Assessment of echocardiographic interpretation of dual-lumen cannula during venovenous extracorporeal membrane oxygenation use for pediatric respiratory failure.

PURPOSE: Echocardiography is considered essential during cannulation placement and manipulations. Literature evaluating transthoracic echocardiography (TTE) usage during pediatric VV-ECMO is scant. The purpose of this study is to describe the use of echocardiography during VV-ECMO at a large, quaternary children's hospital. METHODS: A retrospective, single-year cohort study was performed of pediatric patients on VV-ECMO via dual-lumen cannula at our institution from January 2019 through December 2019. For each echocardiogram, final cannula component (re-infusion port (ReP), distal tip, proximal port and distal port) positions were evaluated by one echocardiographer. For TTEs with ReP in the right atrium, two echocardiographers independently evaluated ReP direction using 2-point (Yes/No) and 4-point scales, which were semi-quantitative protocols using color Doppler images to estimate ReP jet direction to the tricuspid valve. Cohen's kappa or weighted kappa was used to measure interrater agreement. RESULTS: During study period, 11 patients (64% male) received VV-ECMO with 49 TTEs and one transesophageal echocardiogram performed. The median patient age was 4.3 years [IQR: 1.1-11.5] and median VV-ECMO run time of 192 h [90-349]. The median time between TTEs on VV-ECMO was 34 h [8.3-65]. Most common position for the ReP was the right atrium (n = 33, 67%), and ReP location was not identified in five TTEs (10%). For ReP flow direction, echocardiographers agreed on 82% of TTEs using 2-point evaluation. There was only moderate agreement between echocardiographers on the 2-point and 4-point assessments (k = .54, kw = .46 respectively). CONCLUSIONS: TTE is the predominant cardiac ultrasound modality used during VV-ECMO for pediatric respiratory failure. Subjective evaluation of VV-ECMO ReP jet direction in the right atrium is challenging, regardless of assessment method.

Congenital Nasal Pyriform Aperture Stenosis in Association With Polymalformative Syndrome of the Midline.

Congenital nasal pyriform aperture stenosis (CNPAS) is a rare neonatal entity characterized by a reduction in the pyriform orifice of the nasal cavity. Because of its nonspecific clinical presentation as respiratory distress symptoms, it can mimic choanal atresia. Although isolated forms have been described, CNPAS is often associated with other congenital midline malformations. A single median incisor is usually found, with or without other cervical and maxillofacial malformations. The existence of hypothalamic-pituitary axis malformations with endocrine disorders is also possible and, in some cases, a moderate to severe intellectual deficit in association with other brain malformations. Radiological investigation is a central point in the multidisciplinary management of this type of polymalformative syndrome.

S/F and ROX indices in predicting failure of high-flow nasal cannula in children.

BACKGROUND: A high-flow nasal cannula (HFNC) is a useful respiratory support for children with respiratory distress; however, it elevates the risk of belated intubation. Recently, indices based on percutaneous oxygen saturation, a fraction of inspired oxygen, and respiratory rate (RR) have been suggested for the prediction of HFNC failure. We aimed to evaluate various indices predicting HFNC failure in children who started receiving HFNC at a tertiary center for 27 months. METHODS: Cases of HFNC failure were classified as hypoxic respiratory failure (HRF) or non-HRF (NHRF) depending on the cause of intubation. The ratio of percutaneous oxygen saturation to the fraction of inspired oxygen (S/F), the ratio of S/F to RR (ROX), the ratio of S/F to RR/median RR (ROX-M), and the ratio of S/F to z-score of RR (ROX-Z) were calculated and compared between groups. RESULTS: Of the 152 cases, 45 (29.6%) failed to wean from the HFNC support, of which 21 (46.7%) were HRFs and 24 (53.3%) were NHRFs. The S/F and ROX-M at 6 h and 3 h, respectively, predicted HRF well with a high area under the curve. Initial hypercapnia and low weight were good predictors for NHRF. CONCLUSIONS: For the management of children with HFNC, these risk factors and indicators should be monitored to make an early decision regarding intubation.

Hypercarbia in a Pediatric Patient With Relapsed Medulloblastoma.

