Differential requirement for DICER1 activity during the development of mitral and tricuspid valves.

Mitral and tricuspid valves are essential for unidirectional blood flow in the heart. They are derived from similar cell sources, and yet congenital dysplasia affecting both valves is clinically rare, suggesting the presence of differential regulatory mechanisms underlying their development. Here, we specifically inactivated Dicer1 in the endocardium during cardiogenesis and found that Dicer1 deletion caused congenital mitral valve stenosis and regurgitation, whereas it had no impact on other valves.

Health-Related Quality of Life After Single Ventricle Palliation or Tetralogy of Fallot Repair.

Single ventricle (SV) cardiac lesions and tetralogy of Fallot (TOF) are both common forms of cyanotic congenital heart disease. With advances in perioperative care and longitudinal follow-up, survival of these patients has dramatically improved and the majority survive to adulthood. This study compares health-related quality of life (HRQoL) of adult SV and TOF patients to each other and the general population.

An idea to explore: Use of augmented reality for teaching three-dimensional biomolecular structures.

Biology and biochemistry students must learn to visualize and comprehend the complex three-dimensional (3D) structures of macromolecules such as proteins or DNA. However, most tools available for teaching biomolecular structures typically operate in two dimensions. Here, we present protocols and pedagogical approaches for using immersive augmented reality (AR) visors, specifically the Microsoft HoloLens, to reinforce learning with large scale 3D holographic structures.

Development and Utility of Quality Metrics for Ambulatory Pediatric Cardiology in Kawasaki Disease.

The Adult Congenital and Pediatric Cardiology (ACPC) Section of the American College of Cardiology sought to develop quality indicators/metrics for ambulatory pediatric cardiology practice. The objective of this study was to report the creation of metrics for patients with Kawasaki disease. Over a period of 5 months, 12 pediatric cardiologists developed 24 quality metrics based on the most relevant statements, guidelines, and research studies pertaining to Kawasaki disease.

Congenitally Corrected Transposition Presenting in the First Year of Life: Survival and Fate of the Systemic Right Ventricle.

Introduction: Knowledge gaps exist in the life expectancy and functional outcome of patients with congenitally corrected transposition (ccTGA) presenting early in life, which is relevant in the evaluation of early anatomic repair.

Methods: In a single-center analysis, 91 patients with ccTGA were identified over 25 years, of which 31 presented with biventricular anatomy in the first year of life and formed the study cohort. End points for analysis included survival, moderate or worse tricuspid valve regurgitation, and systemic right ventricle (RV) dysfunction.

The impact of ecbolic therapy in the early postpartum period on uterine involution and reproductive health in dairy cows.

Postpartum uterine disease due to poor uterine involution continues to be a significant factor that contributes to poor reproductive efficiency in dairy cattle. Therapy that increases the frequency, duration and strength of uterine contractions in the postpartum period might enhance uterine involution, resulting in improved reproductive performance. The objective of this clinical trial was to study the effect of two uterine ecbolic therapies, oxytocin and prostaglandinF2α on uterine involution, postpartum endometritis, and reproductive performance.

Cardiovascular Health in Turner Syndrome: A Scientific Statement From the American Heart Association.

Girls and women with Turner syndrome face a lifelong struggle with both congenital heart disease and acquired cardiovascular conditions. Bicuspid aortic valve is common, and many have left-sided heart obstructive disease of varying severity, from hypoplastic left-sided heart syndrome to minimal aortic stenosis or coarctation of the aorta. Significant enlargement of the thoracic aorta may progress to catastrophic aortic dissection and rupture.

Persistence of oxytocin receptors in the bovine uterus during the first 7 d after calving: an immunohistochemical study.

Persistence of the oxytocin receptor (OTR) in the bovine uterus during the first 7 d after calving was investigated by means of immunohistochemical staining. Immunoreactive OTRs were present in different locations in the uterus on almost all days except day 2, when staining was seen only in the endothelium of blood vessels in the endometrium and myometrium. This finding supports the hypothesis that oxytocin may be ecbolic in cows through the 1st week post partum, but further studies are required to assess the receptor functionality during this period.

Neurodevelopmental outcomes in children with congenital heart disease: evaluation and management: a scientific statement from the American Heart Association.

