Absorbable metal stents for vascular use in pediatric cardiology: progress and outlook.

The past five years have yielded impressive advancements in fully absorbable metal stent technology. The desired ultimate ability for such devices to treat a vascular stenosis without long-term device-related complications or impeding future treatment continues to evoke excitement in clinicians and engineers alike. Nowhere is the need for fully absorbable metal stents greater than in patients experiencing vascular anomalies associated with congenital heart disease (CHD).

sVEGFR1 Is Enriched in Hepatic Vein Blood-Evidence for a Provisional Hepatic Factor Candidate?

Pulmonary arteriovenous malformations (PAVMs) are common sequelae of palliated univentricular congenital heart disease, yet their pathogenesis remain poorly defined. In this preliminary study, we used paired patient blood samples to identify potential hepatic factor candidates enriched in hepatic vein blood. Paired venous blood samples were collected from the hepatic vein (HV) and superior vena cava (SVC) from children 0 to 10 years with univentricular and biventricular congenital heart disease ( = 40).

Congenital right internal mammary artery to portal vein arteriovenous malformation.

Neonatal cases of systemic artery to portal venous system arteriovenous malformations (AVMs) can present unique challenges in terms of diagnosis, management, and treatment. Prompt identification of these AVMs is necessary for minimizing long-term sequelae and optimizing prognosis. Our report describes the diagnosis and successful endovascular coil embolization of a congenital right internal mammary artery (IMA) to portal vein AVM in a young infant initially presenting during routine fetal screening with an incidentally discovered congenital thoracic vascular abnormality.

Hepatic Vein Blood Increases Lung Microvascular Angiogenesis and Endothelial Cell Survival-Toward an Understanding of Univentricular Circulation.

To improve our understanding of pulmonary arteriovenous malformations in univentricular congenital heart disease, our objective was to identify the effects of hepatic vein and superior vena cava constituents on lung microvascular endothelial cells independent of blood flow. Paired blood samples were collected from the hepatic vein and superior vena cava in children 0-10 years old undergoing cardiac catheterization. Isolated serum was subsequently used for in vitro endothelial cell assays.

Cardiac Arrest Secondary to Traumatic Aortopulmonary Window During Transcatheter Pulmonary Valve Implantation in Supported Ross.

We present a patient with a supported Ross procedure and severe pulmonary homograft stenosis who developed cardiac arrest while undergoing transcatheter pulmonary valve replacement and was found to have a large iatrogenic aortopulmonary window. Cardiopulmonary resuscitation was initiated followed by covered stent placement, extracorporeal membrane oxygenation support, and ultimately emergent surgery with a good outcome. ().

Radiation Risk Categories in Cardiac Catheterization for Congenital Heart Disease: A Tool to Aid in the Evaluation of Radiation Outcomes.

To stratify diverse procedure types into categories with similar radiation exposure in cardiac catheterization for congenital heart disease. Radiation exposures for a comprehensive list of specific procedure types and stratification of outcomes based on radiation risk are not currently available. Data between January 2014 and December 2015 were collected on all cases performed at sites participating in C3PO-QI (Congenital Cardiac Catheterization Outcomes Project-Quality Improvement Initiative) and 9 centers were included.

Ventricular Fibrillation and Myocardial Depression Following Pulmonary Valve Replacement in Tetralogy of Fallot With an Intramural Coronary Artery and Coronary Artery Compression by Chest Tube.

We describe the case of a 10-year-old male with a history of repaired Tetralogy of Fallot and known intramural right coronary artery (RCA) who presented for bioprosthetic pulmonary valve replacement. The operation was complicated by postoperative ventricular fibrillation arrest. Selective coronary angiography revealed external compression of the mid-RCA by a mediastinal chest tube that improved immediately upon removal of the tube.

Acute Success of Balloon Aortic Valvuloplasty in the Current Era: A National Cardiovascular Data Registry Study.

Objectives: The aim of this study was to evaluate practice patterns and outcomes of a contemporary group of patients undergoing balloon aortic valvuloplasty (BAV) for congenital aortic stenosis (AS).

Background: BAV is the most common treatment for isolated congenital AS.

Methods: Within the IMPACT (Improving Pediatric and Adult Congenital Treatments) Registry, all BAV procedures performed between January 2011 and March 2015 were identified.

Melody® pulmonary valve implantation in two teenage patients with congenitally corrected transposition of the great arteries status after Senning atrial switch operation.

