Extracorporeal Membrane Oxygenation in Children with Pulmonary Atresia and Intact Ventricular Septum: Mortality and Associated Outcomes.

Data on outcomes of extracorporeal membrane oxygenation (ECMO) are limited in patients with pulmonary atresia intact ventricular septum (PAIVS). The objective of this study was to describe the use of ECMO and the associated outcomes in patients with PAIVS. We retrospectively reviewed neonates with PAIVS who received ECMO between 2009 and 2019 in 19 US hospitals affiliated with the Collaborative Research for the Pediatric Cardiac Intensive Care Society (CoRe-PCICS).

Comparison of Ductal Stent Versus Surgical Shunt as Initial Intervention for Neonates with Pulmonary Atresia with Intact Ventricular Septum.

Data comparing surgical systemic-to-pulmonary artery shunt and patent ductus arteriosus (PDA) stent as the initial palliation procedure for patients with pulmonary atresia with intact ventricular septum (PA-IVS) are limited. We sought to compare characteristics and outcomes in a multicenter cohort of patients with PA-IVS undergoing surgical shunts versus PDA stents. We retrospectively reviewed neonates with PA-IVS from 2009 to 2019 in 19 United States centers.

Procedural Outcomes of Pulmonary Atresia With Intact Ventricular Septum in Neonates: A Multicenter Study.

Background: Multicenter contemporary data describing short-term outcomes after initial interventions of neonates with pulmonary atresia with intact ventricular septum (PA-IVS) are limited. This multicenter study describes characteristics and outcomes of PA-IVS neonates after their initial catheter or surgical intervention and identifies factors associated with major adverse cardiac events (MACE).

Methods: Neonates with PA-IVS who underwent surgical or catheter intervention between 2009 and 2019 in 19 centers were reviewed.

Pulmonary Atresia with Intact Ventricular Septum: Midterm Outcomes from a Multicenter Cohort.

Contemporary multicenter data regarding midterm outcomes for neonates with pulmonary atresia with intact ventricular septum are lacking. We sought to describe outcomes in a contemporary multicenter cohort, determine factors associated with end-states, and evaluate the effect of right ventricular coronary dependency and coronary atresia on transplant-free survival. Neonates treated during 2009-2019 in 19 United States centers were reviewed.

Anomalous Origin of the Left Coronary Artery From the Pulmonary Artery: A Retrospective Multicenter Study.

Objectives: We aimed to describe characteristics and operative outcomes from a multicenter cohort of infants who underwent repair of anomalous left coronary artery from the pulmonary artery. We also aimed to identify factors associated with major adverse cardiovascular events following anomalous left coronary artery from the pulmonary artery repair.

Design: Retrospective chart review.

Characteristics and Surgical Outcomes of Patients With Late Presentation of Anomalous Left Coronary Artery From the Pulmonary Artery: A Multicenter Study.

We sought to describe the clinical course and outcomes of patients who are diagnosed with anomalous left coronary artery from the pulmonary artery (ALCAPA) after infancy. We conducted a retrospective evaluation of patients who underwent ALCAPA surgery between January 2009 to March 2018 at 21 US centers. Clinical presentation, inpatient management, and postoperative outcomes of patients repaired ≥1 year of age were described.

Pulmonary hypertension and right heart failure due to severe hypernatremic dehydration.

Introduction: Neonates are at risk of developing hypernatremic dehydration and its associated complications, such as stroke, dural sinus thrombosis and renal vein thrombosis. Pulmonary hypertension has not been described as a complication of hypernatremia.

Case Report: We report a case of a seven-day-old neonate with severe hypernatremic dehydration who went on to develop pulmonary hypertension and right heart failure needing extracorporeal membrane oxygenation (ECMO).

A novel surfactant protein C gene mutation associated with progressive respiratory failure in infancy.

Mutations of the Surfactant Protein C (SPC) gene (SFTPC) have been associated with childhood interstitial lung disease (chILD) with variable age of onset, severity of lung disease, and outcomes. We report a novel mutation in SFTPC [c.435G->A, p.

Clinical and genetic profile of children with periodic fever syndromes from a single medical center in South East Michigan.

Objective: To report a cohort of children with periodic fever syndromes (PFS) from Southeast Michigan.

Methods: A retrospective review of medical records for patients referred for periodic fever over 5 years.

Results: Sixty-six patients including 21 FMF, 15 PFAPA, four TRAPS and one patient with combined HIDS and FMF were included.

A child with altered sensorium, hyperglycemia, and elevated troponins.

Background: Diabetic ketoacidosis (DKA) and hyperglycemic hyperosmolar state (HHS) are potentially life-threatening complications of diabetes mellitus. Although DKA and HHS share similar features, they are distinct clinical entities requiring different treatment measures.

Objective: This case illustrates that the clinical distinction between these two entities can be difficult at times, especially in children who can present with an overlapping picture.