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  • Open Access

    ARTICLE

    Age is not a good predictor of irreversibility of pulmonary hypertension in congenital cardiac malformations with left-to-right shunt

    Amir-Reza Hosseinpour1, Marie-Hélène Perez2, David Longchamp2, Jacques Cotting2, Nicole Sekarski3, Michel Hurni1, René Prêtre1, Stefano Di Bernardo3

    Congenital Heart Disease, Vol.13, No.2, pp. 210-216, 2018, DOI:10.1111/chd.12545

    Abstract Objective: Congenital cardiac malformations with high pulmonary blood flow and pressure due to left-to-right shunts are usually repaired in early infancy for both the benefits of early relief of heart failure and the fear that the concomitant pulmonary hypertension may become irreversible unless these defects are corrected at an early age. Age, however, has been a poor predictor of irreversibility of pulmonary hypertension in our experience, which is presented here.
    Design: A retrospective observational study. We defined “late” as age ≥2 years. We examined clinical, echocardiographic, and hemodynamic data from all patients aged ≥2 years with such… More >

  • Open Access

    ARTICLE

    Arrhythmia after cone repair for Ebstein anomaly: The Mayo Clinic experience in 143 young patients

    Philip Wackel1,2, Bryan Cannon1,2, Joseph Dearani3, Kristen Sessions1,2, Kimberly Holst3, Jonathan Johnson1,2, Frank Cetta1,2

    Congenital Heart Disease, Vol.13, No.1, pp. 26-30, 2018, DOI:10.1111/chd.12566

    Abstract Background: The increased incidence of preoperative and postoperative arrhythmia in Ebstein anomaly (EA) prompted some clinicians to perform an electrophysiology study (EPS) in all patients prior to surgery for EA. The cone repair (CR) is the current surgical option of choice for most young patients with EA but the effect of the CR on arrhythmia is not well established.
    Objectives: To assess the burden of arrhythmia in young patients after CR and to assess the utility of selective preoperative EPS.
    Materials and Methods: A retrospective review of all patients <21 years of age with EA who had a… More >

  • Open Access

    REVIEW

    State of the art review: Chemotherapy-induced cardiotoxicity in children

    Robert W. Loar, Cory V. Noel, Hari Tunuguntla, John L. Colquitt, Ricardo H. Pignatelli

    Congenital Heart Disease, Vol.13, No.1, pp. 5-15, 2018, DOI:10.1111/chd.12564

    Abstract Chemotherapy-induced cardiotoxicity in adults and children is a topic with a growing interest in the cardiology literature. The ability to detect cardiac dysfunction in a timely manner is essential in order to begin adequate treatment and prevent further deterioration. This article aims to provide a review on the myocardial injury process, chemotherapeutic agents that lead to cardiotoxicity, the definition of cardiotoxicity, and the methods of timely detection and treatment. More >

  • Open Access

    ARTICLE

    The 745.5 issue in code-based, adult congenital heart disease population studies: Relevance to current and future ICD-9-CM and ICD-10-CM studies

    Fred H. Rodriguez III1,2,3,4, Georges Ephrem1,2, Jennifer F. Gerardin1,2, Cheryl Raskind-Hood5, Carol Hogue5, Wendy Book1,2

    Congenital Heart Disease, Vol.13, No.1, pp. 59-64, 2018, DOI:10.1111/chd.12563

    Abstract Objective: Although the ICD-9-CM code 745.5 is widely used to indicate the presence of a secundum atrial septal defect (ASD), it is also used for patent foramen ovale (PFO) which is a normal variant and for “rule-out” congenital heart disease (CHD). The ICD-10-CM code Q21.1 perpetuates this issue. The objective of this study was to assess whether code 745.5 in isolation or in combination with unspecified CHD codes 746.9 or 746.89 miscodes for CHD, and if true CHD positives decrease with age.
    Design: Echocardiograms of patients with an ICD-9-CM code of 745.5 in isolation or in combination… More >

  • Open Access

    ARTICLE

    Teaching pediatric cardiology with meaning and sense

    Joshua A. Daily1,2, Elijah Bolin1,2, Brian K. Eble1,2

    Congenital Heart Disease, Vol.13, No.1, pp. 154-156, 2018, DOI:10.1111/chd.12561

    Abstract Pediatric cardiologists teach complicated concepts to a diverse group of learners that include medical students, nurses, residents, fellows, patients, and parents. Unfortunately, much of what is taught is not retained. In order to increase the likelihood of long-term retention, a cardiologist should teach with both meaning and sense. The authors provide a review of these concepts and give specific examples of how to teach in ways that both make sense and are meaningful to a cardiologist’s leaners. More >

  • Open Access

    ARTICLE

    Using a statewide survey methodology to prioritize pediatric cardiology core content

    Ashley E. Neal1, Elizabeth Lehto1, Karen Hughes Miller2, Craig Ziegler2, Erin Davis2

