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.

Point-of-Care-Ultrasound in Pediatrics: A Review and Update.

Point-of-Care-Ultrasound (POCUS) has encountered a tremendous expansion in patient care. POCUS has taken a central role during invasive procedures. POCUS has expanded to most subspecialties from adult to pediatric and neonatal health care. POCUS in pediatrics has also become part of specific critical situations such as myocardial function assessment during cardiac arrest, extracorporeal membrane oxygenation deployment and neurological evaluation. In this review we will go over the most important historical aspects of POCUS. We will also review important aspects of POCUS in the intensive care unit, cardiologist evaluation and in the emergency department among others.

Cardiac Point-of-Care Ultrasound and Multi-Disciplinary Improvement Opportunities in Acute Systolic Heart Failure Management in a Pediatric Emergency Center.

Cardiac point-of-care ultrasound (POCUS) has the ability to rapidly assess function and identify systolic heart failure (HF), an often-missed diagnosis. POCUS has the potential to expedite medical intervention, improving overall outcomes. There have been limited studies describing pediatric emergency center (EC) utilization of cardiac POCUS and its impact on outcomes in pediatric patients. Authors performed a retrospective chart review at a tertiary children's hospital to identify all patients admitted from the EC to the Cardiac Intensive Care Unit (CICU) with acute systolic HF between January 2017 and August 2019. Outcome measures included EC length of stay (LOS), CICU LOS, and time until first IV HF medicine was administered. A total of 21 patients and 24 encounters meeting criteria were identified. Cardiac POCUS agreed with standard echocardiography in 8 of 9 cases. Patients who had a cardiac POCUS in the EC seemed more likely to receive their first dose of intravenous heart failure medication while in the Emergency Center (70% vs 43%). There was a trend toward significance, but it did not reach statistical significance (p = 0.1). EC and CICU LOS were not significantly different between POCUS and non-POCUS groups. Cardiac POCUS has the potential to have a valuable role in the early diagnosis of acute systolic HF in children. However, early diagnosis by POCUS did not translate into shorter EC or CICU LOS. This pilot data serves as a baseline for efforts to promote earlier clinical recognition of acute HF and more efficient collaboration between clinical services.

A Novel Tool for Teaching Cardiac Point-of-Care Ultrasound: An Exploratory Application of the Design-Based Research Approach.

OBJECTIVES: To develop a competency-based educational tool for cardiac point-of-care ultrasound image interpretation using the design-based research approach. DESIGN: In accordance with design-based research, multiple methods were used to determine that learners would benefit from achieving competency in cardiac point-of-care ultrasound image interpretation before they attempted it at the bedside. A competency-based educational tool was designed and underwent a rapid prototyping process with experts. Evaluative data was used to redesign and reevaluate the educational tool with a new group of learners to improve its effectiveness. SETTING: Large, university-based children's hospital. SUBJECTS: Pediatric critical care attending physicians, fellow physicians and advanced practice providers, and pediatric resident physicians. INTERVENTIONS: Integrating mastery learning and deliberate practice as theoretical frameworks, the authors designed an online "Image Library" composed of 90 questions of cardiac point-of-care ultrasound image clips with varying degrees of pathology that were organized into three levels of difficulty. Learners answered a set of 10 questions, learned from feedback, and repeated an additional set in the same difficulty until achieving a predefined level of mastery. MEASUREMENTS AND MAIN RESULTS: Two learning cycles were implemented with a total of 41 learners. Forty learners (98%) were able to demonstrate mastery on the Image Library and required a range of 30 to 210 questions to do so. On a 10-question final assessment, learners scored an average of 72%, while experts and novices scored 83% and 17%, respectively. On a 5-point scale, learners rated the quality of the Image Library for facilitation of learning (median 5), enjoyment of learning (5), and useful knowledge and skills to improve clinical practice (5). CONCLUSIONS: An effective and innovative tool for teaching cardiac point-of-care ultrasound image interpretation was developed using the design-based research approach. Our results demonstrate the importance of individualized learning timelines for ultrasound image interpretation.

Deficient histone H3 propionylation by BRPF1-KAT6 complexes in neurodevelopmental disorders and cancer.

