We report a rare case of hereditary hemorrhagic telangiectasia (HHT) in a 4-month-old female infant with findings of child abuse. She presented with poor feeding, vomiting, and irritability after a short fall from the bed. Initial evaluation found subdural hematomas, persistent hypoxia, failure to thrive, a frenulum tear, facial lacerations, and bruising.
View Article and Find Full Text PDFObjectives: To characterize trends in noninvasive ventilation (NIV) and invasive mechanical ventilation (IMV) use over time in children with hematologic malignancy admitted to the PICU with acute respiratory failure (ARF), and to identify risk factors associated with NIV failure requiring transition to IMV.
Design: Retrospective cohort analysis using the Virtual Pediatric Systems (VPS, LLC) between January 1, 2010 and December 31, 2019.
Setting: One hundred thirteen North American PICUs participating in VPS.
Background: The National Pediatric Cardiology Quality Improvement Collaborative (NPC-QIC) lacks a rigorous enrollment audit process, unlike other collaborative networks. Most centers require individual families to consent to participate. It is unknown whether there is variation across centers or biases in enrollment.
View Article and Find Full Text PDFUnlabelled: Patients with hypoplastic left heart syndrome who have been palliated with the Fontan procedure are at risk for adverse neurodevelopmental outcomes, lower quality of life, and reduced employability. We describe the methods (including quality assurance and quality control protocols) and challenges of a multi-center observational ancillary study, SVRIII (Single Ventricle Reconstruction Trial) Brain Connectome. Our original goal was to obtain advanced neuroimaging (Diffusion Tensor Imaging and Resting-BOLD) in 140 SVR III participants and 100 healthy controls for brain connectome analyses.
View Article and Find Full Text PDFChildren with congenital heart disease (CHD) are at risk of malnutrition; however, there is limited information regarding the impact of nutritional status on organ dysfunction and outcomes after surgery for CHD. The study aim was to assess the association between malnutrition, organ dysfunction, and outcomes after surgery for CHD. Retrospective cohort study of patients aged 30 days to 18 years admitted to the cardiac intensive care unit (CICU) following cardiac surgery.
View Article and Find Full Text PDFObjective: The hemoglobin threshold for a decision to transfuse red blood cells in univentricular patients with parallel circulation is unclear. A pediatric expertise initiative put forth a "weak recommendation" for avoiding reflexive transfusion beyond a hemoglobin of 9 g/dL. We have created a mathematical model to assess the impact of hemoglobin thresholds in patients with parallel circulation.
View Article and Find Full Text PDFBackground: Stage 1 palliation (S1P) for hypoplastic left heart syndrome remains associated with high morbidity and mortality. Previous studies on burden of reinterventions did not include patients who remain hospitalized before stage 2 palliation (S2P). This study described the rate of reintervention during S1P hospitalization and sought to determine the impact of reintervention on outcomes.
View Article and Find Full Text PDFObjectives: Arginine vasopressin (AVP) is used to treat hypotension. Because AVP increases blood pressure by increasing systemic vascular resistance, it may have an adverse effect on tissue oxygenation following the Norwood procedure.
Methods: Retrospective analysis of continuously captured hemodynamic data of neonates receiving AVP following the Norwood procedure.
Objective: The optimal timing for neonatal cardiac surgery is a potentially modifiable factor that may affect outcomes. We studied the relationship between age at surgery (AAS) and outcomes across multiple hospitals, focusing on neonatal operations where timing appears is not emergency.
Methods: We studied neonates ≥37 weeks' gestation and ≥2.
Objective: Prematurity, low birth weight, genetic syndromes, extracardiac conditions, and secondary cardiac lesions are considered high-risk conditions associated with mortality after stage 1 palliation. We report the impact of these conditions on outcomes from a prospective multicenter improvement collaborative.
Methods: The National Pediatric Cardiology Quality Improvement Collaborative Phase II registry was queried.
Objectives: To determine if indices of alveolar gas exchange preextubation predict postextubation respiratory support needs as well as the need for escalation of therapies following infant cardiac surgery.
Design: Retrospective chart review.
Setting: Pediatric cardiac ICU in a quaternary-care teaching hospital.
Objectives: Opioids are used to manage pain, comfort, maintain devices, and decrease oxygen consumption around Norwood palliation (NP), but in high dose and prolonged exposure, they increase risk of tolerance and iatrogenic withdrawal syndrome (IAWS). Variability in practice for IAWS prevention potentially increases opioid dose and duration. We hypothesize that protocolized weaning with morphine (MOR) versus nonprotocolized methadone (MTD) is associated with reduction in opioid exposure.
