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  • Open Access

    ARTICLE

    Pulmonary artery interventions after the arterial switch operation: Unique and significant risks

    Jesse Lee, Doaa Abdullah Shahbah, Howaida El‐Said, Rodrigo Rios, Kanishka Ratnayaka, John Moore

    Congenital Heart Disease, Vol.14, No.2, pp. 288-296, 2019, DOI:10.1111/chd.12726

    Abstract Background: In the modern era, results of the arterial switch operation (ASO) for transposition of the great arteries are excellent. However, because of the LeCompte maneuver, there may be a propensity for development of pulmonary artery stenosis. We encountered atypical complications of pulmonary artery stenting in patients after the ASO, including aorto‐pulmonary fistula and coronary compression.
    Methods: We performed a 10‐year retrospective review of catheterizations per‐ formed in patients after ASO in our institution with a focus on adverse events.
    Results: Diagnostic and interventional catheterizations were performed in 47 pa‐ tients. In 29 patients, 37 interventional procedures performed,… More >

  • Open Access

    REVIEW

    Pediatric dysautonomia: Much‐maligned, often overmedicated, but not as complex as you think

    Camden L. Hebson1, Michael E. McConnell2, David W. Hannon3

    Congenital Heart Disease, Vol.14, No.2, pp. 156-161, 2019, DOI:10.1111/chd.12720

    Abstract Dysautonomia is an increasingly recognized yet still poorly understood disease within the field of pediatrics. Symptoms, including dizziness, headaches, fatigue, joint pain, anxiety, and intolerance of heat or cold, are often significant and difficult to sort, especially in terms of their relation to each other. This often leads to referral to multiple subspecialists, who then proceed to treat seemingly familiar symptoms in kind. In the authors’ experience, this leads to more frustration on the part of the patients and their physicians when symptom improvement does not follow (or can even worsen). On the other hand, More >

  • Open Access

    ARTICLE

    Vasopressor magnitude predicts poor outcome in adults with congenital heart disease after cardiac surgery

    Joseph T. Poterucha1, Saraschandra Vallabhajosyula2, Alexander C. Egbe2, Joseph S. Krien3, Devon O. Aganga4, Kimberly Holst5, Adele W. Golden6, Joseph A. Dearani5, Sheri S. Crow4

    Congenital Heart Disease, Vol.14, No.2, pp. 193-200, 2019, DOI:10.1111/chd.12717

    Abstract Background: High levels of vasoactive inotrope support (VIS) after congenital heart surgery are predictive of morbidity in pediatric patients. We sought to discern if this relationship applies to adults with congenital heart disease (ACHD).
    Methods: We retrospectively studied adult patients (≥18 years old) admitted to the intensive care unit after cardiac surgery for congenital heart disease from 2002 to 2013 at Mayo Clinic. Vasoactive medication dose values within 96 hours of admis‐ sion were examined to determine the relationship between VIS score and poor out‐ come of early mortality, early morbidity, or complication related morbidity.
    Results: Overall, 1040… More >

  • Open Access

    ARTICLE

    Historical investigation of medical treatment for adult congenital heart disease: A Canadian perspective

    Jennifer L. Lapum1, Suzanne Fredericks1, Barbara Bailey2, Terrence M. Yau3,4, Jennifer Graham5, Ariane J. Marelli6,7

    Congenital Heart Disease, Vol.14, No.2, pp. 185-192, 2019, DOI:10.1111/chd.12716

    Abstract Objective: The number of adults with congenital heart disease (CHD) has increased substantially because of medical advances that are extending life expectancy beyond childhood. A historical investigation is timely not only because of this significant demographic shift, but also because stakeholder engagement is increasingly important in shaping research directions. The study questions were: From the perspective of stakeholders, what is known about the medical treatment for the adult CHD cohort in Canada and how has it changed over time?
    Design/Methods: We harnessed the use of historical research methods and conducted interviews with 21 key informants in order… More >

  • Open Access

    ARTICLE

    Application of pediatric Appropriate Use Criteria for initial outpatient evaluation of asymptomatic patients with abnormal electrocardiograms

    Soham Dasgupta1, Shae Anderson1, Michael Kelleman2, Ritu Sachdeva1

    Congenital Heart Disease, Vol.14, No.2, pp. 230-235, 2019, DOI:10.1111/chd.12687

    Abstract Introduction: In the pediatric Appropriate Use Criteria (AUC), abnormal electrocardiogram (ECG) in an asymptomatic patient has been rated as an “Appropriate” indication for transthoracic echocardiogram (TTE). We hypothesized that the yield of abnormal findings on TTE for this indication will be low.
    Methods: All asymptomatic patients (≤ 18 years) from January 1, 2015 to December 31, 2017 who underwent initial outpatient evaluation at our center and had a TTE ordered for an abnormal ECG, were included. Clinic records were reviewed to obtain ECG and TTE findings.
    Results: Of the 199 study patients, 13 (6.5%) had abnormal findings. Incomplete… More >

