Multi-Disciplinary Management and Surgical Resection of Intracardiac Fibromas Causing Bilateral Ventricular Outflow Tract Obstructions in an Infant.

Cardiac tumors remain rare in children with benign pathologies predominating. Indications for surgical management often result from compromised ventricular chamber size, biventricular outflow tract obstruction, impaired ventricular function, or the presence of medically refractory dysrhythmias. We present a case of a six-month-old infant with two intracardiac fibromas originating in the interventricular septum. The fibromas were causing significant biventricular outflow obstruction. The patient successfully underwent tumor resection on cardiopulmonary bypass The literature on pediatric cardiac tumors is reviewed. Multi-disciplinary medical planning is necessary for successful anesthetic and surgical treatment of this high-risk patient population.

Metabolomic profiling demonstrates evidence for kidney and urine metabolic dysregulation in a piglet model of cardiac surgery-induced acute kidney injury.

Acute kidney injury (AKI) is a common cause of morbidity after congenital heart disease surgery. Progress on diagnosis and therapy remains limited, however, in part due to poor mechanistic understanding and a lack of relevant translational models. Metabolomic approaches could help identify novel mechanisms of injury and potential therapeutic targets. In the present study, we used a piglet model of cardiopulmonary bypass with deep hypothermic circulatory arrest (CPB/DHCA) and targeted metabolic profiling of kidney tissue, urine, and serum to evaluate metabolic changes specific to animals with histological acute kidney injury. CPB/DHCA animals with acute kidney injury were compared with those without acute kidney injury and mechanically ventilated controls. Acute kidney injury occurred in 10 of 20 CPB/DHCA animals 4 h after CPB/DHCA and 0 of 7 control animals. Injured kidneys showed a distinct tissue metabolic profile compared with uninjured kidneys (R2 = 0.93, Q2 = 0.53), with evidence of dysregulated tryptophan and purine metabolism. Nine urine metabolites differed significantly in animals with acute kidney injury with a pattern suggestive of increased aerobic glycolysis. Dysregulated metabolites in kidney tissue and urine did not overlap. CPB/DHCA strongly affected the serum metabolic profile, with only one metabolite that differed significantly with acute kidney injury (pyroglutamic acid, a marker of oxidative stress). In conclusion, based on these findings, kidney tryptophan and purine metabolism are candidates for further mechanistic and therapeutic investigation. Urine biomarkers of aerobic glycolysis could help diagnose early acute kidney injury after CPB/DHCA and warrant further evaluation. The serum metabolites measured at this early time point did not strongly differentiate based on acute kidney injury.NEW & NOTEWORTHY This project explored the metabolic underpinnings of postoperative acute kidney injury (AKI) following pediatric cardiac surgery in a translationally relevant large animal model of cardiopulmonary bypass with deep hypothermic circulatory arrest. Here, we present novel evidence for dysregulated tryptophan catabolism and purine catabolism in kidney tissue and increased urinary glycolysis intermediates in animals who developed histological AKI. These pathways represent potential diagnostic and therapeutic targets for postoperative AKI in this high-risk population.

Regional lung metabolic profile in a piglet model of cardiopulmonary bypass with circulatory arrest.

INTRODUCTION: Acute lung injury is common following cardiopulmonary bypass and deep hypothermic circulatory arrest for congenital heart surgery with the most severe injury in the dorsocaudal lung. Metabolomics offers promise in deducing mechanisms of disease states, providing risk stratification, and understanding therapeutic responses in regards to CPB/DHCA related organ injury. OBJECTIVES: Using an infant porcine model, we sought to determine the individual and additive effects of CPB/DHCA and lung region on the metabolic fingerprint, metabolic pathways, and individual metabolites in lung tissue. METHODS: Twenty-seven infant piglets were divided into two groups: mechanical ventilation + CPB/DHCA (n = 20) and mechanical ventilation only (n = 7). Lung tissue was obtained from dorsocaudal and ventral regions. Targeted analysis of 235 metabolites was performed using HPLC/MS-MS. Data was analyzed using Principal Component Analysis (PCA), Partial Least Square Discriminant Analysis (PLS-DA), ANOVA, and pathway analysis. RESULTS: Profound metabolic differences were found in dorsocaudal compared to ventral lung zones by PCA and PLS-DA (R2 = 0.7; Q2 = 0.59; p < 0.0005). While overshadowed by the regional differences, some differences by exposure to CPB/DHCA were seen as well. Seventy-four metabolites differed among groups and pathway analysis revealed 20 differential metabolic pathways. CONCLUSION: Our results demonstrate significant metabolic disturbances between dorsocaudal and ventral lung regions during supine mechanical ventilation with or without CPB/DHCA. CPB/DHCA also leads to metabolic differences and may have additive effects to the regional disturbances. Most pathways driving this pathology are involved in energy metabolism and the metabolism of amino acids, carbohydrates, and reduction-oxidation pathways.