View Article and Find Full Text PDFBackground: The modified Blalock-Taussig-Thomas shunt is a critically important palliation for patients with insufficient pulmonary blood flow associated with congenital heart disease. After creating a modified Blalock-Taussig-Thomas shunt patients experience high rates of early postoperative morbidity and mortality.
Methods: This is a single-institution retrospective cohort study.
Objectives: In the vast majority of Children's Hospitals, the critically ill patient can be found in one of three locations: the PICU, the neonatal ICU, and the cardiac ICU. Training, certification, and maintenance of certification for neonatology and critical care medicine are over seen by the Accreditation Council for Graduate Medical Education and American Board of Pediatrics. There is no standardization of training or oversight of certification and maintenance of certification for pediatric cardiac critical care.
View Article and Find Full Text PDFBackground: Cerebral and myocardial hypoperfusion occur during hemodialysis in adults. Pediatric patients receiving chronic hemodialysis have fewer cardiovascular risk factors, yet cardiovascular morbidity remains prominent.
Methods: We conducted a prospective observational study of pediatric patients receiving chronic hemodialysis to investigate whether intermittent hemodialysis is associated with adverse end organ effects in the heart or with cerebral oxygenation (regional tissue oxyhemoglobin saturation [rSO2]).
Medically complex children including infants undergoing cardiac surgery are at increased risk for hospital readmissions. Investigation of this population may reveal opportunities to optimize systems and coordination of care. A retrospective study of all infants undergoing cardiac surgery from 2015 through 2016 at a large tertiary institution who were readmitted within 1 year of discharge from cardiac surgical hospitalization was performed.
View Article and Find Full Text PDFBackground: Interstage mortality (IM) remains high for patients with single-ventricle congenital heart disease (SVCHD) in the period between Stage 1 Palliation (S1P) and Glenn operation. We sought to characterize IM.
Methods: This was a descriptive analysis of 2184 patients with SVCHD discharged home after S1P from 60 National Pediatric Cardiology Quality Improvement Collaborative sites between 2008 and 2015.
Background: Increasing numbers of adults with congenital heart disease (ACHD) undergo cardiac surgical procedures in children's hospitals, yet surgical outcomes data are limited. We sought to better understand the impact of preoperative risk factors on postoperative complications and cardiac intensive care unit (CICU) length of stay (LOS).
Methods: Surgical CICU admissions for patients aged 18 years and older in the Pediatric Cardiac Critical Care Consortium registry from August 2014 to January 2019 in 34 hospitals were included.
Background: Cerebralvascular accidents (CVA) are common complications of pediatric ventricular assist devices (VADs). We employed the Pediatric Interagency Registry for Mechanical Circulatory Support (Pedimacs) to investigate rates, risk factors, and outcomes of CVA in pediatric patients supported on VAD.
Methods: Analysis of Pedimacs (September 2012-June 2019) data to determine rates of all neurologic events and specifically CVA.
Background: Previous studies suggest that birth before 39 weeks' gestational age (GA) is associated with higher perioperative mortality and morbidity after congenital heart surgery. The optimal approach to timing of cardiac operation in premature infants remains unclear. We investigated the impact of GA at birth and corrected GA at surgery on postoperative outcomes using the Pediatric Cardiac Critical Care Consortium (PC) database.
View Article and Find Full Text PDFBackground: Birth weight, preterm delivery, and size for gestational age are surrogate markers for development that are commonly used in congenital heart surgery. Understanding the associations of these variables with patient outcomes is of great importance.
Methods: This study included all patients with hypoplastic left heart syndrome who underwent a Norwood procedure at a single institution from 1995 to 2018.
This scientific statement summarizes the current state of knowledge related to interstage home monitoring for infants with shunt-dependent single ventricle heart disease. Historically, the interstage period has been defined as the time of discharge from the initial palliative procedure to the time of second stage palliation. High mortality rates during the interstage period led to the implementation of in-home surveillance strategies to detect physiologic changes that may precede hemodynamic decompensation in interstage infants with single ventricle heart disease.
View Article and Find Full Text PDFBackground: Mortality after stage 1 palliation of hypoplastic left heart syndrome remains significant. Both cardiac output (CO) and systemic vascular resistance (SVR) contribute to hemodynamic vulnerability. Simultaneous measures of mean arterial pressure and somatic regional near infrared spectroscopy saturation can classify complex hemodynamics into 4 distinct states, with a low-CO state of higher risk.
View Article and Find Full Text PDFObjectives: Early extubation following pediatric cardiac surgery is common, but debate exists whether location affects outcome, with some centers performing routine early extubations in the operating room (odds ratio) and others in the cardiac ICU. We aimed to define early extubation practice variation across hospitals and assess impact of location on hospital length-of-stay and other outcomes.
Design: Secondary analysis of the Pediatric Cardiac Critical Care Consortium registry.
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