Proof of concept: Comparative accuracy of semiautomated VR modeling for volumetric analysis of the heart ventricles.

Introduction: Simpson's rule is generally used to estimate cardiac volumes. By contrast, modern methods such as Virtual Reality (VR) utilize mesh modeling to present the object's surface spatial structure, thus enabling intricate volumetric calculations. In this study, two types of semiautomated VR models for cardiac volumetric analysis were compared to the standard Philips dedicated cardiac imaging platform (PDP) which is based on Simpson's rule calculations.

Dominant Ventricular Morphology and Early Postoperative Course After the Fontan Procedure.

Background: Single ventricle heart disease comprises a wide variety of critical heart defects that lead to the provision of systemic cardiac output by one dominant ventricle. It requires staged surgical palliation that culminates in Fontan circulation. Dominant ventricular morphology in single ventricle patients reportedly has an impact on postoperative morbidity and mortality with varying results.

Prenatal diagnosis of major aortopulmonary collateral arteries (MAPCA) in fetuses with pulmonary atresia with ventricular septal defect and agenesis of ductus arteriosus.

Objective: The aim of this study was to describe the prenatal diagnosis of Major Aortopulmonary Collateral Arteries (MAPCAs), and to present a systematic ultrasound method for evaluating lung vascularity in fetuses with pulmonary atresia with ventricular septal defect (PAVSD) and agenesis of ductus arteriosus (DA).

Method: This retrospective study evaluated fetuses diagnosed with PAVSD with agenesis of DA, for the presence of the MAPCAs anomaly. Fetal pulmonary vasculature was investigated by 2D and 4D Spatio Temporal Image Correlation (STIC) technology using High Definition Color Doppler.

Congenital Aortic Vascular Ring: In-Utero Sonographic Assessment of Tracheal Patency and Postnatal Outcome.

Objective:  Congenital aortic vascular ring may present after birth with variable degrees of respiratory distress due to tracheal compression. The aim of this study was to prospectively evaluate in utero tracheal patency in correlation with postnatal outcome.

Methods:  During an eight-year period, fetuses with aortic arch abnormality encircling the trachea and forming a complete ring were recruited for the study.

The "No ARSA" Sign: A Novel Method of Prenatal Screening for Aberrant Right Subclavian Artery.

An aberrant right subclavian artery (ARSA) can be overlooked by the conventional method as described by Chaoui et al., due to acoustic shadowing. The aim of this study was to evaluate the feasibility and accuracy of a novel screening method for ARSA by demonstrating the brachiocephalic artery bifurcation, referred to as the "No ARSA" sign.

A founder truncating variant in GDF1 causes autosomal-recessive right isomerism and associated congenital heart defects in multiplex Arab kindreds.

The genetic basis of congenital heart malformations associated with disruption of left-right (L-R) asymmetry is broad and heterogenous, with variants in over 25 genes implicated thus far. Of these, deleterious variants in the Growth/Differentiation Factor 1 (GDF1) gene have been shown to cause heterotaxy with varied complex heart malformations of left-right patterning, in 23 individuals reported to date, either in monoallelic or biallelic state. We report three unrelated individuals exhibiting right isomerism with congenital heart defects, each originating from a consanguineous kindred of Arab-Muslim descent.

Mutations in PPCS, Encoding Phosphopantothenoylcysteine Synthetase, Cause Autosomal-Recessive Dilated Cardiomyopathy.

Coenzyme A (CoA) is an essential metabolic cofactor used by around 4% of cellular enzymes. Its role is to carry and transfer acetyl and acyl groups to other molecules. Cells can synthesize CoA de novo from vitamin B5 (pantothenate) through five consecutive enzymatic steps.

Primary Fetal Lung Hypoplasia (PFLH): Imaging and Clinical Characteristics.

 To describe in utero and postnatal imaging and clinical characteristics of primary fetal lung hypoplasia (PFLH).  A retrospective review of fetuses and neonates diagnosed in one academic tertiary center during an eleven-year period.  12 cases of PFLH were identified.

The added value of f-TAPSE in the surveillance of pregnancies complicated by fetal and placental tumors.

Introduction: Fetal and placental tumors are associated with high prevalence of obstetrical complications and poor fetal outcome. The aim of our study was to assess the added value of serial fetal Tricuspid Annular Plane Systolic Excursion (f-TAPSE) measurements for monitoring cardiac function in cases of fetal and placental tumors.

Methods: Serial measurements of f-TAPSE were performed prospectively in fetuses referred for fetal and placental tumors.

Mutations in TMEM260 Cause a Pediatric Neurodevelopmental, Cardiac, and Renal Syndrome.