Selected 2020 Highlights in Congenital Cardiac Anesthesia.

This article is a review of the highlights of pertinent literature published during the 12 months of 2020 that are of interest to the congenital cardiac anesthesiologist. After a search of the US National Library of Medicine's PubMed database, several topics emerged for which significant contributions were made in 2020. The authors of the present article considered the following topics noteworthy to be included in this review: pediatric cardiac care in the coronavirus disease 2019 era, the use of mechanical circulatory support in coronavirus disease 2019-related multisystem inflammatory syndrome in children, transfusion and coagulation management in children undergoing congenital heart surgery, and pulmonary vein stenosis.

Achieving Sustainability in Reducing Unplanned Extubations in a Pediatric Cardiac ICU.

OBJECTIVES: To determine the incidence of unplanned extubations in a pediatric cardiac ICU in order to prove sustainability of our previously implemented quality improvement initiative. Additionally, we sought to identify risk factors associated with unplanned extubations as well as review the overall outcome of this patient population. DESIGN: Retrospective chart review. SETTING: Pediatric cardiac ICU at Children's Hospital of Colorado on the Anschutz Medical Center of the University of Colorado. PATIENTS: Intubated and mechanically ventilated patients in the cardiac ICU from July 2011 to December 2017. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: A total of 2,612 hospitalizations for 2,067 patients were supported with mechanical ventilation. Forty-five patients had 49 episodes of unplanned extubations (four patients > 1 unplanned extubation). The average unplanned extubation rate per 100 ventilator days was 0.4. Patients who had an unplanned extubation were younger (0.09 vs 5.45 mo; p < 0.001), weighed less (unplanned extubation median weight of 3.0 kg [interquartile range, 2.5-4.5 kg] vs control median weight of 6.0 kg [interquartile range, 3.5-13.9 kg]) (p < 0.001), and had a longer length of mechanical ventilation (8 vs 2 d; p < 0.001). Patients who had an unplanned extubation were more likely to require cardiopulmonary resuscitation during their hospital stay (54% vs 18%; p < 0.001) and had a higher likelihood of in-hospital mortality (15% vs 7%; p = 0.001). There was a significant difference in surgical acuity as denoted by The Society of Thoracic Surgeons-European Association for Cardio-Thoracic Surgery score and patients with an unplanned extubation had a higher Society of Thoracic Surgeons-European Association for Cardio-Thoracic Surgery category (p = 0.019). Contributing factors associated with unplanned extubation were poor endotracheal tube tape integrity, inadequate tube securement, and/or inadequate sedation. A low rate of unplanned extubation was maintained even in the setting of increasing patient complexity and an increase in patient volume. CONCLUSIONS: A low rate of unplanned extubation is sustainable even in the setting of increased patient volume and acuity. Additionally, early identification of patients at higher risk of unplanned extubation may also contribute to decreasing the incidence of unplanned extubation.

Alkaline Phosphatase Treatment of Acute Kidney Injury in an Infant Piglet Model of Cardiopulmonary Bypass with Deep Hypothermic Circulatory Arrest.

Acute kidney injury (AKI) is associated with prolonged hospitalization and mortality following infant cardiac surgery, but therapeutic options are limited. Alkaline phosphatase (AP) infusion reduced AKI in phase 2 sepsis trials but has not been evaluated for cardiac surgery-induced AKI. We developed a porcine model of infant cardiopulmonary bypass (CPB) with deep hypothermic circulatory arrest (DHCA) to investigate post-CPB/DHCA AKI, measure serum/renal tissue AP activity with escalating doses of AP infusion, and provide preliminary assessment of AP infusion for prevention of AKI. Infant pigs underwent CPB with DHCA followed by survival for 4 h. Groups were treated with escalating doses of bovine intestinal AP (1, 5, or 25U/kg/hr). Anesthesia controls were mechanically ventilated for 7 h without CPB. CPB/DHCA animals demonstrated histologic and biomarker evidence of AKI as well as decreased serum and renal tissue AP compared to anesthesia controls. Only high dose AP infusion significantly increased serum or renal tissue AP activity. Preliminary efficacy evaluation demonstrated a trend towards decreased AKI in the high dose AP group. The results of this dose-finding study indicate that AP infusion at the dose of 25U/kg/hr corrects serum and tissue AP deficiency and may prevent AKI in this piglet model of infant CPB/DHCA.

The Year in Review: Anesthesia for Congenital Heart Disease 2018.

This review article surveys the published literature from January 2018 to March 2019. Three themes were identified and articles were selected based on their originality and interest to anesthesiologists caring for patients with congenital heart disease.

