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  • Open Access

    ARTICLE

    Practice trends over time in the care of infants with hypoplastic left heart syndrome: A report from the National Pediatric Cardiology Quality Improvement Collaborative

    Waldemar F. Carlo1, James F. Cnota2, Robert J. Dabal3, Jeffrey B. Anderson2

    Congenital Heart Disease, Vol.12, No.3, pp. 315-321, 2017, DOI:10.1111/chd.12442

    Abstract Objective: The National Pediatric Cardiology Quality Improvement Collaborative (NPC-QIC) was established in 2008 to improve outcomes of hypoplastic left heart syndrome (HLHS) during the interstage period. They evaluated changes in patient variables and practice variation between early and late eras.
    Design: Data including demographic, operative, discharge, and follow-up variables from the first 100 patients (6/2008–1/2010) representing 18 centers were compared with the most recent 100 patients (1/2014–11/2014) from these same centers.
    Results: Prenatal diagnosis increased from 69% to 82% (P = .05). There were no differences in gestational age or weight at Norwood. A composite of any preoperative… More >

  • Open Access

    ARTICLE

    Residents’ understanding of adult congenital heart disease

    Paul Cooper1, Morgan Hindes2, Timothy M. Maul3,4, Stephen C. Cook5

    Congenital Heart Disease, Vol.12, No.3, pp. 309-314, 2017, DOI:10.1111/chd.12441

    Abstract Objective: Medical residents are exposed to increasing numbers of adults with congenital heart disease (ACHD). While inadequate ACHD knowledge may lead to inappropriate practice, this educational deficit has not been investigated. Our aim was to analyze residents’ attitudes, perceived ability, and knowledge of ACHD medicine.
    Design, Methods, Outcome Measures: A single center, multiprogram cross-sectional study was conducted in 2015 using an electronic survey to assess 472 medical residents’ perceived knowledge and self-assessed skills related to ACHD medicine. Demographic data obtained included age, gender, level of training and program type.
    Results: The survey was completed by 25% of surveyed… More >

  • Open Access

    ARTICLE

    Reducing variation in feeding newborns with congenital heart disease

    Janet M. Simsic, Kirby-Rose Carpenito, Kristin Kirchner, Stephanie Peters, PNP-AC, Holly Miller-Tate, Brian Joy, Mark Galantowicz

    Congenital Heart Disease, Vol.12, No.3, pp. 275-281, 2017, DOI:10.1111/chd.12435

    Abstract Objective: Enteral feeding is associated with decreased infection rates, decreased mechanical ventilation, decreased hospital length of stay, and improved wound healing. Enteral feeding difficulties are common in congenital heart disease. Our objective was to develop experience-based newborn feeding guidelines for the initiation and advancement of enteral feeding in the cardiothoracic intensive care unit.
    Design: This is a retrospective analysis of a quality improvement project.
    Setting: This quality improvement project was performed in a cardiothoracic intensive care unit.
    Patients: Newborns admitted to the cardiothoracic intensive care unit for cardiac surgery from January 2011 to May 2015 were retrospectively reviewed.
    Intervention: Newborn… More >

  • Open Access

    ARTICLE

    A great imitator in adult cardiology practice: congenitally corrected transposition of the great arteries

    Anushree Agarwal, Fatima Samad, Lindsey Kalvin, Michelle Bush, A. Jamil Tajik

    Congenital Heart Disease, Vol.12, No.2, pp. 143-152, 2017, DOI:10.1111/chd.12453

    Abstract Introduction: Congenitally corrected transposition of the great arteries (ccTGA) is a rare congenital disease that frequently remains undiagnosed until adulthood, especially when there is an absence of other congenital anomalies. Adults with ccTGA may remain asymptomatic and their diagnosis could be missed on initial evaluation, or it could be diagnosed incidentally as an evaluation of murmur. We aim to report the different presentations of ccTGA in eight adult patients and review the key features required to suspect the diagnosis during an initial visit.
    Cases: We present some illustrative cases of ccTGA patients who had diverse presentations ranging… More >

  • Open Access

    ARTICLE

    Neurocognitive functioning in adults with congenital heart disease

    Dawn Ilardi1,2, Kim E. Ono1,2, Rebecca McCartney3, Wendy Book4, Anthony Y. Stringer2

    Congenital Heart Disease, Vol.12, No.2, pp. 166-173, 2017, DOI:10.1111/chd.12434

    Abstract Objective: Adults with congenital heart disease (CHD) are at increased risk of psychological disorders and cognitive deficiencies due to structural/acquired neurological abnormalities and neurodevelopmental disorders as children. However, limited information is known about the neuropsychological functioning of adults with CHD. This study screened neuropsychological abilities and explored group differences related to cardiac disease severity and neurological risk factors in adults with CHD.
    Design: Participants completed brief neuropsychological testing. Information about neurobehavioral and psychological symptoms, employment, education, and disability were also collected from the patient and a family member.
    Results: Forty-eight participants with adult CHD completed neuropsychological testing. Visuospatial… More >

