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Background The global COVID-19 pandemic was particularly concerning for the pediatric PH population due to immature immune systems and developmental comorbidities. This study aims to describe a single-center experience of pediatric PH patients diagnosed with COVID-19 disease. Methods Retrospective cohort study of all pediatric patients followed by the PH Center at Texas Children's Hospital diagnosed with COVID-19 infection from 04/2020 through 02/2021. Results We identified 23 patients with a median age of 58 months (IQR 25-75th, 21-132 months), 48% being Hispanics. Eight patients (35%) required hospitalization; median length of stay 6 days (IQR 25-75th , 5-8 days). Only three of these 8 patients required increased respiratory support. Targeted PH therapy was escalated in four patients (two in dual and two in triple therapy). There was one mortality in a patient with failing Fontan physiology. Ninety-one percent of patients have had post-COVID outpatient follow-up, median of 101 days (IQR 25-75th , 50-159 days) from diagnosis. Of the five patients with 6 minute walk test (6MWT) data, three (60%) children walked less distance, median -12 meters (IQR 25-75th , -12 - +49 meters) compared to pre-COVID testing. Post-infection pulmonary function testing (PFT) was notable for decrease in predicted forced vital capacity (FVC) (median -6%, range -11%-+6%) and forced expiratory volume in one second (FEV1) (median -14%, range -12- -18%) in 75% of the patients with PFT data. Conclusions In our institution, COVID-19 was found more frequently in Hispanics, and associated with low mortality. This article is protected by copyright. All rights reserved.

Pulmonary hypertension (PH), an often unrelenting disease that carries with it significant morbidity and mortality, affects not only the pulmonary vasculature but, in turn, the right ventricle as well. The survival of patients with PH is closely related to the right ventricular function. Therefore, having an understanding of how to manage right ventricular failure (RVF) and acute pulmonary hypertensive crises is imperative for clinicians who encounter these patients. This review addresses the management of these patients in detail, addressing: (a) the pathophysiology of RVF, (b) intensive care monitoring of these patients in the intensive care unit, (c) imaging of the right ventricle, (d) intubation and mechanical ventilation, (e) inotrope and vasopressor selection, (f) pulmonary vasodilator use, (g) interventional and surgical procedures for the acutely failing right ventricle, and (h) mechanical support for RVF.

Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides with pulmonary involvement include granulomatosis with polyangiitis, microscopic polyangiitis, and eosinophilic granulomatosis with polyangiitis, and can present with life-threatening pulmonary hemorrhage in up to 40% of patients. Mortality in those patients who require intubation and mechanical ventilation can reach 77%. Extracorporeal membrane oxygenation (ECMO) can be used to support these patients through definitive diagnosis and treatment, although minimizing the risk of ventilator-induced lung injury. We aimed to determine factors associated with favorable outcomes in patients with (ANCA)-associated vasculitides supported on ECMO. We performed a retrospective observational study using the Extracorporeal Life Support Organization registry of pediatric and adult patients with ANCA-associated vasculitis supported on ECMO from 2010 to 2020. One hundred thirty-five patients were included for analysis. Many patients had renal involvement (39%) in addition to pulmonary involvement (93%). Survival was 73% in AAV patients supported on ECMO. The presence of pulmonary hemorrhage was not associated with worse outcomes in our cohort. Older age, the use of venoarterial ECMO, ECMO-cardiopulmonary resuscitation, or sustaining a cardiac arrest before ECMO was associated with decreased survival. In conclusion, venovenous ECMO should be considered as a supportive bridge to definitive diagnosis and treatment in (ANCA)-associated vasculitides, regardless if pulmonary hemorrhage is present.

The spectrum of pulmonary hypertension associated with congenital heart disease (PH-CHD) is broad and complex. The most common aetiologies are related to left-to-right shunts, resulting in volume loading of the pulmonary circulation (precapillary pulmonary hypertension) and left-sided obstructive disease (post-capillary pulmonary hypertension). However, in addition to these basic classifications are patients with absent pulmonary arteries, large septal defects, duct-dependent lesions, cardiomyopathies, and, perhaps the most challenging, patients with single ventricle physiology lacking a subpulmonic ventricle. It is therefore imperative that physicians taking care of children with PH-CHD are well versed not only in the different physiologies present in these children, but also in those that result from their respective surgical palliations as well and how these flows and pressures relate to their pulmonary vascular disease burden. This chapter presents the case of a child with trisomy 21 and congenital heart disease who presented late to medical care and required significant modulation of her pulmonary vascular bed to successfully undergo surgical correction. The child’s clinical course, an overview of PH-CHD and its diagnosis and management, and important therapeutic considerations are presented.