Pediatric medulloblastoma is a common form of pediatric brain tumor and typically presents with progressive signs of increased intracranial pressure and ataxia. Relapse of the disease is most often diagnosed on surveillance imaging. We present the case of a 13-year-old boy with a previous history of medulloblastoma who presented with chronic hypercarbic respiratory failure as a symptom of a recurrent tumor. Imaging demonstrated a left cerebellar enhancing mass with leptomeningeal thickness and extension to the posterior medulla oblongata, which is the center for respiratory control. His hypercarbic respiratory failure represents a unique presentation of a central nervous system (CNS) tumor. Thus, this case illustrates the importance of thorough evaluation for CNS tumors involving the brainstem in patients with respiratory acidosis and no clear pulmonary etiology.

Cannot Ventilate: An Unexpected Cause of Respiratory Failure in a Ten-Year-Old Child.

Granulomatosis with polyangiitis (GPA) is a necrotizing vasculitis known to affect the respiratory and renal systems. There are a multitude of clinical manifestations, many of which are not specific to the disease, such as dysfunction of the nasal, sinus, auditory, tracheal, pulmonary, ocular, renal, cardiac, and nervous systems. As a multisystemic illness without a "classic" presentation and insidious progression, it is often a challenging diagnosis. We report and discuss a case of a 10-year-old female with no significant past medical history who presented to the emergency department with a 10-day course of worsening respiratory symptoms. As her respiratory and clinical status began to precipitously decline, the decision was made to intubate the patient, which was performed without issue. Unfortunately, attempts at oxygenating and ventilating the patient were met with extreme resistance and difficulty-an airway situation that could have been catastrophic if not for quick reaction maneuvers performed that would ultimately go on to remedy the issue at hand. We hope to raise awareness regarding the airway challenges posed by GPA and delve into its management as a means of improving recognition and preparing clinicians to treat this condition.

Effect of dexmedetomidine and propofol sedation on the prognosis of children with severe respiratory failure: a systematic review and meta-analysis.

Background: During treatment of acute respiratory failure (ARF) in children, sedation can reduce pain, improve tolerance, and reduce the incidence of adverse events, so selecting an appropriate sedation strategy is very important for improving prognosis and quality of life. Both dexmedetomidine and propofol have good sedative effects, so we investigated the application of these drugs in critically ill children with ARF by literature search and meta-analysis. Methods: We searched Embase, The Cochrane Library, PubMed, Ovid, Clinicaltrials.org, and Google Scholar for randomized controlled trials (RCTs) preferentially but not exclusively, and used RevMan 5.4 to analyze the screened literature. Results: Seven studies were included in the quantitative meta-analysis, with a total of 1,188 patients. There was no significant difference in the effect of dexmedetomidine and propofol on the duration of tracheal intubation in children with ARF [mean difference (MD) =-0.05; 95% confidence interval (CI): (-0.42, 0.32); Z=0.26; P=0.79], but dexmedetomidine sedation could reduce the intensive care unit (ICU) stay in children with ARF [MD =-0.62; 95% CI: (-1.08, -0.16); Z=2.65; P=0.008], and shorten the total hospital stay [MD =-1.94; 95% CI: (-2.63, -1.25); Z=5.48; P<0.00001]. There was no significant effect on mortality between the two groups [odds ratio (OR) =0.48; 95% CI: (0.19, 1.25); Z=1.50; P=0.13]. The incidence rate of bradycardia with dexmedetomidine sedation was higher than with propofol [OR =12.30; 95% CI: (2.28, 66.47); Z=2.92; P=0.004], and the incidence of hypotension was also higher [OR =6.99, 95% CI: (1.22, 39.86); Z=2.19; P=0.03]. Discussion: Compared with propofol, dexmedetomidine can significantly reduce the ICU stay and hospital stay. However, bradycardia and hypotension may occur during the use of dexmedetomidine, which requires close attention and timely intervention.

A pumpless artificial lung without systemic anticoagulation: The Nitric Oxide Surface Anticoagulation system.

