Coronary artery ectasia in post-pericardiotomy syndrome.

Post-pericardiotomy syndrome (PPS) is a common inflammatory process following cardiac surgery, in which the pericardial space was opened. Pericardial effusion (PE) is a common manifestation in PPS; however, coronary artery dilation is not associated with PPS. Inflammatory vasculitis in children are known to cause coronary dilation, in conditions such as in Kawasaki Disease (KD).

Contemporary Outcomes in Tetralogy of Fallot With Absent Pulmonary Valve After Fetal Diagnosis.

Background Tetralogy of Fallot with absent pulmonary valve is associated with high mortality, but it remains difficult to predict outcomes prenatally. We aimed to identify risk factors for mortality in a large multicenter cohort. Methods and Results Fetal echocardiograms and clinical data from 19 centers over a 10-year period were collected.

Risk Factors for Mortality and Circulatory Outcome Among Neonates Prenatally Diagnosed With Ebstein Anomaly or Tricuspid Valve Dysplasia: A Multicenter Study.

Background In a recent multicenter study of perinatal outcome in fetuses with Ebstein anomaly or tricuspid valve dysplasia, we found that one third of live-born patients died before hospital discharge. We sought to further describe postnatal management strategies and to define risk factors for neonatal mortality and circulatory outcome at discharge. Methods and Results This 23-center, retrospective study from 2005 to 2011 included 243 fetuses with Ebstein anomaly or tricuspid valve dysplasia.

Challenges With Left Ventricular Functional Parameters: The Pediatric Heart Network Normal Echocardiogram Database.

Background: The reliability of left ventricular (LV) systolic functional indices calculated from blinded echocardiographic measurements of LV size has not been tested in a large cohort of healthy children. The objective of this study was to estimate interobserver variability in standard measurements of LV size and systolic function in children with normal cardiac anatomy and qualitatively normal function.

Methods: The Pediatric Heart Network Normal Echocardiogram Database collected normal echocardiograms from healthy children ≤18 years old distributed equally by age, gender, and race.

Natural history of echocardiographic abnormalities in mucopolysaccharidosis III.

Background: Mucopolysaccharidosis (MPS) type III, Sanfilippo Syndrome, is an autosomal recessive lysosomal storage disorder. MPS I and II patients often develop cardiac involvement leading to early mortality, however there are limited data in MPS III. The objective of this study is to describe cardiac abnormalities in a large group of MPS III patients followed in a longitudinal natural history study designed to determine outcome measures for gene transfer trials.

Abnormal Longitudinal Growth of the Aorta in Children with Familial Bicuspid Aortic Valve.

Bicuspid aortic valve (BAV) is the most common type of congenital heart defect (CHD) and is associated with clinically significant cardiovascular complications including valve calcification and ascending aortopathy (AscAo), predominantly occurring in adulthood. While a limited number of genetic etiologies for BAV have been defined, family members of affected individuals display BAV along with other left-sided CHD. This has led to guidelines from the American Heart Association and American College of Cardiology that recommend echocardiographic screening of first-degree relatives of affected adults.

Patterns of Fever in Children After Primary Treatment for Kawasaki Disease.

Background: We sought to determine if fever in the early postintravenous immunoglobulin (IVIG) time period (first 36 hours after IVIG completion) for Kawasaki disease, with or without additional infliximab, can predict IVIG resistance and coronary artery abnormalities (CAA).

Methods: Acute Kawasaki disease subjects enrolled in a clinical trial of infliximab plus IVIG (n = 96) versus placebo/IVIG (n = 94) had temperatures recorded every 6 hours after completion of IVIG infusion. Fever was defined as temperature >38.

Outcomes and Predictors of Perinatal Mortality in Fetuses With Ebstein Anomaly or Tricuspid Valve Dysplasia in the Current Era: A Multicenter Study.

Background: Ebstein anomaly and tricuspid valve dysplasia are rare congenital tricuspid valve malformations associated with high perinatal mortality. The literature consists of small, single-center case series spanning several decades. We performed a multicenter study to assess the outcomes and factors associated with mortality after fetal diagnosis in the current era.

Infliximab for intensification of primary therapy for Kawasaki disease: a phase 3 randomised, double-blind, placebo-controlled trial.

Background: Kawasaki disease, the most common cause of acquired heart disease in developed countries, is a self-limited vasculitis that is treated with high doses of intravenous immunoglobulin. Resistance to intravenous immunoglobulin in Kawasaki disease increases the risk of coronary artery aneurysms. We assessed whether the addition of infliximab to standard therapy (intravenous immunoglobulin and aspirin) in acute Kawasaki disease reduces the rate of treatment resistance.

Incidence of tracheobronchial anomalies found with hypoplastic left heart syndrome.

Background: Tracheobronchial anomalies are rare congenital malformations that are typically managed conservatively. Several reports have documented their increased incidence in patients with congenital heart disease. However, none of these reports have detailed the incidence found among patients with hypoplastic left heart syndrome (HLHS).

Predictors of ductus arteriosus in-stent stenosis in the hybrid approach to hypoplastic left heart syndrome.

The hybrid approach to palliation of hypoplastic left heart syndrome using pulmonary artery bands, a patent ductus arteriosus (PDA) stent, and atrial septostomy has been well described. One potential complication of hybrid stage 1 palliation is the development of neointimal formation and in-stent stenosis (ISS). This study aimed to identify predictors of ISS development.

Strain echocardiographic assessment of ventricular function after percutaneous pulmonary valve implantation.

