was maintained at 5 mmHg. The rate of fetal lung liquid uptake was linear in all fetuses (mean r2