Multicenter Pivotal Study of the Alterra Adaptive Prestent for the Treatment of Pulmonary Regurgitation.

Background: A dilated native right ventricular outflow tract (RVOT) presents unique challenges for transcatheter management using balloon-expandable valves. The Alterra Adaptive Prestent was designed to expand transcatheter therapy to treat patients with dilated RVOTs.

Objectives: The aim of this study was to report 2-year outcomes of the main cohort of the ALTERRA (Multicenter Study of Congenital Pulmonic Valve Dysfunction Studying the SAPIEN 3 THV With the Alterra Adaptive Prestent) pivotal trial using the prestent with transcatheter pulmonary valve replacement.

Angiographic tool to detect pulmonary arteriovenous malformations in single ventricle physiology.

Objective: Individuals with single ventricle physiology who are palliated with superior cavopulmonary anastomosis (Glenn surgery) may develop pulmonary arteriovenous malformations. The traditional tools for pulmonary arteriovenous malformation diagnosis are often of limited diagnostic utility in this patient population. We sought to measure the pulmonary capillary transit time to determine its value as a tool to identify pulmonary arteriovenous malformations in patients with single ventricle physiology.

Angiographic Tool to Detect Pulmonary Arteriovenous Malformations in Single Ventricle Physiology.

Background: Individuals with single ventricle physiology who are palliated with superior cavopulmonary anastomosis (Glenn surgery) may develop pulmonary arteriovenous malformations (PAVMs). The traditional tools for PAVM diagnosis are often of limited diagnostic utility in this patient population. We sought to measure the pulmonary capillary transit time (PCTT) to determine its value as a tool to identify PAVMs in patients with single ventricle physiology.

Diagnosis and Management of Congenital Coronary Artery Fistulas in Infants and Children.

Purpose Of Review: Coronary artery fistulas (CAFs) are rare coronary anomalies that most often occur as congenital malformations in children. Although most children with CAFs are asymptomatic at the time of diagnosis, some present with symptoms of congestive heart failure in the setting of large left-to-right shunts. Others may develop additional complications including coronary artery ectasia and coronary thrombosis.

Transcatheter Device Therapy and the Integration of Advanced Imaging in Congenital Heart Disease.

Transcatheter device intervention is now offered as first line therapy for many congenital heart defects (CHD) which were traditionally treated with cardiac surgery. While off-label use of devices is common and appropriate, a growing number of devices are now specifically designed and approved for use in CHD. Advanced imaging is now an integral part of interventional procedures including pre-procedure planning, intra-procedural guidance, and post-procedure monitoring.

Role of Cross-Sectional Imaging in Pediatric Interventional Cardiac Catheterization.

Management of congenital heart disease (CHD) has recently increased utilization of cross-sectional imaging to plan percutaneous interventions. Cardiac computed tomography (CT) and cardiac magnetic resonance (CMR) imaging have become indispensable tools for pre-procedural planning prior to intervention in the pediatric cardiac catheterization lab. In this article, we review several common indications for referral and the impact of cross-sectional imaging on procedural planning, success, and patient surveillance.

Pre-procedural CT imaging aids neonatal PDA stenting for ductal-dependent pulmonary blood flow with reduction in overall procedural morbidity.

Patent ductus arteriosus stenting for ductal-dependent pulmonary blood flow is a technically challenging neonatal procedure to maintain a stable pulmonary circulation. Pre-procedural computed tomography imaging aids in outlining ductal origin, insertion, size, course and curvature. Computed tomography imaging may add value to procedural outcomes and reduce overall procedural morbidity in neonatal patent ductus arteriosus stenting.

Comparison of the investigational device exemption and post-approval trials of the Melody transcatheter pulmonary valve.

Objective: We compared 5-year outcomes of transcatheter pulmonary valve (TPV) replacement with the Melody TPV in the post-approval study (PAS) and the investigational device exemption (IDE) trial.

Background: As a condition of approval of the Melody TPV after the IDE trial, the Food and Drug Administration required that a PAS be conducted to evaluate outcomes of TPV replacement in a "real-world" environment. The 5-year outcomes of the PAS have not been published, and the IDE and PAS trials have not been compared.

Use of institutional criteria for transcatheter device closure of Fontan fenestration - Midterm outcomes.

Background: There are no established criteria to decide suitability for Fontan fenestration closure. Our institution has the following criteria: an unobstructed Fontan pathway with no significant decompressing venovenous collaterals, baseline Fontan pressure ≤15 mmHg, baseline cardiac index ≥2 L/min/m, and a decrease in cardiac index ≤20% with test occlusion of the fenestration.

Objective: The objective of the study was to review midterm outcomes following device closure of Fontan fenestration using institutional criteria.

ACCAPA: anomalous circumflex coronary artery origin from pulmonary artery.

Anomalous circumflex coronary artery origin from pulmonary artery is a very rare congenital heart disease. Misdiagnosis of this condition may lead to inadequate myocardial protection during cardiopulmonary bypass surgery.

