Update of How Pediatric Cardiologists Noninvasively Evaluate Patients with Hypoplastic Left Heart Syndrome: 2013 vs. 2023.

Noninvasive functional assessment of systemic right ventricles (RV) in hypoplastic left heart syndrome (HLHS) is challenging. This study aimed to compare pediatric cardiologists' current noninvasive imaging practices to those reported in 2013. A web-based survey was distributed to pediatric cardiologists via various listservs which queried timing of echocardiograms during HLHS palliative stages and measurements of RV function.

Outcomes Following Norwood Procedures: Analysis of a "Small Volume" Program.

Background: Institutional survival following Norwood procedures is traditionally correlated with a center's surgical volume. Multiple single and multi-institutional studies conducted at large-volume centers have recently demonstrated improved survival following Norwood procedures. We report both short- and long-term outcomes at a single, small-volume institution and comment on factors potentially influencing outcomes at this institution.

Clinical update on the hybrid comprehensive stage II operation.

Objective: We previously described the hybrid comprehensive stage II operation as an alternate surgical procedure for a subset of patients with single ventricle congenital heart disease with adequate native ascending aortic outflow. Here we provide a clinical update on the 4 patients who have undergone this procedure.

Methods: After undergoing a hybrid approach to the stage I Norwood palliation, the hybrid comprehensive stage II procedure was performed with an incision to the main pulmonary artery (PA), dilation of the ductal stent, creation of a stented baffle between the branch PAs, and a bidirectional Glenn connection.

Outcomes after anomalous aortic origin of a coronary artery repair: A Congenital Heart Surgeons' Society Study.

Objectives: It remains unclear when sudden cardiac event risk outweighs surgical risk for patients with anomalous aortic origin of a coronary artery. The Congenital Heart Surgeons' Society sought to characterize the surgical risks by determining the techniques, complications, and outcomes of repair.

Methods: Between January 2000 and September 2018, 682 patients with anomalous aortic origin of a coronary artery aged 30 years or less were enrolled.

Development of a validated risk score for interstage death or transplant after stage I palliation for single-ventricle heart disease.

Objective: To develop a risk score to predict mortality or transplant in the interstage period.

Background: The "interstage" period between the stage 1 and stage 2 palliation is a time of high morbidity and mortality for infants with single-ventricle congenital heart disease.

Methods: This was an analysis of patients with single-ventricle congenital heart disease requiring arch reconstruction who were enrolled in the National Pediatric Cardiology Quality Improvement Collaborative registry from 2008 to 2015.

Pulmonary artery banding in complete atrioventricular septal defect.

Objectives: To analyze outcomes after pulmonary artery banding (PAB) in complete atrioventricular septal defect (AVSD), with a focus on surgical pathway outcome and timing, survival, and atrioventricular valve function.

Methods: PAB was performed in 50 of 474 infants (11%) from 28 institutions between 2012 and 2018 at a median age of 1.1 months.

Pre-intervention morphologic and functional echocardiographic characteristics of neonates with critical left heart obstruction: a Congenital Heart Surgeons Society (CHSS) inception cohort study.

Aims: The aims of this study were to provide a detailed descriptive analysis of pre-intervention morphologic and functional echocardiographic parameters in a large, unselected, multicentre cohort of neonates diagnosed with critical left heart obstruction and to compare echocardiographic features between the different subtypes of left-sided lesions.

Methods And Results: Pre-intervention echocardiograms for 651 patients from 19 Congenital Heart Surgeons' Society (CHSS) institutions were reviewed in a core lab according to a standardized protocol including >150 morphologic and functional variables. The four most common subtypes of lesions were: aortic atresia (AA)/mitral atresia (MA) (29% of patients), AA/mitral stenosis (MS) (20%), aortic stenosis (AS)/MS (26%), and isolated AS (iAS) (18%).

Determinants of Physician, Sonographer, and Laboratory Productivity: Analysis of the Third Survey from the American Society of Echocardiography Committee on Pediatric Echocardiography Laboratory Productivity.

Background: The American Society of Echocardiography Committee on Pediatric Echocardiography Laboratory Productivity was formed in 2011 to study institutional factors that could influence the clinical productivity of physicians and sonographers in academic pediatric echocardiography laboratories. In the previous two surveys, staff clinical productivity remained stable while total echocardiography volumes increased. This third survey was designed to assess how clinical productivity is associated with laboratory infrastructure elements such as training, administrative tasks, quality improvement, research, and use of focused cardiac ultrasound (FCU).

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.