  • Open Access

    ARTICLE

    Prevalence and pattern of executive dysfunction in school age children with congenital heart disease

    Jacqueline H. Sanz1,2, Madison M. Berl1,2, Anna C. Armour1, Jichuan Wang3,4, Yao I. Cheng3, Mary T. Donofrio5,6

    Congenital Heart Disease, Vol.12, No.2, pp. 202-209, 2017, DOI:10.1111/chd.12427

    Abstract Objective: Executive function, a set of cognitive skills important to social and academic outcomes, is a specific area of cognitive weakness in children with congenital heart disease (CHD). We evaluated the prevalence and profile of executive dysfunction in a heterogeneous sample of school aged children with CHD, examined whether children with executive dysfunction are receiving school services and support, and identified risk factors for executive dysfunction at school age.
    Design: Ninety-one school aged patients completed questionnaires, including the Behavior Rating Inventory of Executive Function (BRIEF) and a medical history questionnaire. An age- and gendermatched control sample was… More >

  • Open Access

    ARTICLE

    Cerebral tissue oxygenation index and lactate at 24 hours postoperative predict survival and neurodevelopmental outcome after neonatal cardiac surgery

    Safwat A. Aly1, David Zurakowski2, Penny Glass3, Kami Skurow-Todd4, Richard A. Jonas5, Mary T. Donofrio4

    Congenital Heart Disease, Vol.12, No.2, pp. 188-195, 2017, DOI:10.1111/chd.12426

    Abstract Importance: There are no well-established noninvasive biomarkers for identifying patients at risk for poor outcome after surgery for congenital heart disease. Few studies have assessed prognostic accuracy of cerebral tissue oxygenation index (cTOI) measured by near infrared spectroscopy (NIRS).
    Objective: To assess the utility of noninvasive NIRS monitoring as a predictor of outcomes after neonatal cardiac surgery through measurement of cTOI. To examine the utility of noninvasive NIRS monitoring in combination with lactate concentration and inotropic score in prediction of outcomes after neonatal cardiac surgery.
    Design: Prospective longitudinal cohort study.
    Setting: Operating room and cardiac intensive care unit, Children’s… More >

  • Open Access

    ARTICLE

    Use of 3D models of congenital heart disease as an education tool for cardiac nurses

    Giovanni Biglino1,2, Claudio Capelli2,3, Despina Koniordou3, Di Robertshaw2, Lindsay-Kay Leaver2, Silvia Schievano2,3, Andrew M. Taylor2,3, Jo Wray2

    Congenital Heart Disease, Vol.12, No.1, pp. 113-118, 2017

    Abstract Background: Nurse education and training are key to providing congenital heart disease (CHD) patients with consistent high standards of care as well as enabling career progression. One approach for improving educational experience is the use of 3D patient-specific models.
    Objectives: To gather pilot data to assess the feasibility of using 3D models of CHD during a training course for cardiac nurses; to evaluate the potential of 3D models in this context, from the nurses’ perspective; and to identify possible improvements to optimise their use for teaching.
    Design: A cross-sectional survey.
    Setting: A national training week for cardiac nurses.
    Participants: One… More >

  • Open Access

    ARTICLE

    Neurocognitive and executive functioning in adult survivors of congenital heart disease

    Leda Klouda1, Wayne J. Franklin1, Anita Saraf1,2, Dhaval R. Parekh1, David D. Schwartz3

    Congenital Heart Disease, Vol.12, No.1, pp. 91-98, 2017

    Abstract Objective: Congenital heart disease (CHD) can affect the developing central nervous system, resulting in neurocognitive and behavioral deficits. Preoperative neurological abnormalities as well as sequelae of the open heart operations required to correct structural abnormalities of the heart contribute to these deficits. There are few studies examining the neurocognitive functioning of adults with CHD. This study sought to investigate multiple domains of neurocognitive functioning in adult survivors of CHD who had childhood cardiac surgery with either moderate or severe disease complexity.
    Design: A total of 48 adults (18–49 years of age) who had undergone cardiac surgery for… More >

  • Open Access

    ARTICLE

    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)

    Benjamin H. Goot, Sonali Patel, Brian Fonseca

    Congenital Heart Disease, Vol.12, No.1, pp. 84-90, 2017

    Abstract 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.
    Design: Patients with congenital heart disease who underwent a cardiac MRI (CMR) that included a delayed VIBE sequence from 5/2008… More >

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