3D advanced imaging overlay with rapid registration in CHD to reduce radiation and assist cardiac catheterisation interventions.

Novel commercially available software has enabled registration of both CT and MRI images to rapidly fuse with X-ray fluoroscopic imaging. We describe our initial experience performing cardiac catheterisations with the guidance of 3D imaging overlay using the VesselNavigator system (Philips Healthcare, Best, NL). A total of 33 patients with CHD were included in our study.

Two-dimensional systolic speckle tracking echocardiography provides a noninvasive aid in the identification of acute pediatric heart transplant rejection.

Background: Acute rejection is an important cause of morbidity and mortality in the pediatric heart transplant (HT) population. A reliable noninvasive method for diagnosis of clinical rejection could substantially reduce these negative outcomes.

Objective: Evaluate left ventricular (LV) global longitudinal strain (GLS), and global circumferential strain (GCS) as early noninvasive indicators of acute pediatric HT rejection.

Association between patient age at implant and outcomes after transcatheter pulmonary valve replacement in the multicenter Melody valve trials.

Objectives: To investigate whether age and valve size at implant contribute to outcomes after Melody transcatheter pulmonary valve replacement (TPVR).

Background: Patient age and valve size at implant contribute to longevity of surgical pulmonary valves.

Methods: All patients discharged with a Melody valve in the pulmonary position, as part of three prospective Melody valve multicenter studies, comprised the study cohort.

Acute and Midterm Outcomes of Transcatheter Pulmonary Valve Replacement for Treatment of Dysfunctional Left Ventricular Outflow Tract Conduits in Patients With Aortopulmonary Transposition and a Systemic Right Ventricle.

Background: Transcatheter pulmonary valve replacement (TPVR) is an established therapy for dysfunctional right ventricular (RV) outflow tract conduits. TPVR in patients with congenitally corrected transposition of the great arteries, subpulmonary left ventricle, and left ventricular outflow tract (LVOT) conduit dysfunction has not been studied. Unique anatomic and physiological aspects of this population may contribute to distinct risks and outcomes.

Invasive Hemodynamics of Adult Congenital Heart Disease: From Shunts to Coarctation.

Adults with congenital heart disease are a growing population with increasingly more complex disease, in large part due to improvements in delivery of care to the pediatric population. Cardiac catheterization is an integral component of diagnosis and management in these patients. Careful attention to detail and a thorough understanding of intracardiac hemodynamics are critical to performing complete diagnostic evaluations.

Initial Experience With the Pediatric Impella Device: A Feasibility Study in a Porcine Model.

Objective: This study's objective was to evaluate insertion techniques and device fit of the pediatric version of the Impella ventricular assist device in swine which had similar sized carotids and left ventricles (LVs) as children weighing 10-20 kg.

Background: Options for minimally invasive circulatory support in children are limited. A modified device based on the current Impella 2.

One-year follow-up of the Melody transcatheter pulmonary valve multicenter post-approval study.

Objectives: This study sought to confirm that the short-term hemodynamic effectiveness of the Melody transcatheter pulmonary valve (TPV) (Medtronic, Inc., Minneapolis, Minnesota) achieved by real-world providers is equivalent to the historical results established in the initial 5-center Investigational Device Exemption trial.

Background: TPV replacement has been used to treat right ventricular outflow tract (RVOT) conduit dysfunction for >10 years.

Management and outcomes of heterotaxy syndrome associated with pulmonary atresia or pulmonary stenosis.

Background: Historic outcomes of patients with heterotaxy and pulmonary atresia or pulmonary stenosis (PA/PS) have been poor and in the current era are incompletely described. We reviewed our management of these patients and associated risk factors for death.

Methods: We retrospectively reviewed the records of all patients with heterotaxy and PA/PS treated in our institution from January 1, 2002, to August 31, 2012.

Comparison of surgical, stent, and balloon angioplasty treatment of native coarctation of the aorta: an observational study by the CCISC (Congenital Cardiovascular Interventional Study Consortium).

Objectives: The purpose of this study was to compare the safety and efficacy of surgical, stent, and balloon angioplasty (BA) treatment of native coarctation acutely and at follow-up.

Background: Controversy surrounds the optimal treatment for native coarctation of the aorta. This is the first multicenter study evaluating acute and follow-up outcomes of these 3 treatment options in children weighing >10 kg.

Stenting of aortic coarctation: acute, intermediate, and long-term results of a prospective multi-institutional registry--Congenital Cardiovascular Interventional Study Consortium (CCISC).

Introduction: Since the 1980s, stent implantation has evolved as an important therapeutic strategy for coarctation of the aorta. However, available data is frequently flawed by short follow-up, lack of adequate follow-up imaging, and retrospective nature of data collection.

Methods: Data was prospectively collected using a multicenter registry congenital cardiovascular interventional study consortium (CCISC).