Preoperative Aortic Arch Size and Late Outcome After Coarctation Repair by Lateral Thoracotomy.

Background: The approach (lateral thoracotomy versus median sternotomy) to repair coarctation of the aorta is frequently based on arch dimensions from the preoperative echocardiogram. Few studies have assessed the relationship between preoperative arch dimensions and late postoperative outcome. This study aimed to define how preoperative arch dimensions relate to late outcomes and identify long-term predictors of a successful operation.

Optimizing patient care and outcomes through the congenital heart center of the 21st century.

Pediatric cardiovascular services are responding to the dynamic changes in the medical environment, including the business of medicine. The opportunity to advance our pediatric cardiology field through collaboration is now realized, permitting us to define meaningful quality metrics and establish national benchmarks through multicenter efforts. In March 2016, the American College of Cardiology hosted the first Adult Congenital/Pediatric Cardiology Section Congenital Heart Community Day.

Fontan-Associated Liver Disease: Proceedings from the American College of Cardiology Stakeholders Meeting, October 1 to 2, 2015, Washington DC.

Over the past decade, as the majority of patients with single ventricle anatomy who have undergone the Fontan operation reach adulthood, a newly recognized disease process, Fontan-associated liver disease (FALD), has emerged. FALD is an extracardiac complication that may lead to substantial comorbid disease and premature mortality. The risk factors, pathophysiology, longitudinal consequences, and therapeutic options related to FALD remain poorly defined.

The Care of Children With Congenital Heart Disease in Their Primary Medical Home.

Congenital heart disease (CHD) is the most common birth anomaly. With advances in repair and palliation of these complex lesions, more and more patients are surviving and are discharged from the hospital to return to their families. Patients with CHD have complex health care needs that often must be provided for or coordinated for by the primary care provider (PCP) and medical home.

2017 ACC/AAP/AHA Health Policy Statement on Opportunities and Challenges in Pediatric Drug Development: Learning From Sildenafil.

The STARTS-1 and -2 trials (Sildenafil in Treatment-Naive Children, Aged 1 to 17 Years, With Pulmonary Arterial Hypertension) and subsequent 2012 U.S. Food and Drug Administration (FDA) product labeling for sildenafil use in pediatric patients with pulmonary hypertension highlight many of the challenges to the development and approval of medications for children.

2017 ACC/AAP/AHA Health Policy Statement on Opportunities and Challenges in Pediatric Drug Development: Learning From Sildenafil.

The STARTS-1 and -2 trials (Sildenafil in Treatment-Naive Children, Aged 1 to 17 Years, With Pulmonary Arterial Hypertension) and subsequent 2012 U.S. Food and Drug Administration (FDA) product labeling for sildenafil use in pediatric patients with pulmonary hypertension highlight many of the challenges to the development and approval of medications for children.

Variability of antithrombotics use in patients with hypoplastic left heart syndrome and its variants following first- and second-stage palliation surgery: a national report using the National Pediatric Cardiology Quality Improvement Collaborative registry.

Unlabelled: Purpose Patients with hypoplastic left heart syndrome and its variants following palliation surgery are at risk for thrombosis. This study examines variability of antithrombotic practice, the incidence of interstage shunt thrombosis, and other adverse events following Stage I and Stage II palliation within the National Pediatric Cardiology Quality Improvement Collaborative registry.

Methods: We carried out a multicentre, retrospective review using the National Pediatric Cardiology Quality Improvement Collaborative registry including patients from 2008 to 2013 across 52 surgical sites.

Diagnosis of occult diastolic dysfunction late after the Fontan procedure using a rapid volume expansion technique.

Objectives: Diastolic dysfunction (DD), a key driver of long-term Fontan outcomes, may be concealed during standard haemodynamic evaluation. We sought to identify Fontan patients with occult DD using 'ventricular stress testing' with rapid volume expansion (RVE).

Methods: Cardiac catheterisation with RVE was performed routinely in Fontan patients between 11/2012 and 4/2015.

