Clinical Variables Associated with Pre-Fontan Aortopulmonary Collateral Burden.

Aortopulmonary collaterals (APCs) develop universally, but to varying degrees, in patients with single ventricle congenital heart disease (CHD). Despite their ubiquitous presence, APCs remain poorly understood. We sought to evaluate the association between APC burden and common non-invasive clinical variables.

The association between cardiac magnetic resonance-derived aortic stiffness parameters and aortic dilation in young adults with bicuspid aortic valve: With and without coarctation of aorta.

Background: Bicuspid aortic valve (BAV) is associated with progressive aortic dilation. Studies in aortopathies have shown a correlation between increased aortic stiffness and aortic dilation. We aimed to evaluate aortic stiffness measures as predictors of progressive aortic dilation by cardiac magnetic resonance (CMR) in BAV patients.

Double-Orifice Tricuspid Valve in an Infant with Multiple Noncardiac Anomalies.

• DOTV can be seen in patients with noncardiac anomalies. • Subcostal windows allow an en face echocardiographic assessment of the tricuspid valve. • DOTV is rare, with a little known natural history warranting long-term follow-up.

Three-Dimensional Echocardiography Right Ventricular Volumes and Ejection Fraction Reference Values in Children: A North American Multicentre Study.

Background: Three-dimensional echocardiography (3DE) evaluation of right ventricular (RV) volumes and ejection fraction (EF) is increasingly used for clinical serial assessments and management in children. This study aims to generate sex-specific reference values and z-score equations for RV volumetric parameters, independent of age and body size indices, derived from multiple populations across North America.

Methods: We prospectively recruited 455 healthy children (ages 0 to 18 years) from 5 centres.

Cardiac MRI-Derived Myocardial Deformation Parameters Correlate with Pulmonary Valve Replacement Indications in Repaired Tetralogy of Fallot.

Right ventricular (RV) volumetric cardiac magnetic resonance (CMR) criteria serve as indicators for pulmonary valve replacement (PVR) in repaired tetralogy of Fallot (rTOF). Myocardial deformation and tricuspid valve displacement parameters may be more sensitive measures of RV dysfunction. This study's aim was to describe rTOF RV deformation and tricuspid displacement patterns using novel CMR semi-automated software and determine associations with standard CMR measures.

Outcomes Following Surgery to Address Shone Syndrome in Children.

Background: Shone syndrome is characterized by coincident mitral valve stenosis and left ventricular outflow tract obstruction. Although first described in 1963, little research has expounded surgical outcomes. We sought to evaluate our experience with this cohort, emphasizing outcomes including mortality, morbidity, and cardiac function.

A multicenter study of three-dimensional echocardiographic evaluation of normal pediatric left ventricular volumes and function.

Background: Three-dimensional echocardiography (3DE) evaluation of left ventricular (LV) volume and function in pediatrics compares favorably with cardiac magnetic resonance imaging. The aim of this study was to establish from a multicenter, normal pediatric z-score values of 3DE left ventricular volumes and function.

Methods: Six hundred and ninety-eight healthy children (ages 0-18 years) were recruited from five centers.

Evaluating Chest Pain and Heart Murmurs in Pediatric and Adolescent Patients.

Chest pain and heart murmurs are common issues primary care providers must evaluate and manage. Both are a source of anxiety for patients, parents, and providers, necessitating evaluation and understanding to ensure appropriate management. Most pediatric chest pain can be treated symptomatically and with reassurance.

Echocardiographic Identification of Pulmonary Artery Flow Reversal: An Indicator of Adverse Outcomes in Single Ventricle Physiology.

Individuals with single ventricle congenital heart disease (CHD) undergo multiple staged surgical palliations. Staged single ventricle palliation with a superior cavopulmonary connection (SCPC) in infancy followed by a Fontan in early childhood relies on passive, unobstructed pulmonary blood flow and normal pulmonary vasculature. We hypothesized that patients with echocardiographic identification of retrograde flow in a branch pulmonary artery (PA) after SCPC or Fontan are at increased risk for adverse outcomes.

Echocardiography vs cardiac magnetic resonance imaging assessment of the systemic right ventricle for patients with d-transposition of the great arteries status post atrial switch.

Objective: Patients with Dextro-transposition of the great arteries status post atrial switch (dTGA s/p atrial switch) are "at-risk" for systemic right ventricular (RV) dysfunction. Due to complex RV geometry, echocardiography (Echo) does not allow accurate determination of ejection fraction (EF), but cardiac magnetic resonance imaging (CMR) allows quantitative right ventricular assessment. Measures of ventricular deformation may be precursors to global ventricular dysfunction.

Morphine Pharmacokinetics in Children With Down Syndrome Following Cardiac Surgery.

Objectives: To assess if morphine pharmacokinetics are different in children with Down syndrome when compared with children without Down syndrome.

Design: Prospective single-center study including subjects with Down syndrome undergoing cardiac surgery (neonate to 18 yr old) matched by age and cardiac lesion with non-Down syndrome controls. Subjects were placed on a postoperative morphine infusion that was adjusted as clinically necessary, and blood was sampled to measure morphine and its metabolites concentrations.

Imaging adult patients with Fontan circulation.

Purpose Of Review: Survival after the Fontan procedure for palliation of single ventricle congenital heart disease has improved. However, adults with Fontan circulation are at risk for several complications including heart failure, thromboembolism, and protein-losing enteropathy. This review discusses the role of noninvasive imaging for surveillance and early detection of anatomic and functional abnormalities of the Fontan circulation that can impact the risk for Fontan failure over time.

Rapid 3D imaging of the lower airway by MRI in patients with congenital heart disease: A retrospective comparison of delayed volume interpolated breath-hold examination (VIBE) to turbo spin echo (TSE).

Objective: When imaging the lower airway by MRI, the traditional technique turbo spin echo (TSE) results in high quality 2D images, however planning and acquisition times are lengthy. An alternative, delayed volume interpolated breath-holds examination (VIBE), is a 3D gradient echo technique that produces high spatial resolution imaging of the airway in one breath-hold. The objective of this study is to retrospectively evaluate the accuracy of lower airway measurements obtained by delayed VIBE when compared to TSE.

Cardiac Magnetic Resonance Imaging in Pediatric Turner Syndrome.

Objective: To compare the detection of cardiac lesions with the use of cardiac magnetic resonance imaging (CMR) and conventional echocardiography in children with Turner syndrome.

Study Design: Twenty-four girls with Turner syndrome, 8-18 years of age, were recruited through the Pediatric Endocrinology Program. Participants underwent CMR and echocardiography within a 2-year period, and discrepancies between the results of each modality were identified.

Multivalvular Replacement and Ventricular Arrhythmias in a Female Child With Congenital Polyvalvular Disease.

We report the clinical course of a female child with a normal karyotype and chromosomal microarray who presented as an infant with clinical findings consistent with congenital polyvalvular disease (CPVD). This clinical entity describes patients with multiple congenitally dysplastic valves, often showing nodular or cystic malformation in at least two cardiac valves. This patient then developed medically refractory multifocal ventricular arrhythmia and required radiofrequency ablation at seven months of age.

The use of Arginine Vasopressin in neonates following the Norwood procedure.

Background: Following the Norwood palliation, neonates may require an escalation of inotropic and vasoactive support. Arginine Vasopressin may be uniquely useful in supporting this population.

Materials And Methods: A retrospective evaluation of neonates at this institution between November, 2007 and October, 2010 who received Arginine Vasopressin following the Norwood procedure.