Despite the accelerated discovery of genes associated with syndromic traits, the majority of families affected by such conditions remain undiagnosed. Here, we employed whole-exome sequencing in two unrelated consanguineous kindreds with central nervous system (CNS), cardiac, renal, and digit abnormalities. We identified homozygous truncating mutations in TMEM260, a locus predicted to encode numerous splice isoforms.

Cardiac failure in very long chain acyl-CoA dehydrogenase deficiency requiring extracorporeal membrane oxygenation (ECMO) treatment: A case report and review of the literature.

Fatty acid oxidation (FAO) defects often present with multi-system involvement, including several life-threatening cardiac manifestations, such as cardiomyopathy, pericardial effusion and arrhythmias. We report herein a fatal case of cardiac dysfunction and rapid-onset tamponade following an acute illness in a neonate with molecularly proven very long chain acyl-CoA dehydrogenase (VLCAD) deficiency (harboring the known del799_802 mutation), requiring 15 days of extracorporeal membrane oxygenation (ECMO) treatment. As data regarding the use of ECMO in FAO defects in general, and VLCAD in particular, are scarce, we review the literature and discuss insights from in vitro models and several successful reported cases.

Micro-RNA-208a, -208b, and -499 as Biomarkers for Myocardial Damage After Cardiac Surgery in Children.

Objectives: To test the hypothesis that cardiac-enriched micro-RNAs can serve as accurate biomarkers that reflect myocardial injury and to predict the postoperative course following pediatric cardiac surgery. Micro-RNAs have emerged as plasma biomarkers for many pathologic states. We aimed to quantify preoperative and postoperative plasma levels of cardiac-enriched micro-RNA-208a, -208b, and -499 in children undergoing cardiac surgery and to evaluate correlations between their levels, the extent of myocardial damage, and the postoperative clinical course.

Perinatal and infant outcomes of prenatal diagnosis of heterotaxy syndrome (asplenia and polysplenia).

Patients with heterotaxy syndrome (HS) have a range of anomalies and outcomes. There are limited data on perinatal outcomes after prenatal diagnosis. To determine the factors influencing perinatal and infant outcomes, we analyzed prenatal and postnatal variables in fetuses with HS from 1995 to 2011.

The role of cardiac computed tomography in infants with congenital heart disease.

Background: Patients with complex congenital heart disease (CHD) have a high incidence of extracardiac vascular and non-vascular malformations. Those additional abnormalities may have an impact on the precise planning of surgical or non-surgical treatment.

Objectives: To assess the role of electrocardiography-gated CT-angiography (ECG-CTA) in the routine evaluation of CHD in neonates and infants particularly for the assessment of extracardiac findings.

Dobutamine stress MRI for the assessment of coronary artery disease: initial clinical experience in Israel.

Background: Over the past few years dobutamine stress magnetic resonance (DSMR) has proven its efficacy as an integral part of the diagnosis of coronary artery disease (CAD).

Objectives: To present the feasibility and safety of DSMR in Israel.

Methods: Thirty patients with suspected or known CAD were studied.

Fetal liver T2* values: defining a standardized scale.

Purpose: To define the normal T2* values of liver in the third trimester of pregnancy in normal fetuses.

Materials And Methods: Multi-echo gradient echo T2* sequence was applied to the fetal abdomen in the axial plane in women undergoing a fetal MRI (1.5 Tesla [T], MRI system).

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.

Repeatability of cardiac-MRI-measured right ventricular size and function in congenital heart disease.

Background: The measurement error for right ventricular (RV) size and function assessed by cardiac MRI (CMRI) in congenital heart disease has not been fully characterized. As CMRI parameters are being increasingly utilized to make clinical decisions, defining error in the clinical setting is critical.

Objective: This investigation examines the repeatability of CMRI for RV size and function.

Delta chemokine (fractalkine)--a novel mediator of pulmonary arterial hypertension in children undergoing cardiac surgery.

Background: Fractalkine (FKN), a unique chemokine associated with pulmonary hypertension, may be involved in the acute stress response that regulates inflammation after cardiopulmonary bypass (CPB) surgery. We characterized FKN levels and correlated them with clinical parameters in children undergoing cardiac surgery involving CPB.

Methods: Twenty-seven consecutive patients, aged 30 days to 11.

Is propofol safe for procedural sedation in children? A prospective evaluation of propofol versus ketamine in pediatric critical care.

Objectives: To compare propofol with ketamine sedation delivered by pediatric intensivists during painful procedures in the pediatric critical care department (PCCD).

Design: Prospective 15-month study.

Setting: An 18-bed multidisciplinary, university-affiliated PCCD.