Digoxin Use Is Associated With Reduced Interstage Mortality in Patients With No History of Arrhythmia After Stage I Palliation for Single Ventricle Heart Disease.

Background: Interstage mortality (IM) remains significant after stage 1 palliation (S1P) for single-ventricle heart disease (SVD), with many deaths sudden and unexpected. We sought to determine whether digoxin use post-S1P is associated with reduced IM, utilizing the multicenter database of the National Pediatric Cardiology Quality Improvement Collaborative (NPCQIC).

Methods And Results: From June 2008 to July 2013, 816 infants discharged after S1P from 50 surgical sites completed the interstage to stage II palliation, transplant, or IM.

Transparency in a Pediatric Quality Improvement Collaborative: A Passionate Journey by NPC-QIC Clinicians and Parents.

Transparency-sharing data or information about outcomes, processes, protocols, and practices-may be the most powerful driver of health care improvement. In this special article, the development and growth of transparency within the National Pediatric Cardiology Quality Improvement Collaborative is described. The National Pediatric Cardiology Quality Improvement Collaborative transparency journey is guided by equal numbers of clinicians and parents of children with congenital heart disease working together in a Transparency Work Group.

Adjusting for Risk Associated With Pediatric and Congenital Cardiac Catheterization: A Report From the NCDR IMPACT Registry.

Background: As US health care increasingly focuses on outcomes as a means for quantifying quality, there is a growing demand for risk models that can account for the variability of patients treated at different hospitals so that equitable comparisons between institutions can be made. We sought to apply aspects of prior risk-standardization methodology to begin development of a risk-standardization tool for the National Cardiovascular Data Registry (NCDR) IMPACT (Improving Pediatric and Adult Congenital Treatment) Registry.

Methods And Results: Using IMPACT, we identified all patients undergoing diagnostic or interventional cardiac catheterization between January 2011 and March 2013.

Characteristics and safety of interventions and procedures performed during catheterisation of patients with congenital heart disease: early report from the national cardiovascular data registry.

Objectives: The objective of this study was to report procedural characteristics and adverse events on the data collected in the IMproving Paediatric and Adult Congenital Treatment registry.

Background: The IMproving Paediatric and Adult Congenital Treatment- registry is a catheterisation registry focussed on paediatric and adult patients with congenital heart disease who are undergoing diagnostic catheterisations and catheter-based interventions. This study reports procedural characteristics and adverse events of patients who have undergone selected catheterisation procedures from January, 2011 to June, 2013.

Practice Variation in Single-Ventricle Patients Undergoing Elective Cardiac Catheterization: A Report from the Congenital Cardiac Catheterization Project on Outcomes (C3PO).

Objective: The objective of this study was to investigate variation in practice surrounding elective cardiac catheterization in patients with single-ventricle (SV) congenital heart disease.

Design: Patient and procedural characteristics and outcomes during SV catheterization were collected prospectively from eight centers using a web-based registry (Congenital Cardiac Catheterization Project on Outcomes). We attempted to identify a population of elective procedures by limiting the cohort in case type and timing.

Association of Interstage Home Monitoring With Mortality, Readmissions, and Weight Gain: A Multicenter Study from the National Pediatric Cardiology Quality Improvement Collaborative.

Background: Daily home monitoring of oxygen saturation and weight has been reported to improve outcomes for patients with single-ventricle heart disease during the period between stage I palliation and stage II palliation. However, these studies have been limited to single institutions and used historical control subjects. Our objective was to determine the association of various interstage home monitoring strategies with outcomes using a multicenter cohort with contemporary control subjects.

Quality improvement through collaboration: the National Pediatric Quality improvement Collaborative initiative.

Purpose Of Review: The National Pediatric Quality Improvement Collaborative (NPCQIC) was established to improve outcomes and quality of life in children with hypoplastic left heart syndrome and other single ventricle lesions requiring a Norwood operation. The NPCQIC consists of a network of providers and families collecting longitudinal data, conducting research, and using quality improvement science to decrease variations in care, develop and spread best practices, and decrease mortality.