Introduction: Percutaneous pulmonary valve implantation (PPVI) is an emerging therapy for pulmonary valve dysfunction. Minimal data on the midterm effects of PPVI on ventricular function exist. We describe the effects of PPVI on right and left ventricular (RV, LV) function with speckle tracking echocardiography.

Histopathologic evaluation of patent ductus arteriosus stents after hybrid stage I palliation.

The aim of this study was to determine the histopathology of patent ductus arteriosus (PDA) in-stent stenosis after hybrid stage I palliation. The hybrid approach to palliation of hypoplastic left heart syndrome can be complicated by the development of in-stent stenosis of the PDA. This may obstruct retrograde aortic arch flow, decrease systemic circulation, and lead to interstage interventional procedures.

Predictors of retrograde aortic arch obstruction after hybrid palliation of hypoplastic left heart syndrome.

A potential complication after hybrid stage 1 palliation for hypoplastic left heart syndrome (HLHS) is retrograde aortic arch obstruction (RAAO). This can lead to increased morbidity and unplanned surgical or interventional procedures in the interstage period. This study aimed to identify potential predictors of RAAO by analyzing initial echocardiograms and angiograms before hybrid stage 1 palliation.

Late thrombus formation on the Helex septal occluder after double-lung transplant.

Percutaneous device closure in patients with an atrial septal defect (ASD) or patent foramen ovale (PFO) has gained popularity because of the short learning curve, cosmetic advantage and relative safety compared with surgery. Device complications may include device embolism, erosion, pericardial tamponade or thrombus formation, and most complications occur early. Herein we describe the previously unreported finding of a late thrombus on a Helex device after PFO closure in a patient with cystic fibrosis and double-lung transplantation.

Amplatzer septal occluder associated with late pulmonary venous obstruction requiring surgical removal with acquired aorta to left atrial fistula.

The Amplatzer septal occluder is currently the preferred device for the transcatheter closure of secundum atrial septal defects. Multiple studies have shown that device complications with the Amplatzer occluder are rare and often acute in presentation. The investigators describe the first reported case of late obstruction of the right pulmonary veins with an Amplatzer septal occluder and, in the same patient, an unusual intraoperative finding of a noncoronary aortic sinus to left atrium fistula after device removal.

Fetal cardiac malposition: incidence and outcome of associated cardiac and extracardiac malformations.

Cardiac malposition is a rare but important finding when detected on fetal ultrasound. The purpose of this study was to evaluate the incidence of fetal cardiac malposition, associated abnormalities, and clinical outcome in a tertiary-care medical center. Records of fetuses (1993 to 2006) with dextroposition, dextrocardia, mesocardia, ectopia cordis, or heterotaxy were reviewed.

Three-dimensional shape analysis of right ventricular remodeling in repaired tetralogy of Fallot.

Understanding of right ventricular (RV) remodeling is needed to elucidate the mechanism of RV dysfunction in the overloaded right ventricle, but is hampered by the chamber's complex shape. We imaged 15 patients with repaired tetralogy of Fallot (TOF) and 8 normal subjects by magnetic resonance imaging in long- and short-axis views. We reconstructed the right ventricles in 3 dimensions using the piecewise smooth subdivision surface method.

Accuracy and reproducibility of real-time three-dimensional echocardiography for assessment of right ventricular volumes and ejection fraction in children.

Background: Measurement of right ventricular (RV) volumes and ejection fraction (EF) by two-dimensional echocardiography has limited accuracy and reproducibility because of the complex RV geometry.

Objectives: This study sought to validate real-time three-dimensional echocardiography (RT3DE) using a disk summation method for assessment of RV volumes and RVEF in children by comparing it with magnetic resonance imaging (MRI) measurements.

Methods: A total of 20 children (mean age 10.

Anomalous right coronary artery from the pulmonary artery: noninvasive diagnosis and serial evaluation.

Isolated anomalous origin of the right coronary artery from the main pulmonary artery (ARCAPA) is a rare congenital cardiac malformation. We reviewed the current literature and found only 31 patients with ARCAPA. We report the first case that was diagnosed and followed on a noninvasive basis with cardiovascular magnetic resonance after surgical re-implantation.

Left ventricular non-compaction cardiomyopathy in children: characterisation of clinical status using tissue Doppler-derived indices of left ventricular diastolic relaxation.

Background: Left ventricular non-compaction (LVNC) may manifest an undulating phenotype ranging from dilated to hypertrophic appearance. It is unknown whether tissue Doppler (TD) velocities can predict adverse clinical outcomes including death and need for transplantation in children with LVNC.

Methods And Results: 56 children (median age 4.

Characteristics and outcomes of fetuses with pericardial effusions.

Little is known about the characteristics and outcomes of fetuses with pericardial effusions (PEs); therefore, this study sought to identify factors associated with fetal PEs and the natural histories and outcomes of fetuses with PEs. Large PEs are associated with a greater likelihood of structural heart disease, impaired cardiac function, and chromosomal abnormalities, and PEs with hydrops or extracardiac malformations are associated with death. Most fetal PEs resolve, and fetuses with isolated PEs have a very good prognosis.

Impact of chronic left ventricular preload and afterload on Doppler tissue imaging velocities: a study in congenital heart disease.

Background: Doppler tissue imaging (DTI) velocities have been reported to be relatively independent of changes in ventricular loading conditions in adult studies. The clinical impact of altered left ventricular (LV) preload and afterload on DTI velocities in children with congenital heart disease has not been adequately evaluated. The purpose of this study was to evaluate the impact of chronic LV preload and afterload on DTI velocities in children with isolated ventricular septal defect and aortic valve stenosis compared with age-matched normal and abnormal (dilated cardiomyopathy) control groups.