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  • Open Access

    ARTICLE

    Bicuspid and unicuspid aortic valves: Different phenotypes of the same disease? Insight from the GenTAC Registry

    Joseph M. Krepp1, Mary J. Roman2, Richard B. Devereux2, Adrienne Bruce1, Siddharth K. Prakash3, Shaine A. Morris4, Dianna M. Milewicz3, Kathryn W. Holmes5, William Ravekes5, Ralph V. Shohet6, Reed E. Pyeritz7, Cheryl L. Maslen8, Barbara L. Kroner9, Kim A. Eagle10, Liliana Preiss9, GenTAC Investigators*, Federico M. Asch1

    Congenital Heart Disease, Vol.12, No.6, pp. 740-745, 2017, DOI:10.1111/chd.12520

    Abstract Background: Unicuspid aortic valve (UAV) is a rare disorder, often difficult to distinguish from bicuspid aortic valve (BAV). BAV and UAV share valve pathology such as the presence of a raphe, leaflet fusion, aortic stenosis, aortic regurgitation, and/or ascending aortic dilatation, but a comprehensive echocardiographic comparison of patients with UAV and BAV has not been previously performed.
    Methods: We investigated UAV and BAV patients at an early stage of disease included in GenTAC, a national registry of genetically related aortic aneurysms and associated cardiac conditions. Clinical and echocardiographic data from the GenTAC Registry were compared between 17… More >

  • Open Access

    ARTICLE

    Changing prevalence of severe congenital heart disease: Results from the National Register for Congenital Heart Defects in Germany

    Constanze Pfitzer1,2,3, Paul C. Helm4, Hannah Ferentzi1,5, Lisa-Maria Rosenthal1, Ulrike M. M. Bauer4,6, Felix Berger1,3,7, Katharina R. L. Schmitt1,3

    Congenital Heart Disease, Vol.12, No.6, pp. 787-793, 2017, DOI:10.1111/chd.12515

    Abstract Objective: To assess the prevalence of congenital heart disease (CHD) in Germany in relation to phenotypes, severity and gender.
    Design: Cross-sectional registry study.
    Setting: We analyzed data from patients with CHD born between 1996 and 2015.
    Patients: A total of 26 630 patients, registered with the NRCHD, were born between 1996 and 2015. 10 927 patients were excluded from the current analysis due to prior registration with the NRCHD under the German PAN Prevalence Study, which showed a potential bias in the inclusion of this patient population (proportion of mild cardiac lesions was comparatively high due to improved diagnostic… More >

  • Open Access

    ARTICLE

    Interobserver variability in the classification of congenital coronary abnormalities: A substudy of the anomalous connections of the coronary arteries registry

    Athanasios Koutsoukis1, Xavier Halna du Fretay2, Patrick Dupouy3, Phalla Ou4, Jean-Pierre Laissy4, Jean-Michel Juliard5, Fabien Hyafil6, Pierre Aubry5

    Congenital Heart Disease, Vol.12, No.6, pp. 726-732, 2017, DOI:10.1111/chd.12504

    Abstract Objective: The diagnosis of anomalous connections of the coronary arteries (ANOCOR) requires an appropriate identification for the management of the patients involved. We studied the observer variability in the description and classification of ANOCOR between a nonexpert group of physicians and a group of expert physicians, using the ANOCOR cohort.
    Patients and design: Consecutive patients identified by 71 referring cardiologists were included in the ANOCOR cohort. Anomalous connection was diagnosed by invasive and/or computed tomography coronary angiography. Angiographic images were reviewed by an angiographic committee with experience in this field. Both investigators and angiographic committee filled out… More >

  • Open Access

    EDITORIAL

    Consolidate or constipate: What are we going to do with all our registries?

    Andrew N. Redington

    Congenital Heart Disease, Vol.12, No.5, pp. 559-560, 2017, DOI:10.1111/chd.12527

    Abstract This article has no abstract. More >

  • Open Access

    ARTICLE

    Educational intervention for improving the appropriateness of transthoracic echocardiograms ordered by pediatric cardiologists

    Ritu Sachdeva1, Pamela S. Douglas2, Michael S. Kelleman1, Courtney E. McCracken1, Leo Lopez3, Kenan W.D. Stern4, Benjamin W. Eidem5, Oscar J. Benavidez6, Rory B. Weiner6, Elizabeth Welch3, Robert M. Campbell1, Wyman W. Lai7

    Congenital Heart Disease, Vol.12, No.3, pp. 373-381, 2017, DOI:10.1111/chd.12455

    Abstract Objective: The objective of this study was to evaluate effectiveness of educational intervention (EI) in the Pediatric Appropriate Use of Echocardiography (PAUSE) study to improve appropriateness of transthoracic echocardiograms (TTEs) ordered in pediatric cardiology clinics.
    Design: Data were prospectively collected after the publication of the Appropriate Use Criteria (AUC) document during 2 phases: the pre-EI phase (1/1/15 to 4/30/15) and the post-EI phase (7/ 1/15 to 10/30/15). Pre-EI, site-investigators (SI) determined AUC indications, by reviewing the clinic records. Post-EI, providers assigned indications prior to obtaining TTE.
    Setting: Pediatric cardiology clinics at six centers.
    Patients: Those ≤18 years old, receiving… More >

