Truncus Arteriosus and Absent Ascending Aorta With Unusual Head and Neck Vessel Origins.

We describe a neonate with a unique variant of truncus arteriosus with interrupted aortic arch, an absent ascending aorta, persistent right dorsal aorta, and an unusual brachiocephalic artery pattern in which all head and neck vessels were supplied from the ductal arch-descending aorta continuum. ().

Correlation of transthoracic echocardiography-derived pulmonary to systemic flow ratio with hemodynamically estimated left to right shunt in atrial septal defects.

Background: Transthoracic echocardiographic (TTE) estimation of the pulmonary to systemic flow ratio (Qp/Qs) is routinely used in clinical practice and is included in the American Society of Echocardiography Guidelines. We sought to assess its real-world applicability with a particular focus on hemodynamically significant shunt lesions.

Methods: Retrospective single institutional review of TTE's in patients with secundum atrial septal defect prior to cardiac catheterization (cath) from 2012 to 2018 was performed ( = 109), those with technically limited images for Qp/Qs calculation ( = 11) and those with time interval between TTE and cath >60 days were excluded ( = 14).

Juxtaposition of the Atrial Appendages: A Large Echocardiographic Series.

Juxtaposition of the atrial appendages is known to occur in specific congenital heart lesions. Recognition of these variants is critical in balloon atrial septostomy and atrial switch operations. There remains little clinical data on the prevalence and associations of these lesions.

Congenitally "Inverted" Pulmonary Valve in Tetralogy of Fallot: When Nature Falters.

A cyanotic neonate with tetralogy of Fallot was found to have a congenitally inverted pulmonary valve. Diagnosis was made via echocardiography and cardiac catheterization. The valve opened retrograde into the right ventricle, which allowed severe regurgitation and prevented anterograde flow.

Comparison of chloral hydrate and pentobarbital sedation for pediatric echocardiography.

Background: In 2013, outpatient use of chloral hydrate (CH) was limited and other alternatives such as oral pentobarbital (PB) were explored to achieve conscious sedation in young children for transthoracic echocardiography (TTE). We aimed to assess efficacy and safety of the two medications.

Methods: Clinical information, from a computerized database, about children who received sedation with either CH or PB for TTE at our center (2008-2015) was reviewed, and the two groups were compared for sedation effectiveness and complications.

Risk factors for development of obesity in an ethnically diverse CHD population.

Objectives: Previous cross-sectional studies have demonstrated obesity rates in children with CHD and the general paediatric population. We reviewed longitudinal data to identify factors predisposing to the development of obesity in children, hypothesising that age may be an important risk factor for body mass index growth.Study designRetrospective electronic health records were reviewed in all 5-20-year-old CHD patients seen between 2011 and 2015, and in age-, sex-, and race/ethnicity-matched controls.

Abnormalities in Left Ventricular Rotation Are Inherent in Young Children with Repaired Tetralogy of Fallot and Are Independent of Right Ventricular Dilation.

Left ventricular (LV) dysfunction is a risk factor for adverse outcomes in older children and adults with repaired Tetralogy of Fallot (rToF). Pulmonary regurgitation (PR), right ventricular (RV) dilation, and dysfunction have been shown to result in abnormal LV myocardial mechanics and dysfunction. The aim of our study was to evaluate LV rotational mechanics, especially apical rotation in young children with rToF with and without RV dilation.

Validation of Right Atrial Area as a Measure of Right Atrial Size and Normal Values of in Healthy Pediatric Population by Two-Dimensional Echocardiography.

Right atrial (RA) size is a prognostic indicator for heart failure and cardiovascular death in adults. Data regarding use of RA area (RAA) by two-dimensional echocardiography as a surrogate for RA size and allometric modeling to define appropriate indexing of the RAA are lacking. Our objective was to validate RAA as a reliable measure of RA size and to define normal reference values by transthoracic echocardiography (TTE) in a large population of healthy children and develop Z-scores using a validated allometric model for indexing RAA independent of age, sex, and body size.

Malattached septum primum and deficient septal rim predict unsuccessful transcatheter closure of atrial communications.

Objectives: Assess impact of malattached septum primum (MASP) on transcatheter closure of interatrial communications.

Background: Large defect size and deficient rims have been described as predictors for complications and unsuccessful device placement in closure of interatrial communications. MASP is an underappreciated morphologic atrial septal variation whose prevalence in isolated atrial communications and its influence on percutaneous device closure has not been systematically assessed.