ObjectiveTo investigate effects of a cognitive intervention based on isolation of red flags (I-RED) on diagnostic accuracy of ‘do-not-miss diagnoses.’DesignA 2 × 2 randomized case vignette-based experiment with manipulation of I-RED strategy between subjects and case complexity within subjects.SettingTwo university-based residency programs.ParticipantsOne-hundred and nine pediatric residents from all levels of training.InterventionsParticipants were randomly assigned to the I-RED vs. control group, and within each group, they were further randomized to the order in which they saw simple and complex cases. The I-RED strategy involved an instruction to look for a constellation of symptoms, signs, clinical data or circumstances that should heighten suspicion for a serious condition.Main Outcome MeasuresPrimary outcome was diagnostic accuracy, scored as 1 if any of the three differentials given by participants included the correct diagnosis, and 0 if not. We analyzed effects of I-RED strategy on diagnostic accuracy using logistic regression.ResultsI-RED strategy did not yield statistically higher diagnostic accuracy compared to controls (62 vs. 48%, respectively; odd ratio = 2.07 [95% confidence interval, 0.78–5.5], P = 0.14) although participants reported higher decision confidence compared to controls (7.00 vs. 5.77 on a scale of 1 to 10, P ConclusionsA cognitive strategy of prompting red flag isolation prior to differential diagnosis did not improve diagnostic accuracy of ‘do-not-miss diagnoses.’ Given the paucity of evidence-based solutions to reduce diagnostic error and the intervention’s potential effect on confidence, findings warrant additional exploration.

* ALT: : alanine aminotransferase AST: : aspartate aminotransferase BUN: : blood urea nitrogen CBC: : complete blood count CNS: : central nervous system CSF: : cerebrospinal fluid CT: : computed tomography ECG: : electrocardiography ED: : emergency department EEG: : electroencephalography ESR: : erythrocyte sedimentation rate GI: : gastrointestinal GU: : genitourinary Hct: : hematocrit Hgb: : hemoglobin MRI: : magnetic resonance imaging WBC: : white blood cell A 5-year-old boy is brought to the ED by emergency medical services in status epilepticus after a 3-day history of headaches. This morning he had a generalized tonic-clonic seizure, and paramedics administered lorazepam at his home. He continues to seize and requires a second dose of lorazepam on arrival to the ED. Once he is stabilized, a history is obtained from the parents. They describe the headaches as generalized, they start suddenly, and nothing seems to alleviate them. The headaches are not worse in the early morning and not associated with vomiting or vision changes. The boy has had no recent illnesses and has been eating well and acting well between the headaches. In the ED, his blood pressure is 137/106 mm Hg, heart rate is 109 beats/min, respiratory rate is 20 breaths/min, and temperature is 37.8°C. On physical examination, he is postictal but has no focal signs. All other physical findings, including on funduscopic examination, are normal. Results of laboratory evaluation, including CBC, basic metabolic panel, urinalysis, thyroid panel, complement assessment, antistreptolysin O titer, and cardiac enzymes, are within normal limits. CT scan of the brain also yields normal results, as does echocardiography. The patient is admitted to the intensive care unit. Further imaging studies reveal the diagnosis. A 13-year-old previously healthy competitive gymnast collapses while climbing a rope during practice. She exhibits cyanosis and apnea, and cardiopulmonary resuscitation is started immediately. An automated external defibrillator is connected by emergency personnel. The monitor shows a shockable rhythm (Fig. 1), and she is defibrillated once. Thereafter, she develops agonal respirations and sinus tachycardia. She remains unresponsive, has a Glasgow Coma Scale score of 5, and is endotracheally intubated. She has no history of fainting in the past, and her family history is negative for any sudden cardiac deaths, arrhythmias, or …