BACKGROUND: Artificial lungs have the potential to serve as a bridge to transplantation or recovery for children with end-stage lung disease dependent on extracorporeal life support, but such devices currently require systemic anticoagulation. We describe our experience using the novel Nitric Oxide (NO) Surface Anticoagulation (NOSA) system-an NO-releasing circuit with NO in the sweep gas-with the Pediatric MLung-a low-resistance, pumpless artificial lung. METHODS: NO flux testing: MLungs (n = 4) were tested using veno-venous extracorporeal life support in a sheep under anesthesia with blood flow set to 0.5 and 1 L/min and sweep gas blended with 100 ppm NO at 1, 2, and 4 L/min. NO and NO2 were measured in the sweep and exhaust gas to calculate NO flux across the MLung membrane. Pumpless implants: Sheep (20-100 kg, n = 3) underwent thoracotomy and cannulation via the pulmonary artery (device inflow) and left atrium (device outflow) using cannulae and circuit components coated with an NO donor (diazeniumdiolated dibutylhexanediamine; DBHD-N2O2) and argatroban. Animals were connected to the MLung with 100 ppm NO in the sweep gas under anesthesia for 24 h with no systemic anticoagulation after cannulation. RESULTS: NO flux testing: NO flux averaged 3.4 ± 1.0 flux units (x10-10 mol/cm2/min) (human vascular endothelium: 0.5-4 flux units). Pumpless implants: 3 sheep survived 24 h with patent circuits. MLung blood flow was 716 ± 227 mL/min. Outlet oxygen saturation was 98.3 ± 2.6%. Activated clotting time was 151±24 s. Platelet count declined from 334,333 ± 112,225 to 123,667 ± 7,637 over 24 h. Plasma free hemoglobin and leukocyte and platelet activation did not significantly change. CONCLUSIONS: The NOSA system provides NO flux across a gas-exchange membrane of a pumpless artificial lung at a similar rate as native vascular endothelium and achieves effective local anticoagulation of an artificial lung circuit for 24 h.

High-Frequency Jet Ventilation in Infants With Congenital Heart Disease.

BACKGROUND: High-frequency jet ventilation (HFJV) is primarily used in neonates but may also have a role in the treatment of infants with congenital heart disease and severe respiratory failure. We hypothesized that HFJV would result in improved gas exchange in these infants. METHODS: We retrospectively reviewed the records of all pediatric patients with complex congenital heart disease treated HFJV in our pediatric cardiac ICU between 2014 and 2018. Patients in whom HFJV was started while on extracorporeal membrane oxygenation (ECMO) were excluded. We extracted data on demographics, pulmonary mechanics, gas exchange, the subsequent need for ECMO, use of inhaled nitric oxide, and outcomes. RESULTS: We included 27 subjects (median [interquartile range {IQR}] weight 4.4 [3.3-5.4] kg; median [IQR] age 2.5 [0.3-5.4] months), 22 (82%) of whom had cyanotic heart disease. Thirteen subjects (48%) survived and 6 (22%) required ECMO. HFJV was started after a median (IQR) of 8.4 (2.1-26.3) d of conventional mechanical ventilation. The subjects spent a median (IQR) of 1.2 (0.5-2.8) d on HFJV. The median (IQR) pre-HFJV blood gas results (n = 25) were pH 7.22 (7.17-7.31), [Formula: see text] 69 (51-77) mm Hg, and [Formula: see text] 51 (41-76) mm Hg. Median (IQR) initial HFJV settings were peak inspiratory pressure of 45 (36-50) cm H2O, breathing frequency of 360 (360-380) breaths/min, and inspiratory time of 0.02 (0.02-0.03) s. Compared with conventional mechanical ventilation, at 4-6 h after HFJV initiation, there were significant improvements in the median pH (7.22 vs 7.34; P = .001) and [Formula: see text] (69 vs 50 mm Hg; P = .001), respectively, but no difference in median [Formula: see text] (51 vs 53 mm Hg; P = .97). CONCLUSIONS: HFJV was associated with a decrease in [Formula: see text] and an increase in pH in infants with congenital heart disease who remained on HFJV 4 to 6 h after initiation.

High-Frequency Jet Ventilation in Pediatric Acute Respiratory Failure.

