Randomized, Placebo-Controlled Trial of Peri-Operative Treprostinil in Pediatric Patients Undergoing the Fontan Operation.

The objective of this study is to assess the effect of subcutaneous treprostinil (TRE) administered peri-operatively after Fontan operation on chest tube duration (CTD), hospital length of stay (LOS), and post-operative hemodynamics. This is a single center randomized, blinded, placebo-controlled study of pediatric patients with single ventricle congenital heart disease undergoing Fontan operation between September 2015 and September 2019. Patients were randomized to receive subcutaneous TRE (target dose 10 ng/kg/min) or saline placebo starting intraoperatively through post-operative day 7.

Programmatic Approach to Outpatient Cardiac Catheterization in Children With Stable Pulmonary Arterial Hypertension.

When performing cardiac catheterization in pediatric outpatients with pulmonary arterial hypertension (PH), our approach is to allow spontaneous ventilation, minimize procedural length, and evaluate for same-day discharge whenever safe and feasible. We describe our experience with this approach and identify clinical characteristics that influenced procedural safety. Outpatients < 21 years who underwent catheterization for PH from 2009 to 2018 were included in the retrospective cohort.

Hemodynamic Effects of Altering Tidal Volume During Positive Pressure Ventilation in the Fontan Circulation: A Randomized Crossover Trial.

Background: Primary determinants of pulmonary blood flow in the Fontan circulation are low transpulmonary gradient and pulmonary vascular resistance (PVR). Changes in intrathoracic pressure during intermittent positive pressure ventilation can influence the transpulmonary gradient, PVR, pulmonary blood flow, and cardiac output. The aim of this study was to evaluate the effect of low (5 mL/kg) versus high (10 mL/kg) tidal volume (V) ventilation on Fontan circulation hemodynamics.

Comparison of the Point-Of-Care Coagulation Device Quantra With the TEG-5000 for Congenital Cardiac Surgery-A Pilot Study.

Background: Congenital cardiac surgery with cardiopulmonary bypass (CPB) alters patients' hemostasis. Viscoelastic testing is a modern technology identifying coagulation abnormalities. A new device, the Quantra-QPlus System (HemoSonics LLC, Charlottesville, VA) has not yet been investigated during congenital cardiac surgery.

Single-Stage Midline Unifocalization Is Associated With Excellent Outcomes in Infants of All Ages.

ObjectiveTetralogy of Fallot, pulmonary atresia, and major aortopulmonary collateral arteries are a complex congenital heart defect. For years, our program has recommended early single-stage midline unifocalization at three to six months of age. However, many patients are referred beyond six months.

Acute Hemodynamics in the Fontan Circulation: Open-Label Study of Vasopressin.

Objective: To describe the acute hemodynamic effect of vasopressin on the Fontan circulation, including systemic and pulmonary pressures and resistances, left atrial pressure, and cardiac index.

Design: Prospective, open-label, nonrandomized study (NCT04463394).

Setting: Cardiac catheterization laboratory at Lucile Packard Children's Hospital, Stanford.

Clinical Study of Continuous Non-Invasive Blood Pressure Monitoring in Neonates.

The continuous monitoring of arterial blood pressure (BP) is vital for assessing and treating cardiovascular instability in a sick infant. Currently, invasive catheters are inserted into an artery to monitor critically-ill infants. Catheterization requires skill, is time consuming, prone to complications, and often painful.

Analgesia, Sedation, and Anesthesia for Neonates With Cardiac Disease.

Analgesia, sedation, and anesthesia are a continuum. Diagnostic and/or therapeutic procedures in newborns often require analgesia, sedation, and/or anesthesia. Newborns, in general, and, particularly, those with heart disease, have an increased risk of serious adverse events, including mortality under anesthesia.

Bronchopulmonary dysplasia.

Bronchopulmonary dysplasia is the most frequent adverse outcome of prematurity. Before implementation of antenatal steroids and surfactant therapy, bronchopulmonary dysplasia was mostly characterized by fibrotic, scarred, and hyper-inflated lungs due to pulmonary injury following mechanical ventilation and oxygen toxicity. With advances in neonatal medicine, this "old" bronchopulmonary dysplasia has changed to a "new" bronchopulmonary dysplasia characterized by an arrest in lung growth, leading to alveolar simplification and pulmonary vascular dysangiogenesis.

Neurosurgical intervention in children with ventricular assist devices: A single-center case series review.

Background: The incidence of neurological complications related to ventricular assist devices (VAD) remains high and includes life-threatening conditions such as intracranial hemorrhage or ischemic stroke. Although no definitive management guidelines exist, operative interventions may be required for major neurological injuries.

Aims: This case series describes the perioperative management of children at a single center who underwent neurosurgical procedures for major intracranial bleeds or ischemic strokes while on VAD support.

Impact of Modified Anesthesia Management for Pediatric Patients With Williams Syndrome.