  • Open Access

    ARTICLE

    Bioelectrical impedance analysis in the management of heart failure in adult patients with congenital heart disease

    Masaki Sato1, Kei Inai1,2, Mikiko Shimizu1, Hisashi Sugiyama1, Toshio Nakanishi2

    Congenital Heart Disease, Vol.14, No.2, pp. 167-175, 2019, DOI:10.1111/chd.12683

    Abstract Objective: The recognition of fluid retention is critical in treating heart failure (HF). Bioelectrical impedance analysis (BIA) is a well-known noninvasive method; however, data on its role in managing patients with congenital heart disease (CHD) are limited. Here, we aimed to clarify the correlation between BIA and HF severity as well as the prognostic value of BIA in adult patients with CHD.
    Design: This prospective single-center study included 170 patients with CHD admitted between 2013 and 2015. We evaluated BIA parameters (intra- and extracellular water, protein, and mineral levels, edema index [EI, extracellular water-to-total body water ratio]),… More >

  • Open Access

    ARTICLE

    What is a hemodynamically significant PDA in preterm infants?

    Jennifer L. Shepherd, Shahab Noori

    Congenital Heart Disease, Vol.14, No.1, pp. 21-26, 2019, DOI:10.1111/chd.12727

    Abstract Objective: There is no consensus on the definition of a hemodynamically significant patent ductus arteriosus (hsPDA). In this review article, our objective is to discuss the main variables that one should consider when determining the hemodynamic signifi‐ cance of a PDA.
    Results: We describe the various approaches that have been utilized over time to define an hsPDA and discuss the strengths and weaknesses of each echocardio‐ graphic index. Finally, we propose a comprehensive and individualized approach in determining the hemodynamic significance of the PDA.
    Conclusion: There are several PDA‐related clinical, echocardiographic, and other ob‐ jective variables to take More >

  • Open Access

    ARTICLE

    Molecular and mechanical factors contributing to ductus arteriosus patency and closure

    Stacey L. Crockett, Courtney D. Berger, Elaine L. Shelton, Jeff Reese

    Congenital Heart Disease, Vol.14, No.1, pp. 15-20, 2019, DOI:10.1111/chd.12714

    Abstract Regulation of the ductus arteriosus, an essential fetal vessel connecting the pulmo‐ nary artery and aorta, is complex. Failure of this vessel to close after birth may result in a persistent left‐to‐right shunt through the patent ductus arteriosus, a condition associated with significant morbidities. Numerous factors contribute to the shift from fetal ductus patency to postnatal closure, requiring precise coordination of molecular cues with biomechanical forces and underlying genetic influences. Despite significant advances, questions remain regarding signaling dynamics and the natural time course of ductus closure, particularly in preterm neonates. This review highlights the contri‐ More >

  • Open Access

    ARTICLE

    Comparative case studies: PDAs treated with medication, surgical ligation, and transcatheter device closure

    Ruth Seaton, Chandler Williams, Julia Peredo

    Congenital Heart Disease, Vol.14, No.1, pp. 65-68, 2019, DOI:10.1111/chd.12711

    Abstract The ductus arteriosus is a lifeline for the developing fetus prior to delivery, allowing the circulation of oxygen‐rich blood from the placenta to bypass the lungs and per‐ fuse the body. However, when the ductus fails to close after birth, the pressures can cause blood to shunt from the aorta back into the lungs, causing pulmonary edema. This is called a left‐to‐right shunt. The patent ductus arteriosus (PDA) can also shunt blood from the pulmonary arteries to the aorta, bypassing the lungs and causing oxy‐ gen‐poor blood to mix with the oxygen‐rich blood circulating to More >

  • Open Access

    ARTICLE

    Effect of Patent Ductus Arteriosus on Pulmonary Vascular Disease

    Ranjit Philip1, Jason Nathaniel Johnson1,2, Ronak Naik1, Dai Kimura1,3, Umar Boston1, Sandeep Chilakala1, Benjamin Hendrickson1, Benjamin Rush Waller1, Shyam Sathanandam1

    Congenital Heart Disease, Vol.14, No.1, pp. 37-41, 2019, DOI:10.1111/chd.12702

    Abstract The hemodynamic effects of a patent ductus arteriosus (PDA) are well known including systemic hypoperfusion and volume overload on the left ventricle. This article aims to provide a review of the long-standing effect of a hemodynamically significant PDA on the pulmonary vasculature and the role of cardiac catheterization in preterm infants with a PDA and pulmonary hypertension. More >

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