Surgical Palliation of Right Aortic Arch With an Isolated Innominate Artery From the Left Ductus Arteriosus.

A right aortic arch with an isolated left innominate artery from the left patent ductus arteriosus is a rare arch anomaly, and establishing continuity between the innominate artery and aorta can be challenging. We describe repair of this lesion in a three-week-old male using an autologous pedicle flap of ascending aorta as well as a homograft patch as the roof to recreate continuity between the aorta and left innominate artery.

Development of quality metrics for ambulatory pediatric cardiology: Transposition of the great arteries after arterial switch operation.

Objective: To develop quality metrics (QMs) for the ambulatory care of patients with transposition of the great arteries following arterial switch operation (TGA/ASO).

Design: Under the auspices of the American College of Cardiology Adult Congenital and Pediatric Cardiology (ACPC) Steering committee, the TGA/ASO team generated candidate QMs related to TGA/ASO ambulatory care. Candidate QMs were submitted to the ACPC Steering Committee and were reviewed for validity and feasibility using individual expert panel member scoring according to the RAND-UCLA methodology.

Fontan Fenestration Closure Prior to Posterior Spinal Fusion in Patients With Single-Ventricle Heart Disease.

Study Design: Case series.

Objective: To describe transcatheter closure of the Fontan fenestration prior to posterior spinal fusion in two children to prevent paradoxical venous air embolism during the operation.

Summary Of Background Data: Scoliosis is common among patients with single-ventricle congenital heart disease who have undergone Fontan operation and spinal surgery can offer physiologic benefits.

Determinants of Pediatric Echocardiography Laboratory Productivity: Analysis from the Second Survey of the American Society of Echocardiography Committee on Echocardiography Laboratory Productivity.

Background: The American Society of Echocardiography Committee on Pediatric Echocardiography Laboratory Productivity aimed to study factors that could influence the clinical productivity of physicians and sonographers and assess longitudinal trends for the same. The first survey results indicated that productivity correlated with the total volume of echocardiograms.

Methods: Survey questions were designed to assess productivity for (1) physician full-time equivalent (FTE) allocated to echocardiography reading (echocardiograms per physician FTE per day), (2) sonographer FTE (echocardiograms per sonographer FTE per year), and (3) machine utilization (echocardiograms per machine per year).

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.

Hybrid approach to the comprehensive stage II operation in a subset of single-ventricle variants.

Objective: The objective of a hybrid approach to staged palliation of single-ventricle anomalies is designed to minimize the trauma of the first stage. However, the second stage is a complex procedure that may negate the advantages of the first stage. We sought to devise a "hybrid" approach to the second stage when aortic outflow is expected to remain unobstructed.

Survey of how pediatric cardiologists noninvasively evaluate patients with hypoplastic left heart syndrome.

Background: The evaluation of right ventricular (RV) function is important in patients with hypoplastic left heart syndrome (HLHS). Echocardiographic qualitative grading has been the prevalent method used in the past, but newer technologies allowing for quantitative assessment of RV function may have changed this fact. The goal of this study was to determine the current routine noninvasive evaluation of patients with HLHS and what, if any, methods are used to assess systolic and diastolic function in this population.

Results of the U.S. Food and Drug Administration continued access clinical trial of the GORE HELEX septal occluder for secundum atrial septal defect.

Objectives: This report describes the immediate, 1-, and 5-year follow-up results of the U.S. Food and Drug Administration Continued Access clinical trial of the GORE HELEX Septal Occluder (W.

New imaging modalities in the catheterization laboratory.

Purpose Of Review: To provide an overview of the various modalities that have emerged in the last years as adjunctive tools to provide detailed imaging of the cardiovascular system in the catheterization laboratory.

Recent Findings: Completion angiography after cardiac surgery for congenital heart disease using a dedicated hybrid operating cardiac suite may aid in early diagnosis and therapy of postsurgical abnormalities. The application of three-dimensional rotational digital angiography results in a more precise delineation of the three-dimensional anatomy of vascular structures outside the heart with rapid image processing and reduction of the amount of contrast used and ionizing radiation dose.

Results of the U.S. multicenter pivotal study of the HELEX septal occluder for percutaneous closure of secundum atrial septal defects.

Objectives: This study sought to compare the safety and efficacy of the HELEX septal occluder (HSO) with surgical repair of atrial septal defect (ASD).

Background: The HSO is a low-profile, double-disk occluder device for percutaneous closure of secundum ASD.

Methods: Patients were enrolled (HSO arm prospectively, surgery arm prospectively/retrospectively) from 14 U.