BACKGROUND: High-frequency jet ventilation (HFJV) is primarily used in premature neonates; however, its use in pediatric patients with acute respiratory failure has been reported. The objective of this study was to evaluate HFJV use in the pediatric critical care setting. We hypothesized that HFJV would be associated with improvements in oxygenation and ventilation. METHODS: Medical records of all patients who received HFJV in the pediatric ICU of a quaternary care center between 2014 and 2018 were retrospectively reviewed. Premature infants who had not been discharged home were excluded, as were those in whom HFJV was started while on extracorporeal membrane oxygenation. Data on demographics, pulmonary mechanics, gas exchange, and outcomes were extracted and analyzed using chi-square testing for categorical variables, nonparametric testing for continuous variables, and a linear effects model to evaluate gas exchange over time. RESULTS: A total of 35 subjects (median age = 2.9 months, median weight = 5.2 kg) were included. Prior to HFJV initiation, median (interquartile range) oxygenation index (OI) was 11.3 (7.2-16.9), [Formula: see text] = 133 (91.3-190.0), pH = 7.18 (7.11-7.27), [Formula: see text] = 64 (52-87) mm Hg, and [Formula: see text] = 74 (64-125) mm Hg. For subjects still on HFJV (n = 25), there was no significant change in OI, [Formula: see text], or [Formula: see text] at 4-6 h after initiation, whereas pH increased (P = .001) and [Formula: see text] decreased (P = .001). For those remaining on HFJV for > 72 h (n = 12), the linear effects model revealed no differences over 72 h for OI, [Formula: see text], [Formula: see text], or mean airway pressure, but there was a decrease in [Formula: see text] while pH and [Formula: see text] increased. There were 9 (26%) subjects who did not survive, and nonsurvivors had higher Pediatric Index of Mortality 2 scores (P = .01), were more likely to be immunocompromised (P = .01), were less likely to have a documented infection (P = .02), and had lower airway resistance (P = .02). CONCLUSIONS: HFJV was associated with improved ventilation among subjects able to remain on HFJV but had no significant effect on oxygenation.

Clinical Manifestations and Outcomes of Critically Ill Children and Adolescents with Coronavirus Disease 2019 in New York City.

OBJECTIVES: To describe the clinical manifestations and outcomes of critically ill children with coronavirus disease-19 (COVID-19) in New York City. STUDY DESIGN: Retrospective observational study of children 1 month to 21 years admitted March 14 to May 2, 2020, to 9 New York City pediatric intensive care units (PICUs) with severe acute respiratory syndrome coronavirus 2 infection. RESULTS: Of 70 children admitted to PICUs, median age was 15 (IQR 9, 19) years; 61.4% male; 38.6% Hispanic; 32.9% black; and 74.3% with comorbidities. Fever (72.9%) and cough (71.4%) were the common presenting symptoms. Twelve patients (17%) met severe sepsis criteria; 14 (20%) required vasopressor support; 21 (30%) developed acute respiratory distress syndrome (ARDS); 9 (12.9%) met acute kidney injury criteria; 1 (1.4%) required renal-replacement therapy, and 2 (2.8%) had cardiac arrest. For treatment, 27 (38.6%) patients received hydroxychloroquine; 13 (18.6%) remdesivir; 23 (32.9%) corticosteroids; 3 (4.3%) tocilizumab; and 1 (1.4%) anakinra; no patient was given immunoglobulin or convalescent plasma. Forty-nine (70%) patients required respiratory support: 14 (20.0%) noninvasive mechanical ventilation, 20 (28.6%) invasive mechanical ventilation (IMV), 7 (10%) prone position, 2 (2.8%) inhaled nitric oxide, and 1 (1.4%) extracorporeal membrane oxygenation. Nine (45%) of the 20 patients requiring IMV were extubated by day 14 with median IMV duration of 218 (IQR 79, 310.4) hours. Presence of ARDS was significantly associated with duration of PICU and hospital stay, and lower probability of PICU and hospital discharge at hospital day 14 (P < .05 for all). CONCLUSIONS: Critically ill children with COVID-19 predominantly are adolescents, have comorbidities, and require some form of respiratory support. The presence of ARDS is significantly associated with prolonged PICU and hospital stay.