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  • Open Access


    Incidence, morphology, and progression of bicuspid aortic valve in pediatric and young adult subjects with coexisting congenital heart defects

    Talha Niaz1, Joseph T. Poterucha1, Jonathan N. Johnson1,2, Cecilia Craviari1, Thomas Nienaber1, Jared Palfreeman1, Frank Cetta1,2, Donald J. Hagler1,2

    Congenital Heart Disease, Vol.12, No.3, pp. 261-269, 2017, DOI:10.1111/chd.12429

    Abstract Background: Bicuspid aortic valve (BAV) occurs both as an isolated cardiac lesion and in association with congenital heart defects (CHD). Their aim was to identify the incidence and morphology of BAV in patients with coexisting CHD and compare their disease progression to patients with isolated BAV.
    Methods: The Mayo Clinic echocardiography database was retrospectively analyzed to identify pediatric and young adult patients (≤22 years) who were diagnosed with BAV from 1990 to 2015. The morphology of BAV was determined from the echocardiographic studies before any intervention.
    Results: Overall, 1010 patients with BAV were identified, 619 (61%) with isolated… More >

  • Open Access


    Interventions in children with renovascular hypertension: A 27-year retrospective single-center experience

    Hitesh Agrawal1,2, Douglas Moodie1, Athar M. Qureshi1,2, Alisa A. Acosta3, Jose A. Hernandez4, Michael C. Braun3, Henri Justino1,2

    Congenital Heart Disease, Vol.13, No.3, pp. 349-356, 2018, DOI:10.1111/chd.12608

    Abstract Background: Renovascular hypertension (RVH) can be caused by renal artery stenosis (RAS) and/ or middle aortic syndrome (MAS).
    Methods: Patients who received surgical or transcatheter treatment for RVH between 1/1991 and 11/2017 were retrospectively reviewed using age = adjusted blood pressure ratio (BPR).
    Results: Fifty-three patients diagnosed with RVH at a median age of 4.5 (0–18) years were included. Vascular involvement ranged from MAS with RAS (20), RAS only (32), and MAS only (1). The first intervention was transcatheter in 47 patients (transcatheter group: angioplasty = 41, stenting = 5, and thrombectomy = 1), and surgical in 6… More >

  • Open Access


    Computational simulation of postoperative pulmonary flow distribution in Alagille patients with peripheral pulmonary artery stenosis

    Weiguang Yang1, Frank L. Hanley2, Frandics P. Chan3, Alison L. Marsden1,4, Irene E. Vignon-Clementel5, Jeffrey A. Feinstein1,4

    Congenital Heart Disease, Vol.13, No.2, pp. 241-250, 2018, DOI:10.1111/chd.12556

    Abstract Background: Up to 90% of individuals with Alagille syndrome have congenital heart diseases. Peripheral pulmonary artery stenosis (PPS), resulting in right ventricular hypertension and pulmonary flow disparity, is one of the most common abnormalities, yet the hemodynamic effects are illdefined, and optimal patient management and treatment strategies are not well established. The purpose of this pilot study is to use recently refined computational simulation in the setting of multiple surgical strategies, to examine the influence of pulmonary artery reconstruction on hemodynamics in this population.
    Materials and Methods: Based on computed tomography angiography and cardiac catheterization data, preoperative… More >

  • Open Access


    Pulmonary vein stenosis with collateralization via esophageal varices: Long-term follow-up after successful treatment with drug-eluting stent

    Jason F. Goldberg1, Craig L. Jensen2, Rajesh Krishnamurthy3, Nidhy P. Varghese4, Henri Justino1

    Congenital Heart Disease, Vol.13, No.1, pp. 124-130, 2018, DOI:10.1111/chd.12537

    Abstract Objective: We describe the long-term follow-up of a child with recurrent hemoptysis due to severe pulmonary vein stenosis decompressing via collaterals to esophageal varices.
    Design: Case report
    Setting: Tertiary children’s hospital
    Patient: Single child through ages 2- to 11-year old
    Interventions: The child underwent cutting balloon angioplasty, bare metal stenting, and implantation of a PTFE-covered stent, all of which failed rapidly. Only after placement of a paclitaxel drug eluting stent did he have prolonged relief from hemoptysis and long-term patency of the treated vein. The stents were serially dilated to keep up with somatic growth of the child, eventually culminating More >

  • Open Access


    Long‐term outcomes up to 25 years following balloon pulmonary valvuloplasty: A multicenter study

    Rachel L. Hansen1,2, Iman Naimi3, Hongyue Wang1, Nader Atallah3,4, Frank Smith3,4, Craig Byrum3,4, Daniel Kveselis3,4, Glenn Leonard1,5, Rajiv Devanagondi1, Matthew Egan3,4

    Congenital Heart Disease, Vol.14, No.6, pp. 1037-1045, 2019, DOI:10.1111/chd.12788