Background: The goal of this statement was to review the available literature on surveillance, screening, evaluation, and management strategies and put forward a scientific statement that would comprehensively review the literature and create recommendations to optimize neurodevelopmental outcome in the pediatric congenital heart disease (CHD) population.

Methods And Results: A writing group appointed by the American Heart Association and American Academy of Pediatrics reviewed the available literature addressing developmental disorder and disability and developmental delay in the CHD population, with specific attention given to surveillance, screening, evaluation, and management strategies. MEDLINE and Google Scholar database searches from 1966 to 2011 were performed for English-language articles cross-referencing CHD with pertinent search terms.

Novel technique of valve-sparing aortic root replacement in two children younger than 3 years of age.

A novel technique of valve-sparing aortic root replacement was applied to 2 children younger than 3 years of age with Marfan syndrome and large aortic root aneurysms. Using elements of both the remodeling and reimplantation techniques, circumferential rings from a 20-mm to 22-mm polyester graft provide stabilization at the subannular and sinotubular levels, and bovine pericardial patches create pseudosinuses. Follow-up at 2 years in 1 patient and 7 months in a second patient revealed satisfactory valve function with stable aortic root size.

Pretreatment and routine echocardiogram monitoring during chemotherapy for anthracycline-induced cardiotoxicity rarely identifies significant cardiac dysfunction or alters treatment decisions: a 5-year review at a single pediatric oncology center.

Background: The widespread use of anthracycline chemotherapy has contributed to improved outcomes in children with cancer. The most feared complication of the anthracyclines is cardiotoxicity. Routine echocardiographic monitoring typically is used before, during, and after treatment to minimize cardiotoxicity.

Interferon gamma-inducible protein-10 (IP-10) and eotaxin as biomarkers in age-related macular degeneration.

Purpose: To analyze serum cytokine levels in subjects with different stages of AMD and to study the expression of salient cytokines in postmortem eyes with AMD.

Methods: A suspension array system was used to analyze sera (n = 18 to 20/group) from control subjects and those with early AMD (AREDS stage 1), intermediate dry AMD (AREDS stage 3), advanced AMD with geographic atrophy (GA), or neovascular AMD (CNV). Postmortem eyes with AMD or control eyes were examined immunohistochemically for expression of IP-10 and eotaxin (n = 4 to 8/group).

Is the "perfect Fontan" operation routinely achievable in the modern era?

Objective: In 1990, Fontan, Kirklin, and colleagues published equations for survival after the so-called "Perfect Fontan" operation. After 1988, we evolved a protocol using an internal or external polytetraflouroethylene tube of 16 to 19 millimetres diameter placed from the inferior caval vein to either the right or left pulmonary artery along with a bidirectional cava-pulmonary connection. The objective of this study was to test the hypothesis that a "perfect" outcome is routinely achievable in the current era when using a standardized surgical procedure.

Use of somatic cell nuclear transfer to study meiosis in female cattle carrying a sex-dependent fertility-impairing X-chromosome abnormality.

Animal models have played an important part in establishing our knowledge base on reproduction, development, and the occurrence and impact of chromosome abnormalities. Translocations involving the X chromosome and an autosome are unique in that they elicit sex-dependent infertility, with male carriers rendered sterile by synaptic anomalies during meiosis, whereas female carriers conceive but repeatedly abort. Until now the limited access to relevant fetal oocytes has precluded direct study of meiotic events in female carriers.

A comparison of the cytobrush and uterine lavage techniques to evaluate endometrial cytology in clinically normal postpartum dairy cows.

This study compares cytobrush and lavage techniques for the assessment of endometrial cytology (EC) in clinically normal postpartum dairy cows. The EC samples were collected from Holstein cows (n = 35) during visit 1 (V1) at 20 to 33 d in milk (DIM) and 2 wk later during visit 2 (V2) at 34 to 47 DIM by using both techniques. A minimum of 100 cells were counted to determine the percentage of cells that were neutrophils (%PMN).

Glycogen storage diseases presenting as hypertrophic cardiomyopathy.

