Measuring Acuity and Pediatric Critical Care Nursing Workload by Using ICU CAMEO III.

Background: The Intensive Care Unit Complexity Assessment and Monitoring to Ensure Optimal Outcomes (ICU CAMEO III) acuity tool measures patient acuity in terms of the complexity of nursing cognitive workload.

Objective: To validate the ICU CAMEO III acuity tool in US children's hospitals.

Methods: Using a convenience sample, 9 sites enrolled children admitted to pediatric intensive care units (ICUs).

Accessory pathway ablation in Ebstein anomaly: A challenging substrate.

Background: Catheter ablation of accessory pathways (APs) in Ebstein anomaly (EA) has been associated with a high recurrence risk.

Objective: The purpose of this study was to compare outcomes of AP ablation in EA in an early (1990-2004) vs a recent (2005-2019) era and identify variables associated with recurrence.

Methods: A retrospective review of all catheter ablations for supraventricular tachycardia in EA at our institution was performed.

Safety and Efficacy of Warfarin Therapy in Kawasaki Disease.

Objective: To describe the safety and efficacy of warfarin for patients with Kawasaki disease and giant coronary artery aneurysms (CAAs, ≥8 mm). Giant aneurysms are managed with combined anticoagulation and antiplatelet therapies, heightening risk of bleeding complications.

Study Design: We reviewed the time in therapeutic range; percentage of international normalization ratios (INRs) in range (%); bleeding events, clotting events; INRs ≥6; INRs ≥5 and <6; and INRs <1.

Coronary Artery Aneurysms in Kawasaki Disease: Risk Factors for Progressive Disease and Adverse Cardiac Events in the US Population.

Background: The natural history of coronary artery aneurysms (CAA) after intravenous immunoglobulin (IVIG) treatment in the United States is not well described. We describe the natural history of CAA in US Kawasaki disease (KD) patients and identify factors associated with major adverse cardiac events (MACE) and CAA regression.

Methods And Results: We evaluated all KD patients with CAA at 2 centers from 1979 to 2014.

Mixed aortic valve disease in the young: initial observations.

The short-term surgical results for mixed aortic valve disease (MAVD) and the long-term effects on the left ventricle (LV) are unknown. Retrospective review identified patients with at least both moderate aortic stenosis (AS) and aortic regurgitation (AR) before surgical intervention. A one-to-one comparison cohort of patients with MAVD not referred for surgical intervention was identified.

Acute outcomes after introduction of a standardized clinical assessment and management plan (SCAMP) for balloon aortic valvuloplasty in congenital aortic stenosis.

Introduction: Standardization of care can reduce practice variation, optimize resource utilization, and improve clinical outcomes. We have created a standardized clinical assessment and management plan (SCAMP) for patients having balloon aortic valvuloplasty (BAV) for congenital aortic stenosis (AS). This study compares acute outcomes of BAV at our institution before and after introduction of this SCAMP.

Vascular health in Kawasaki disease.

Objectives: The objective of our study was to compare the indices of vascular health in Kawasaki disease (KD) patients to those of control subjects.

Background: The literature on peripheral vascular health after KD is conflicting.

Methods: Subjects were patients 11 to 29 years of age with the onset of KD >12 months before the study visit (n = 203) and healthy control subjects (n = 50).

Echocardiographic predictors of left ventricular dysfunction after aortic valve surgery in children with chronic aortic regurgitation.

Objective: Postoperative left ventricular dysfunction is associated with poor prognosis in adults with severe chronic aortic regurgitation and published practice guidelines aim to minimize this risk. However, only limited information exists in pediatrics. The goal of this study was to define preoperative risk factors for postoperative left ventricular dysfunction in children with chronic aortic regurgitation.

The Barth Syndrome Registry: distinguishing disease characteristics and growth data from a longitudinal study.

Barth syndrome (BTHS); MIM accession # 302060) is a rare X-linked recessive cardioskeletal mitochondrial myopathy with features of cardiomyopathy, neutropenia, and growth abnormalities. The objectives of this study were to further elucidate the natural history, clinical disease presentation, and course, and describe growth characteristics for males with BTHS. Patients with a confirmed genetic diagnosis of BTHS are referred to the BTHS Registry through the Barth Syndrome Foundation, self-referral, or physician referral.

Surgical outcome for patients with the mitral stenosis-aortic atresia variant of hypoplastic left heart syndrome.

Objective: We sought to identify and characterize a subgroup of patients with hypoplastic left heart syndrome who might be at higher risk for stage I failure.

Methods: From January 2001 through December 2006, all patients with hypoplastic left heart syndrome who underwent stage I palliation at Children's Hospital Boston were retrospectively reviewed. The subgroup with the mitral stenosis-aortic atresia variant was studied separately.

Hypoplastic left heart syndrome with intact or highly restrictive atrial septum: surgical experience from a single center.

Background: The presence of an intact or highly restrictive atrial septum (I/HRAS) has long been recognized as a predictor of poor outcome among patients with hypoplastic left heart syndrome (HLHS), although the rarity of this condition has precluded conclusive study. The purpose of this review is to summarize recent surgical outcomes for these patients at our center and to identify predictors.

Methods: We retrospectively identified all neonates with a diagnosis of HLHS and I/HRAS who underwent stage I palliation at Children's Hospital Boston between January 2001 and December 2006.