Bilateral suprazygomatic maxillary nerve blocks vs. infraorbital and palatine nerve blocks in cleft lip and palate repair: A double-blind, randomised study.

BACKGROUND: Cleft defects are common craniofacial malformations which require early surgical repair. These patients are at high risk of postoperative airway obstruction and respiratory failure. Cleft surgery may require high doses of opioids which may contribute to these complications. OBJECTIVES: To compare the effectiveness of proximal and distal approaches to blocking the maxillary nerve in patients undergoing cleft lip or cleft palate surgery. DESIGN: Randomised, controlled and double-blind study. SETTING: The current study was carried out in Guwahati (Assam, India) between April 2014 and June 2014. PATIENTS: A total of 114 patients older than 6 months who underwent cleft lip or cleft palate surgery were included. Exclusion criteria included coagulation disorders, peripheral neuropathy or chronic pain syndrome, infection in the puncture site, allergy to local anaesthetics, lack of consent and language problems or other barriers that could impede the assessment of postoperative pain. INTERVENTIONS: Patients were randomly assigned to one of two groups: proximal group (bilateral suprazygomatic maxillary nerve blocks) and distal group (bilateral infraorbital nerve blocks for cleft lip repair and bilateral greater and lesser palatine nerve blocks and nasopalatine nerve block for cleft palate surgery). MAIN OUTCOME MEASURE: The primary endpoint was the percentage of patients requiring extra doses of opioids. Secondary endpoints included pain scores, respiratory and nerve block-related complications during the first 24 h. RESULTS: In the intra-operative period, there was a significant reduction of nalbuphine consumption in the proximal group (9.1 vs. 25.4%, P = 0.02). The percentage of patients requiring intra-operative fentanyl was lower in the proximal group (16.4 vs. 30.5%, P = 0.07). There were no differences in either postoperative pain scores or in postoperative complications. No technical failure or block-related complications were reported. CONCLUSION: Bilateral suprazygomatic maxillary nerve block is an effective and safe alternative to the traditional peripheral nerve blocks for cleft lip and cleft palate surgery, in a mixed paediatric and adult population.

Noteworthy Literature published in 2017 for Congenital Cardiac Anesthesiologists.

This review focuses on the literature published during the 13 months from December 2016 to December 2017 that is of interest to anesthesiologists taking care of children and adults with congenital heart disease. Five themes are addressed during this time period and 100 peer-reviewed articles are discussed.

The anesthetic management of button battery ingestion in children.

PURPOSE: Injuries related to button battery ingestion are common in children. This review provides an outline of the epidemiology, pathophysiology, management, and anesthetic implications in children who have ingested a button battery. SOURCE: A literature search was conducted in the United States National Library of Medicine PubMed database using the terms "button battery ingestion" and "children' and "removal" and "surgery" and "anesthesia". Ninety-six articles published in English were found from 1983-2017, and 62 of these articles were incorporated into this review. Additionally, the Internet was searched with the terms "button battery ingestion and children" to identify further entities, organizations, and resources affiliated with button battery ingestion in children. These additional sources were studied and included in this review. PRINCIPAL FINDINGS: Button batteries are ubiquitous in homes and electronic devices. Since 2006, larger-diameter and higher-voltage batteries have become available. These are more likely to become impacted in the esophagus after ingestion and lead to an increase in severe morbidity and mortality due to caustic tissue injury. Children at the highest risk for complications are those under six years of age who have ingested batteries > 20 mm in diameter and sustain prolonged esophageal impaction at the level of the aortic arch with the negative pole oriented anteriorly. CONCLUSION: Anesthesiologists need to know about the epidemiology, pathophysiology, complications, and anesthetic management of children who have ingested button batteries.

Cardiac Failure in a Trisomy 9 Patient Undergoing Anesthesia: A Case Report.

A 27-year-old female with Trisomy 9 mosaicism presented to Children's Hospital Colorado for outpatient dental surgery under general anesthesia. The patient's past medical history was also significant for premature birth, gastroesophageal reflux, scoliosis and kyphosis, obesity, and developmental delay. Per her mother's report, the patient had no cardiac issues. She had undergone multiple previous general anesthetics, some of which documented respiratory complications such as laryngospasm, bronchospasm, and possible aspiration. During this anesthetic, the patient became hypotensive on induction, with sluggish response to intravenous fluids, glycopyrrolate, and ephedrine. Her electrocardiogram demonstrated what appeared to be left bundle branch block at baseline, with possible ST segment changes after induction. Due to her abnormal reaction to the induction and subsequent treatment for hypotension, an echocardiogram was performed. The patient was found to have an ejection fraction of 25%-30%. The anesthetic was uneventful for the remainder of the procedure, and following recovery, the patient was admitted by the heart failure team for further care.