Recent Findings: Initial descriptive investigation of the collaborative data found interstage care process variations, different surgical strategies, diverse feeding practices, and variable ICU approaches between centers and within sites.

Balloon valvuloplasty for congenital aortic stenosis: Multi-center safety and efficacy outcome assessment.

Objective: To describe contemporary outcomes of balloon aortic valvuloplasty (BAVP) performed in 22 US centers.

Background: BAVP constitutes first-line therapy for congenital aortic stenosis (cAS) in many centers.

Methods: We used prospectively-collected data from two active, multi-institutional, pediatric cardiac catheterization registries.

Procedural characteristics and adverse events in diagnostic and interventional catheterisations in paediatric and adult CHD: initial report from the IMPACT Registry.

Objectives: To report procedural characteristics and adverse events on data collected in the registry.

Background: The IMPACT--IMproving Paediatric and Adult Congenital Treatment--Registry is a catheterisation registry of paediatric and adult patients with CHD undergoing diagnostic and interventional cardiac catheterisation. We are reporting the procedural characteristics and adverse events of patients undergoing diagnostic and interventional catheterisation procedures from January, 2011 to March, 2013.

Procedural results and safety of common interventional procedures in congenital heart disease: initial report from the National Cardiovascular Data Registry.

Background: The National Cardiovascular Data Registry (NCDR) launched the IMPACT (Improving Pediatric and Adult Congenital Treatment) Registry in 2010. By 2013, its patient enrollment exceeded that of other current and historical congenital catheterization registries.

Objectives: This study sought to describe procedural results and safety of 6 common congenital interventions performed in patients enrolled during the IMPACT Registry's initial periods.

A new predictive equation for oxygen consumption in children and adults with congenital and acquired heart disease.

Objective: To develop a new predictive equation for oxygen consumption (VO2) in children and adults with congenital and acquired heart disease.

Methods: We retrospectively reviewed data from 502 consecutive patients (age 0-59 years) undergoing cardiac catheterisation with measured VO2 (M-VO2) and compared M-VO2 with VO2 from the LaFarge equations (LF-VO2) in patients <3 years (Group 1) and ≥3 years (Group 2). Factors associated with inaccurate LF-VO2 were used to develop a new predictive equation, which was prospectively validated in 100 consecutive patients (age 0-59 years).

Use of a learning network to improve variation in interstage weight gain after the Norwood operation.

Background: Growth failure is common in infants with single ventricle. This study evaluated the use of a learning network, the National Pediatric Cardiology Quality Improvement Collaborative (NPC-QIC), to spread optimized nutritional practices and improve infant growth.

Methods: A previously identified Nutritional Bundle was spread among NPC-QIC sites.

Impact of Percutaneous Interventions for Pulmonary Artery Stenosis in Alagille Syndrome.

Objective: The study aims to examine acute and midterm outcomes after percutaneous interventions for treatment of pulmonary artery stenosis (PAS) in patients with Alagille Syndrome (ALGS).

Background: PAS affects up to two thirds of ALGS patients. Responsiveness to transcatheter therapies may differ from other causes of PAS.

Impact of prenatal diagnosis in survivors of initial palliation of single ventricle heart disease: analysis of the National Pediatric Cardiology Quality Improvement Collaborative database.

Among infants with single ventricle congenital heart disease (SVD) requiring Stage I palliation (S1P), the impact of prenatal diagnosis (PD) on outcomes has been variably characterized. We investigated the impact of PD in a large multi-center cohort of survivors of S1P in the National Pediatric Cardiology Quality Improvement Collaborative (NPCQIC) registry. Retrospective analysis of demographic and outcomes data among infants enrolled in the NPCQIC database; eligibility includes SVD requiring S1P and survival to discharge.