Lysine acetyltransferase 6A (KAT6A) and its paralog KAT6B form stoichiometric complexes with bromodomain- and PHD finger-containing protein 1 (BRPF1) for acetylation of histone H3 at lysine 23 (H3K23). We report that these complexes also catalyze H3K23 propionylation in vitro and in vivo. Immunofluorescence microscopy and ATAC-See revealed the association of this modification with active chromatin. Brpf1 deletion obliterates the acylation in mouse embryos and fibroblasts. Moreover, we identify BRPF1 variants in 12 previously unidentified cases of syndromic intellectual disability and demonstrate that these cases and known BRPF1 variants impair H3K23 propionylation. Cardiac anomalies are present in a subset of the cases. H3K23 acylation is also impaired by cancer-derived somatic BRPF1 mutations. Valproate, vorinostat, propionate and butyrate promote H3K23 acylation. These results reveal the dual functionality of BRPF1-KAT6 complexes, shed light on mechanisms underlying related developmental disorders and various cancers, and suggest mutation-based therapy for medical conditions with deficient histone acylation.

Bridging the Cardiac Needs of a Large, Underserved Immigrant and Resettled Refugee Population.

OBJECTIVE: To describe a monthly outreach pediatric cardiology clinic established to better understand the cardiac needs of immigrant/resettled refugee children. STUDY DESIGN: Data obtained between 2014 and 2017 from a monthly pediatric cardiology clinic at a Federally Qualified Health Center were analyzed using descriptive statistics. RESULTS: A total of 366 patients (222 male, 61%) were evaluated. Indications for referral included murmur (242, 66%), nonexertional symptoms (31, 9%), exertional symptoms (16, 4%), history of cardiac surgery/transcatheter interventions (15, 4%), previous diagnosis of heart conditions without intervention (13, 4%), arrhythmia/bradycardia (13, 4%), and others (36, 10%). Echocardiograms were performed on 136 patients (67 were abnormal, 49%). The most common final diagnoses include innocent murmur in 201 (55%), simple congenital heart disease in 61 (16%), complex congenital heart disease in 3 (1%), and acquired heart disease in 3 (1%). A total of 15 patients (4%) were ultimately determined to require surgical or cardiac catherization as an intervention. Patients have been followed for a median of 0.7 years (range 0-3.3 years). CONCLUSIONS: Rates of abnormal echocardiograms suggest a greater likelihood of congenital or acquired heart disease at time of initial consultation compared with nonimmigrant/refugee populations. The most common indication for referral to the outreach pediatric cardiology clinic was a murmur. Collaborative efforts between physicians and support services are essential in assisting this vulnerable population access pediatric subspecialty care.

Hand-held echocardiography in children with hypoplastic left heart syndrome.

BACKGROUND: When performed by cardiologists, hand-held echocardiography (HHE) can assess ventricular systolic function and valve disease in adults, but its accuracy and utility in congenital heart disease is unknown. In hypoplastic left heart syndrome (HLHS), the echocardiographic detection of depressed right ventricular (RV) systolic function and higher grade tricuspid regurgitation (TR) can identify patients who are at increased risk of morbidity and mortality and who may benefit from additional imaging or medical therapies. METHODS: Children with HLHS after Stage I or II surgical palliation (Norwood or Glenn procedures) were prospectively enrolled. Subjects underwent HHE by a pediatric cardiologist on the same day as standard echocardiography (SE). Using 4-point scales, bedside HHE assessment of RV systolic function and TR were compared with blinded assessment of offline SE images. Concordance correlation coefficient (CCC) was used to evaluate agreement. RESULTS: Thirty-two HHEs were performed on 15 subjects (Stage I: n = 17 and Stage II: n = 15). Median subject age was 3.4 months (14 days-4.2 years). Median weight was 5.9 kg (2.6-15.4 kg). Bedside HHE assessment of RV systolic function and TR severity had substantial agreement with SE (CCC = 0.80, CCC = 0.74, respectively; P < .001). HHE sensitivity and specificity for any grade of depressed RV systolic function were 100% and 92%, respectively, and were 94% and 88% for moderate or greater TR, respectively. Average HHE scan time was 238 seconds. CONCLUSIONS: HHE offers a rapid, bedside tool for pediatric cardiologists to detect RV systolic dysfunction and hemodynamically significant TR in HLHS.