    Congenital Heart Disease, Vol.13, No.1, pp. 147-153, 2018, DOI:10.1111/chd.12559

    Abstract Objective: Although pediatrician-reported relevance of Canadian cardiology-specific objectives has been studied, similar data are not available for the 2016 American Board of Pediatrics (ABP) cardiology-specific objectives. This study asked Kentucky trainees, pediatricians, and pediatric cardiologists to identify “most important” content within these objectives.
    Design, Methods, Outcome Measures: This cross-sectional study used an original, online survey instrument based on the 2016 ABP cardiology-specific objectives. We collected quantitative data (numerical indications of importance) and qualitative data (open-ended replies regarding missing content and difficulty in teaching and learning). Respondents indicated the top two choices of most important items within eight… More >

  • Open Access

    ARTICLE

    A composite noninvasive index correlates with liver fibrosis scores in post-Fontan patients: Preliminary findings

    William N. Evans1,2, Ruben J. Acherman1,2, Michael L. Ciccolo1,3, Sergio A. Carrillo1,2, Alvaro Galindo1,2, Abraham Rothman1,2, Gary A. Mayman1,2, Elizabeth A. Adams1,2, Leigh C. Reardon1,4, Brody J. Winn5, Noel S. Yumiaco5, Lesley Shimuizu1, Yoko Inanaga1, Rowena J. Deleon1, Humberto Restrepo1,2

    Congenital Heart Disease, Vol.13, No.1, pp. 38-45, 2018, DOI:10.1111/chd.12558

    Abstract Objective: We hypothesized that clinic-based, hepatic-ultrasound, elastography measurements, either alone or in combination with other noninvasive variables, might correlate with liver-biopsy fibrosis scores in patients post-Fontan.
    Methods: Between March 2012 and February 2017, we identified patients post-Fontan that underwent elective cardiac catheterization and simultaneous transvenous hepatic biopsy. From this group, we selected patients that met inclusion criteria for liver-ultrasound, shear-wave elastography. Utilizing the results of elastography, laboratory testing, and time post-Fontan, we constructed a composite Fontan hepatic index as a sum of elastography measurements in kilopascals, model for end-stage liver disease excluding INR scores, and the square… More >

  • Open Access

    REVIEW

    Heparin-induced thrombocytopenia complicating children after the Fontan procedure: Single-center experience and review of the literature

    Uri Pollak1,2,3, David Mishaly3,4, Gili Kenet3,5, Amir Vardi1,3

    Congenital Heart Disease, Vol.13, No.1, pp. 16-25, 2018, DOI:10.1111/chd.12557

    Abstract Heparin-induced thrombocytopenia (HIT) is a life-threatening complication of heparin therapy. The risk for HIT correlates with the cumulative dosage of heparin exposure. In Fontan patients, recurrent systemic anticoagulation, traditionally with heparin, is used to alleviate the thrombotic complications that may occur postoperatively when the venous pressure rises and the systemic venous flow into the pulmonary arteries becomes sluggish, putting them at increased risk. As a pressure gradient-dependent circulation, elevation in systemic venous pressure, most often by venous thrombosis, contributes to circuit failure. Therefore, when HIT complicates patients after the Fontan procedure, it is associated with More >

  • Open Access

    ARTICLE

    Body composition, appetite-related hormones, adipocytokines, and heart failure in adult patients with congenital heart disease: A preliminary study

    Yumi Shiina1, Tomoaki Murakami2, Noriko Matsumoto3, Daisuke Okamura4, Yuta Takahashi4, Yosuke Nishihata1, Nobuyuki Komiyama1, Koichiro Niwa1

    Congenital Heart Disease, Vol.13, No.1, pp. 79-84, 2018, DOI:10.1111/chd.12555

    Abstract Objectives: To assess body composition and relationships among body composition, appetiterelated hormones, adipocytokines, and heart failure (HF) in adult patients with congenital heart disease (CHD).
    Patients: This prospective study enrolled 46 consecutive adult patients with CHD and 12 agematched healthy controls. The patients and control subjects were divided into four groups: 13 patients with Fontan circulation (group A), 16 patients with cyanosis (group B), 17 patients who previously underwent biventricular repair (group C), and 12 age-matched healthy controls.
    Design: Body composition was measured using InBody730, and levels of appetite-related hormones (ghrelin and leptin) and adipocytokines (leptin, interleukin-6, and… More >

  • Open Access

    ARTICLE

    Toward standardization of care: The feeding readiness assessment after congenital cardiac surgery

    Daniel E. Ehrmann1, Matthew Mulvahill2, Shaunda Harendt1,3, Jessica Church1, Amy Stimmler1, Piyagarnt Vichayavilas1,4, Sanja Batz1,5, Jennifer Rodgers1,5, Michael DiMaria1, James Jaggers1, Cindy Barrett1, Jon Kaufman1

    Congenital Heart Disease, Vol.13, No.1, pp. 31-37, 2018, DOI:10.1111/chd.12550

    Abstract Background: Feeding practices after neonatal and congenital heart surgery are complicated and variable, which may be associated with prolonged hospitalization length of stay (LOS). Systematic assessment of feeding skills after cardiac surgery may earlier identify those likely to have protracted feeding difficulties, which may promote standardization of care.
    Methods: Neonates and infants ≤3 months old admitted for their first cardiac surgery were retrospectively identified during a 1-year period at a single center. A systematic feeding readiness assessment (FRA) was utilized to score infant feeding skills. FRA scores were assigned immediately prior to surgery and 1, 2, and… More >

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