The Melody® transcatheter pulmonary valve system was developed for placement within right ventricle-to-pulmonary artery conduits in patients with CHD for treatment of stenosis or regurgitation, providing an alternative to open-heart surgery. Abnormal systemic venous connections altering the catheter course to the right ventricle-to-pulmonary artery conduit may present a challenge to Melody® valve implantation. We present two such cases, in which the Melody® valve was successfully implanted in teenage patients with congenitally corrected transposition of the great arteries after Senning atrial switch operation.

Implementation of Methodology for Quality Improvement in Pediatric Cardiac Catheterization: A Multi-center Initiative by the Congenital Cardiac Catheterization Project on Outcomes-Quality Improvement (C3PO-QI).

The Congenital Cardiac Catheterization Project on Outcomes (C3PO) launched in 2007 as a multi-center collaborative to establish standardized and comparable metrics for pediatric cardiac catheterization procedures. The limitations of larger registries at the time led to the development of the next phase in 2013, C3PO-Quality Improvement (C3PO-QI), focusing on instituting QI initiatives within the field. The objective of this manuscript is to provide a detailed overview of C3PO-QI and report data on case characteristics and outcome metrics being explored.

Dual-Axis Rotational Angiography is Safe and Feasible to Detect Coronary Allograft Vasculopathy in Pediatric Heart Transplant Patients: A Single-Center Experience.

Coronary allograft vasculopathy (CAV) is the leading cause of graft failure in pediatric heart transplant recipients, also adding to mortality in this patient population. Coronary angiography is routinely performed to screen for CAV, with conventional single-plane or bi-plane angiography being utilized. Dual-axis rotational coronary angiography (RA) has been described, mostly in the adult population, and may offer reduction in radiation dose and contrast volume.

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.

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.

Safety of percutaneous patent ductus arteriosus closure: an unselected multicenter population experience.

Background: The technique and safety of transcatheter patent ductus arteriosus (PDA) closure have evolved during the past 20 years. We sought to report a multicenter experience of PDA closure with a focus on the rate of adverse events (AE) and a review of institutional practice differences.

Methods And Results: Outcome data on transcatheter PDA closure were collected at 8 centers prospectively using a multicenter registry (Congenital Cardiac Catheterization Project on Outcome Registry).

Catheterization for Congenital Heart Disease Adjustment for Risk Method (CHARM).

Objectives: This study sought to develop a method to adjust for case mix complexity in catheterization for congenital heart disease to allow equitable comparisons of adverse event (AE) rates.

Background: The C3PO (Congenital Cardiac Catheterization Project on Outcomes) has been prospectively collecting data using a Web-based data entry tool on all catheterization cases at 8 pediatric institutions since 2007.

Methods: A multivariable logistic regression model with high-severity AE outcome was built using a random sample of 75% of cases in the multicenter cohort; the models were assessed in the remaining 25%.

Ventricular remodeling and survival are more favorable for myocarditis than for idiopathic dilated cardiomyopathy in childhood: an outcomes study from the Pediatric Cardiomyopathy Registry.

Background: Myocarditis is a cause of a new-onset dilated cardiomyopathy phenotype in children, with small studies reporting high rates of recovery of left ventricular (LV) function.

Methods And Results: The presenting characteristics and outcomes of children with myocarditis diagnosed clinically and with biopsy confirmation (n=119) or with probable myocarditis diagnosed clinically or by biopsy alone (n=253) were compared with children with idiopathic dilated cardiomyopathy (n=1123). Characteristics at presentation were assessed as possible predictors of outcomes.

Pediatric heart failure therapy with beta-adrenoceptor antagonists.

Management of chronic heart failure in pediatrics has been altered by the adult literature showing improvements in mortality and hospitalization rates with the use of beta-adrenoceptor antagonists (beta-blockers) for routine therapy of all classes of ischemic and non-ischemic heart failure. Many pediatric heart failure specialists have incorporated these agents into their routine management of pediatric heart failure related to dilated cardiomyopathy or ventricular dysfunction in association with congenital heart disease. Retrospective and small prospective case series have shown encouraging improvements in cardiac function and symptoms, but interpretation has been complicated by the high rate of spontaneous recovery in pediatric patients.

Importance of totally anomalous pulmonary venous connection and postoperative pulmonary vein stenosis in outcomes of heterotaxy syndrome.

Historically, outcomes of patients with heterotaxy syndrome and congenital heart disease have been poor. Published series include patients treated over many decades or focus on specific patient/operative subgroups. This study was performed to evaluate midterm outcomes and determine anatomic risk factors for death in patients with all types of heterotaxy syndrome treated after 1985.