  • Open Access

    ARTICLE

    Practice variability in management of infectious issues in heterotaxy: A survey of pediatric cardiologists

    Rohit S. Loomba1, Gabrielle Geddes1, Amanda J. Shillingford2, David A. Hehir2

    Congenital Heart Disease, Vol.12, No.3, pp. 332-339, 2017, DOI:10.1111/chd.12448

    Abstract Background: Splenic dysfunction is common in heterotaxy syndrome, and increases the risk of bacteremia and bacteremia related mortality. Despite the risks associated with bacteremia in this setting, best practice guidelines for management of infectious concerns are lacking. We conducted a survey of pediatric cardiologists to characterize practice regarding the diagnosis of splenic dysfunction, approach to antibiotic prophylaxis, and management of possible bacterial infection.
    Methods: A 22-item web-based survey was distributed via email to pediatric cardiologists in North America.
    Results: We received 230 responses from 63 centers, for a response rate of 22%. The majority (83%) always obtain abdominal… More >

  • Open Access

    ARTICLE

    A population-based analysis of mortality in patients with Turner syndrome and hypoplastic left heart syndrome using the Texas Birth Defects Registry

    Diego A. Lara1, Mary K. Ethen2, Mark A. Canfield2, Wendy N. Nembhard3, Shaine A. Morris1

    Congenital Heart Disease, Vol.12, No.1, pp. 105-112, 2017

    Abstract Background: Hypoplastic left heart syndrome (HLHS) is strongly associated with Turner syndrome (TS); outcome data when these conditions coexist is sparse. We aimed to investigate long-term survival and causes of death in this population.
    Methods: The Texas Birth Defects Registry was queried for all live born infants with HLHS during 1999–2007. We used Kaplan–Meier and Cox regression analyses to compare survival among patients with HLHS with TS (HLHS/TS+) to patients who had HLHS without genetic disorders or extracardiac birth defects (HLHS/TS–).
    Results: Of the 542 patients with HLHS, 11 had TS (2.0%), 71 had other extracardiac birth defects… More >

  • Open Access

    ARTICLE

    In vitro antifungal/fungistatic activity of manganese phosphite against soybean soil-borne pathogens

    Carmona MA1, E Simonetti2,3, ME Ravotti1, MM Sc,iani4, AG Luque4, NA Formento5, FJ Sautua1

    Phyton-International Journal of Experimental Botany, Vol.86, pp. 265-269, 2017, DOI:10.32604/phyton.2017.86.265

    Abstract Soybean root and stem rots caused by soil-borne pathogens are diseases commonly found in soybean fields, and one of the most important causes of crop losses. In the present study, the mycelial sensitivity of Fusarium virguliforme, F. tucumaniae, Sclerotinia sclerotiorum and Macrophomina phaseolina was evaluated on potato dextrose agar media (25 mL) supplemented with different concentrations of manganese phosphite (MnPhi) diluted in water (0, 25, 37.5, 50, 100, 200, 300, 400, 500, 800 and 1000 μg/mL). Mycelial growth sensitivity was analyzed using logarithmic linear regression analysis. The MnPhi concentration needed to inhibit 50% of the mycelial growth (IC50) More >

  • Open Access

    ARTICLE

    Classifying Machine Learning Features Extracted from Vibration Signal with Logistic Model Tree to Monitor Automobile Tyre Pressure

    P. S. Anoop1, V. Sugumaran2

    Structural Durability & Health Monitoring, Vol.11, No.2, pp. 191-208, 2017, DOI:10.3970/sdhm.2017.011.191

    Abstract Tyre pressure monitoring system (TPMS) is compulsory in most countries like the United States and European Union. The existing systems depend on pressure sensors strapped on the tyre or on wheel speed sensor data. A difference in wheel speed would trigger an alarm based on the algorithm implemented. In this paper, machine learning approach is proposed as a new method to monitor tyre pressure by extracting the vertical vibrations from a wheel hub of a moving vehicle using an accelerometer. The obtained signals will be used to compute through statistical features and histogram features for More >

  • Open Access

    ARTICLE

    ABT-737, a Bcl-2 Selective Inhibitor, and Chloroquine Synergistically Kill Renal Cancer Cells

    Pei Yin*1, Jinpeng Jia†1, Jijun Li*, Yan Song*, Yiyan Zhang*, Fengkun Chen*

    Oncology Research, Vol.24, No.1, pp. 65-72, 2016, DOI:10.3727/096504016X14587366983838

    Abstract Renal cell carcinoma (RCC) is the most common malignancy in the kidney in the world, and the 5-year overall survival for patients remains poor due to the lack of effective treatment strategies. Although ABT-737, as a Bcl-2/Bcl-xL inhibitor, has recently emerged as a novel cancer therapeutic reagent, apoptosis induced by ABT-737 is often blocked in several types of cancer cells. This study investigated whether the combination of the small-molecule BH3 mimetic ABT-737 and the lysosome inhibitor chloroquine was an effective strategy for treating renal cancer cells. We found that the combination of ABT-737 and chloroquine… More >

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