Polyvalvular Dysplasia and Vascular Abnormalities in a Neonate With an Variant.

There is growing appreciation for inherited structural heart diseases and their genetic causes. One causal gene for congenital cardiac and vascular lesions is which encodes a critical protein for cytoskeletal and extracellular matrix development. A newborn infant male, with prenatally diagnosed polyvalvular dysfunction, presented with low cardiac output and postnatally detected aortic arch hypoplasia and coarctation.

Antibiotic Prophylaxis for Infective Endocarditis: A Survey of Practice Among Pediatric Cardiology Providers.

The 2007 American Heart Association (AHA) guidelines limited antibiotic prophylaxis (AP) for infective endocarditis (IE) to fewer patients with predisposing cardiac conditions (PCC). We surveyed the American Academy of Pediatrics Section on Cardiology and Cardiac Surgery (AAP SOCCS) on their recommendations for AP for a number of PCC and procedures. We report on those 173 respondents who follow the 2007 AHA guidelines.

Terbutaline-triggered fetal arrhythmia prior to neonatal diagnosis of Wolff-Parkinson-White syndrome: A case report.

Introduction: Short-term maternal administration of betamimetics is a common obstetric practice with uses including tocolysis during antenatal corticosteroid administration for fetal lung maturity, intrapartum tachysystole, and prior to external cephalic version. While previous research has demonstrated adverse effects of prolonged use of maternal betamimetics, no prior documentation exists of fetal tachyarrhythmias beyond sinus tachycardia after administration of terbutaline.

Case: This case documents a transient fetal tachyarrhythmia consistent with presumed atrial flutter after maternal administration of terbutaline for external cephalic version.

A unique indication for the senning procedure: Atrioventricular discordance with ventriculoarterial concordance.

Objective: We describe experience treating a patient with atrioventricular (AV) discordance with ventriculoarterial (VA) concordance, ventricular septal defect (VSD), and situs inversus. This is a rare congenital lesion in which closing the VSD would septate D-transposition physiology and performing an arterial switch and VSD closure would produce congenitally corrected transposition of the great arteries physiology. As such, it is the only lesion for which an isolated atrial switch (and VSD closure) remains the preferred correction.

In-utero idiopathic ductal constriction: a prenatal manifestation of Alagille and Williams syndrome arteriopathy.

Objective: Williams and Alagille syndromes are genetic disorders associated with pathologic arterial narrowing. We hypothesized that fetal idiopathic ductus arteriosus (DA) constriction may represent a prenatal manifestation of the arteriopathy associated with these syndromes.

Methods: Multi-institutional case series review of the pre- and postnatal medical records, echocardiograms, and genetic test results of fetuses presenting with idiopathic DA constriction.

Hemodynamic study of TCPC using in vivo and in vitro 4D Flow MRI and numerical simulation.

Altered total cavopulmonary connection (TCPC) hemodynamics can cause long-term complications. Patient-specific anatomy hinders generalized solutions. 4D Flow MRI allows in vivo assessment, but not predictions under varying conditions and surgical approaches.

Ventricular kinetic energy may provide a novel noninvasive way to assess ventricular performance in patients with repaired tetralogy of Fallot.

Objective: Ventricular kinetic energy measurements may provide a novel imaging biomarker of declining ventricular efficiency in patients with repaired tetralogy of Fallot. Our purpose was to assess differences in ventricular kinetic energy with 4-dimensional flow magnetic resonance imaging between patients with repaired tetralogy of Fallot and healthy volunteers.

Methods: Cardiac magnetic resonance, including 4-dimensional flow magnetic resonance imaging, was performed at rest in 10 subjects with repaired tetralogy of Fallot and 9 healthy volunteers using clinical 1.

4D cardiovascular magnetic resonance velocity mapping of alterations of right heart flow patterns and main pulmonary artery hemodynamics in tetralogy of Fallot.

Background: To assess changes in right heart flow and pulmonary artery hemodynamics in patients with repaired Tetralogy of Fallot (rTOF) we used whole heart, four dimensional (4D) velocity mapping (VM) cardiovascular magnetic resonance (CMR).

Methods: CMR studies were performed in 11 subjects with rTOF (5M/6F; 20.1 ± 12.

Assessment of left ventricular pre-ejection period in the fetus using simultaneous magnetocardiography and echocardiography.

Introduction: Fetal magnetocardiography (fMCG) is a promising new technique for assessing fetal rhythm; however, no prior studies have utilized fMCG to evaluate human fetal electromechanical physiology. Pre-ejection period (PEP) is an important measure of the electromechanical activation of the heart, and is altered by disease states and arrhythmias.

Materials And Methods: A novel technique was used to assess fetal PEP and its relationship to other fetal systolic time intervals, RR interval, and gestational age (GA).

In utero premature closure of the ductus arteriosus presenting as isolated right ventricular hypertrophy.

Background: The etiology of isolated right ventricular hypertrophy (RVH) is distinct from other forms of hypertrophic cardiomyopathy. RVH is typically seen in the setting of pulmonary valve stenosis or Tetralogy of Fallot. A rare cause of isolated RVH is premature closure of the patent ductus arteriosus (PDA) in utero that results in pulmonary hypertension.

Overview of fetal arrhythmias.

Purpose Of Review: Though fetal arrhythmias account for a small proportion of referrals to a fetal cardiologist, they may be associated with significant morbidity and mortality. The present review outlines the current literature with regard to the diagnosis and, in brief, some management strategies in fetal arrhythmias.

Recent Findings: Advances in echocardiography have resulted in significant improvements in our ability to elucidate the mechanism of arrhythmia at the bedside.