Objective: This study compared the percent change in systolic blood pressure and the incidence of adverse cardiac events (ACEs; defined as cardiac arrest, cardiopulmonary resuscitation, arrhythmias, or ST-segment changes) during anesthesia induction in patients with Williams syndrome (WS) before and after implementation of a perioperative management strategy.

Design: Retrospective observational cohort study.

Setting: Single quaternary academic referral center.

Moyamoya disease in children and its anesthetic implications: A review.

Moyamoya disease is a rare, progressive cerebral vasculopathy which most commonly presents in the first and fourth decades of life. The mainstay of treatment is surgical revascularization; without treatment, most patients experience ischemic or hemorrhagic strokes. This report reviews moyamoya disease, its associated conditions, surgical treatment techniques, and anesthetic management of patients with moyamoya disease.

Applying Lessons from an Inaugural Clinical Pathway to Establish a Clinical Effectiveness Program.

Introduction: Clinical effectiveness (CE) programs promote standardization to reduce unnecessary variation and improve healthcare value. Best practices for successful and sustainable CE programs remain in question. We developed and implemented our inaugural clinical pathway with the aim of incorporating lessons learned in the build of a CE program at our academic children's hospital.

Accuracy of a portable pulse oximeter in monitoring hypoxemic infants with cyanotic heart disease.

Objective: Infants with single ventricle physiology have arterial oxygen saturations between 75 and 85%. Home monitoring with daily pulse oximetry is associated with improved interstage survival. They are typically sent home with expensive, bulky, hospital-grade pulse oximeters.

Pharmacokinetics of Cefazolin and Vancomycin in Infants Undergoing Open-Heart Surgery With Cardiopulmonary Bypass.

Background: Gram-positive bacteria account for nearly three-quarters of all surgical site infections. Antibiotic prophylaxis against these bacteria with cephalosporins or, in select circumstances, with vancomycin is considered standard of care for prevention of surgical site infections. There is little evidence to describe the optimal dosing regimen for surgical site infection prophylaxis in infants undergoing cardiac surgery, and a great deal of institutional variability exists in dosing prophylactic antibiotics.

Assessment of Ketamine Adult Anesthetic Doses in Pediatrics Using Pharmacokinetic Modeling and Simulations.

Background: Although few studies have used ketamine for induction and maintenance of pediatric anesthesia, official dosage recommendations are lacking. This study evaluates the outcomes of adult anesthetic doses in a pediatric population through pharmacokinetic modeling and computer simulations in an attempt to recommend an adequate ketamine dosing regimen.

Methods: Ketamine plasma concentration-time data in 19 children (age 8 months to 16 years; weight 5.

Safety of ferumoxytol in children undergoing cardiac MRI under general anaesthesia.

Background: Ferumoxytol, an "off-label" contrast agent, allows for better cardiac MRI quality as compared with gadolinium-based contrast agents. However, hypotension has been reported with the use of ferumoxytol for indications other than cardiac MRI. The purpose of our investigation was to evaluate the safety of ferumoxytol in children undergoing general anaesthesia for cardiac MRI.

LVOT-VTI is a Useful Indicator of Low Ventricular Function in Young Patients.

Left ventricular outflow tract velocity time integral (LVOT-VTI), a Doppler-derived measure of stroke distance, is used as a surrogate marker of cardiac function in adults. LVOT-VTI is easily obtained, independent of ventricular geometry and wall motion abnormalities. We investigated the relationship between LVOT-VTI and conventional measures of function in young patients by comparing controls to children with dilated cardiomyopathy (DCM).

Perioperative management of pediatric en-bloc combined heart-liver transplants: a case series review.

Background: 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.

Cognitive Dysfunction in Children with Heart Disease: The Role of Anesthesia and Sedation.

As physicians and caregivers of children with congenital heart disease, we are aware of the increasing need for procedures requiring anesthesia. While these procedures may be ideal for medical and cardiac surgical management, the risks and benefits must be assessed carefully. There are well known risks of cardiovascular and respiratory complications from anesthesia and sedation and a potentially under-appreciated risk of neurocognitive dysfunction.

Hemodynamic Effects of Phenylephrine, Vasopressin, and Epinephrine in Children With Pulmonary Hypertension: A Pilot Study.

Objectives: During a pulmonary hypertensive crisis, the marked increase in pulmonary vascular resistance can result in acute right ventricular failure and death. Currently, there are no therapeutic guidelines for managing an acute crisis. This pilot study examined the hemodynamic effects of phenylephrine, arginine vasopressin, and epinephrine in pediatric patients with pulmonary hypertension.

Accuracy of Pulse Oximeters Intended for Hypoxemic Pediatric Patients.

Objectives: Prior studies have shown inaccuracies in pulse oximetry readings at saturations less than 85%; however, no large studies have evaluated new sensors marketed for these low saturations. This study's purpose was to evaluate two sensors with claims of improved accuracy in children with saturations less than 85%.

Design: Prospective observational study.