    Abstract Objective: Evaluate long‐term outcomes following balloon pulmonary valvuloplasty (BPV) for pulmonary stenosis (PS).
    Background: Long‐term data following BPV is limited to small, single center studies.
    Methods: BPV from April 12, 1985 to January 7, 2015 from three centers were included. Outcomes studied were ≥ moderate PI by echocardiogram and residual PS ≥ 40 mm Hg. Risk factors for ≥ moderate PI, residual PS, and repeat intervention were assessed by univariate and multivariate analysis.
    Results: Among 254 patients, mean age at BPV was 3.8 years (range 1 day‐67 years), initial PS catheter gradient was 56 mm Hg (IQR 40‐70), 19%… More >

  • Open Access


    Long‐term cardiovascular outcome of Williams syndrome

    Seul Gi Cha1, Mi Kyung Song1, Sang Yun Lee1, Gi Beom Kim1, Jae Gun Kwak2, Woong Han Kim2, Eun Jung Bae1

    Congenital Heart Disease, Vol.14, No.5, pp. 684-690, 2019, DOI:10.1111/chd.12810

    Abstract Objective: Cardiovascular lesions are the leading cause of morbidity and mortality in patients with Williams syndrome. Recent studies have rebutted conventional reports about the natural course of cardiovascular anomalies in Williams syndrome.
    Design: Retrospective study.
    Setting: Single tertiary center.
    Patients: Eighty patients with Williams syndrome followed up for more than 5 years.
    Interventions: Not applicable.
    Outcome Measures: Long‐term outcome of cardiovascular lesions, peak velocity change in obstructive cardiovascular lesions over time, post‐interventional courses of disease‐specific intervention, and intervention‐free survival of obstructive cardio‐ vascular lesions.
    Results: The median follow‐up duration was 11.0 (5.1‐28.3) years. Among 80 pa‐ tients, supravalvular aortic stenosis (87.5%) was… More >

  • Open Access


    Prevalence of Noonan spectrum disorders in a pediatric population with valvar pulmonary stenosis

    Kailyn Anderson1, James Cnota2,3, Jeanne James4,5, Erin M. Miller2,3, Ashley Parrott3, Valentina Pilipenko1,2, Kathryn Nicole Weaver1,2, Amy Shikany3

    Congenital Heart Disease, Vol.14, No.2, pp. 264-273, 2019, DOI:10.1111/chd.12721

    Abstract Objective: To evaluate the prevalence of Noonan spectrum disorders (NSD) in a pediatric population with valvar pulmonary stenosis (vPS) and identify the clinical characteristics that differentiate those with NSD from those without NSD.
    Design: A retrospective chart review of 204 patients diagnosed with vPS between 9/1/2012 and 12/1/2016 at a pediatric medical center was performed. The quantitative features of vPS, genetic diagnosis information, and phenotypic characteristics of Noonan syndrome were collected. Chi‐square test, Fisher’s exact test, t test, Wilcoxon rank‐sum test, and ANOVA were used for comparisons among the groups. Logistic regression was used to test for… More >

  • Open Access


    Bicuspid and Unicuspid Aortic Valve: Fate of Moderate/Severe Mixed Aortic Valve Disease

    Alexander C. Egbe*, Heidi M. Connolly*, Joseph T. Poterucha*,†, Carole A. Warnes*

    Congenital Heart Disease, Vol.12, No.1, pp. 24-31, 2017

    Abstract Objectives. There is a paucity of data about mixed aortic valve disease (MAVD) in patients with bicuspid/unicuspid aortic valve (BAV). This study sought to describe the outcomes of patients with moderate/severe MAVD.
    Methods. We queried our database for patients with BAV and moderate/severe MAVD seen between 1994 and 2013. We excluded patients with baseline New York Heart Association (NYHA) III/IV symptoms, left ventricular ejection fraction <50%, aortic dimension >50 mm, and significant disease of other valves. The purpose of the study was to determine the freedom from NYHA III/IV symptoms and aortic valve replacement (AVR).
    Results. We identified… More >

  • Open Access


    Effect of the Wall Thickness of the Vessel on FFRCT of Carotid Artery Stenosis

    Long Yu1, Kesong Xu1, Jun Wan2, Haiyan Lu3,*, Shengzhang Wang1,*

    CMES-Computer Modeling in Engineering & Sciences, Vol.121, No.3, pp. 835-844, 2019, DOI:10.32604/cmes.2019.07428

    Abstract Fractional flow reserve (FFR) computed from computed tomography angiography (CTA), i.e., FFRCT has been used in the clinic as a noninvasive parameter for functional assessment of coronary artery stenosis. It has also been suggested to be used in the assessment of carotid artery stenosis. The wall thickness of the vessel is an important parameter when establishing a fluid-structure coupling model of carotid stenosis. This work studies the effect of the vessel wall thickness on hemodynamic parameters such as FFRCT in carotid stenosis. Models of carotid stenosis are established based on CTA image data using computer-aided design… More >

  • Open Access


    Patient-Specific Computational Approach for Trans Catheter Aortic Valve Replacement (TAVR): Pre-Procedural Planning for Enhancing Performance and Clinical Outcomes

    Ram P. Ghosh1, Matteo Bianchi1, Gil Marom2, Oren M. Rotman1, Brandon Kovarovic1, Danny Bluestein1,*

    Molecular & Cellular Biomechanics, Vol.16, Suppl.2, pp. 12-14, 2019, DOI:10.32604/mcb.2019.07379

    Abstract This article has no abstract. More >

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