Background: Unexplained left ventricular hypertrophy often prompts the diagnosis of hypertrophic cardiomyopathy, a sarcomere-protein gene disorder. Because mutations in the gene for AMP-activated protein kinase gamma2 (PRKAG2) cause an accumulation of cardiac glycogen and left ventricular hypertrophy that mimics hypertrophic cardiomyopathy, we hypothesized that hypertrophic cardiomyopathy might also be clinically misdiagnosed in patients with other mutations in genes regulating glycogen metabolism.

Methods: Genetic analyses performed in 75 consecutive unrelated patients with hypertrophic cardiomyopathy detected 40 sarcomere-protein mutations.

Clinical, genetic, and biophysical characterization of a homozygous HERG mutation causing severe neonatal long QT syndrome.

Previous studies have identified mutations in five ion channel genes as a cause of long QT syndrome, a heterogeneous disorder characterized by prolongation of the QT interval, multiform ventricular tachycardia (torsades de pointes), seizures, syncope, and sudden death. However, in these studies, the average age of initial symptoms is in the third decade of life or later, and few reports have described the genetic causes of long QT syndrome presenting in the prenatal or neonatal period. We used a candidate gene approach to identify the genetic cause of long QT syndrome in an infant whose initial manifestations were detected in utero.

Nitric oxide: lifesaving measure for pulmonary vasospasm after modified Blalock-Taussig shunt.

Pulmonary vasospasm and hypertension may occur after repair or palliation of congenital cardiac defects, and can be fatal in spite of conventional treatment. Nitric oxide has been shown to improve pulmonary hypertension unresponsive to conventional measures after a variety of repairs, but use has infrequently been reported after palliative systemic to pulmonary artery shunts. We report a case of pulmonary hypertension and life threatening desaturation after a modified Blalock-Taussig shunt that responded rapidly to inhaled nitric oxide.

The effect of prepartum injection of vitamin E on health in transition dairy cows.

The objective of this study was to investigate parenteral vitamin E for the prevention of peripartum disease in dairy cows. A randomized clinical trial was conducted in 21 commercial dairy herds. Cows (n = 1142) were randomly assigned to receive either a single subcutaneous injection of 3000 IU of vitamin E, or placebo, 1 wk before expected calving.

Determination of the Enthalpies of Combustion and Formation of Substituted Triazines in an Adiabatic Rotating Bomb Calorimeter.

To obtain reliable thermodynamic data on substituted triazines, it is necessary to use a calorimeter that is capable of high precision with small quantities of sample and in which a homogeneous solution of the corrosive combustion products can be maintained. The enthalpies of combustion of six substituted triazines have been determined in a platinum-lined adiabatic rotating bomb calorimeter. These are the first determinations of enthalpies of combustion or formation to have been reported for these compounds.

The Enthalpies of Combustion and Formation of 2,2'-Dichloroethyl Sulfide.

The enthalpies of combustion and formation of 2,2'-dichlorethyl sulfìde (mustard gas) have been determined by combustion in an adiabatic rotating-bomb calorimeter. The bomb process has been corrected to: for which the following values were obtained: .

The Enthalpies of Combustion and Formation of Thianthrene.

The enthalpy of combustion of thianthrene (diphenylene disulfide) has been determined in an oxygen-bomb calorimeter. The enthalpy of formation has been derived using data from the available literature. The results obtained are as follows: A comparison is given of the results of this investigation with those of previous investigators.

The Enthalpies of Combustion and Formation of Cholesterol [cholest-5-en-3-ol (3 )].

The enthalpy of combustion of cholesterol was measured in an adiabatic, rotating-bomb calorimeter capable of high precision with relatively small samples. The random error for the experimental measurements was 0.006 percent which may be compared with approximately 0.

The Enthalpies of Combustion and Formation of Acetanilide and Urea.

The enthalpies of combustion of acetanilide and urea have been determined in an oxygen-bomb calorimeter. The following values and their estimated uncertainties were obtained. [Table: see text].

The Enthalpies of Combustion and Formation of Ortho- and Parafluorobenzoic Acid.

The enthalpies of combustion and formation of one sample of ortho- and two samples of parafluorobenzoic acids have been determined by combustion in an oxygen-bomb calorimeter. The data obtained by other investigators are discussed briefly. The values obtained and their estimated uncertainties are as follows: [Table: see text] where Δ° corresponds to the reaction: .