Application of Virtual Three-Dimensional Models for Simultaneous Visualization of Intracardiac Anatomic Relationships in Double Outlet Right Ventricle.

Our goal was to construct three-dimensional (3D) virtual models to allow simultaneous visualization of the ventricles, ventricular septal defect (VSD) and great arteries in patients with complex intracardiac anatomy to aid in surgical planning. We also sought to correlate measurements from the source cardiac magnetic resonance (CMR) image dataset and the 3D model. Complicated ventriculo-arterial relationships in patients with complex conotruncal malformations make preoperative assessment of possible repair pathways difficult.

Two-dimensional speckle-tracking-derived segmental peak systolic longitudinal strain identifies regional myocardial involvement in patients with myocarditis and normal global left ventricular systolic function.

The presence of myocardial late gadolinium enhancement (LGE) by cardiac magnetic resonance (CMR) imaging in concert with electrocardiography and elevated biomarkers helps support the diagnosis of acute myocarditis. Two-dimensional echocardiography is limited to global and qualitative regional function assessment and may not contribute to the diagnosis, especially in the presence of normal LV systolic function. Two-dimensional speckle-tracking (2D-STE)-derived segmental peak systolic (pkS) longitudinal strain (LS) may identify segmental myocardial involvement in myocarditis.

Applicability of published guidelines for assessment of left ventricular diastolic function in adults to children with restrictive cardiomyopathy: an observational study.

Guidelines for diagnosis and grading of diastolic dysfunction (DD) in children have not been established. The applicability of adult parameters of DD to children has been questioned by recent studies. Although normal diastolic parameters in children have been published, the data to support application of these indices for the non-invasive diagnosis of DD and quantifying its degree are still being developed.

Prevalence and outcomes of coronary artery ectasia associated with isolated congenital coronary artery fistula.

Isolated congenital coronary artery fistula (CAF) is rare and varies with respect to size and hemodynamic significance. The prevalence of coronary artery ectasia in association with isolated congenital CAF, regardless of size, and after closure of large fistulae has not been systematically evaluated in the literature. This study aimed to characterize the demographic and echocardiographic differences between patients with large and small fistulae and to describe outcomes with respect to coronary ectasia in those who underwent closure.

Subpulmonary stenosis assessed in midtrimester fetuses with tetralogy of Fallot: a novel method for predicting postnatal clinical outcome.

This study aimed to determine whether quantification of subpulmonary stenosis (SPS) in tetralogy of Fallot (TOF) in the second-trimester fetus can predict postnatal clinical outcome measured by pulmonary valve size and/or timing or type of intervention. The study retrospectively identified fetuses with TOF from 1998 to 2010 diagnosed at 26 weeks gestation or earlier. The data evaluated included pre- and postnatal pulmonary valve z-scores (PVZ).

Echocardiographic myocardial deformation evaluation of right ventricular function in comparison with CMRI in repaired tetralogy of Fallot: a cross-sectional and longitudinal validation study.

Background: Decreased right ventricular (RV) ejection fraction (EF) increases morbidity in repaired tetralogy of Fallot (rToF). Cardiac magnetic resonance (CMR) is the accepted reference standard for RV EF measurement. There are no established color Doppler tissue imaging (cDTI) values that identify decreased RV EF in rToF.

The role of right ventricular function in paediatric idiopathic dilated cardiomyopathy.

Introduction: The prevalence of right ventricular dysfunction in idiopathic dilated cardiomyopathy is incompletely studied in children. Furthermore, right ventricular function may signal worse outcomes. We evaluated recently published right ventricular function echocardiographic indices in identifying dysfunction in children with idiopathic dilated cardiomyopathy and the impact of right ventricular dysfunction on long-term prognosis.

Imaging of congenital coronary artery anomalies.

Imaging of the coronary arteries is an important part of the evaluation of children with congenital heart disease and isolated congenital coronary artery anomalies. Echocardiography remains the main imaging modality and is complemented by MRI and CT angiography in the older or difficult-to-image child. We review echocardiography, MRI, and CT angiography for coronary artery imaging, with emphasis on techniques.

Outcomes of mitral regurgitation associated with large ventricular septal defect and a normal mitral valve apparatus: does intact atrial septum have an impact?

The hemodynamics of isolated large ventricular septal defect with an intact atrial septum results in greater mitral valve (MV) annular dilation and an increased incidence of mitral regurgitation (MR). MR will resolve in most patients after surgical VSD closure alone without MV repair.