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  • 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 >

  • Open Access

    ARTICLE

    Hemodynamic effects of ketamine in children with congenital heart disease and/or pulmonary hypertension

    Rohit S. Loomba1, Seth B. Gray2, Saul Flores3

    Congenital Heart Disease, Vol.13, No.5, pp. 646-654, 2018, DOI:10.1111/chd.12662

    Abstract Introduction: Ketamine is a drug often used for procedural sedation or as adjunct agent for general sedation in children with congenital heart disease. In the clinical realm, there is often confusion regarding the effects of ketamine on hemodynamics, particularly pulmonary vascular resistance and systemic vascular resistance. We per‐ formed a meta‐analysis of studies investigating the effects of ketamine on hemodynamics.
    Methods: A systematic review was conducted to identify studies characterizing the hemodynamic effects of ketamine in children with congenital heart disease. Studies were assessed for quality and those of satisfactory quality with pre‐ and postketa‐ mine hemodynamics… More >

  • Open Access

    ARTICLE

    Platelet activation markers in children with congenital heart disease associated with pulmonary arterial hypertension

    Timur Mese1, Baris Guven2, Murat Muhtar Yilmazer1, Cem Karadeniz3, Rahmi Ozdemir1, Onder Doksoz1

    Congenital Heart Disease, Vol.13, No.4, pp. 506-511, 2018, DOI:10.1111/chd.12616

    Abstract Background: Mean platelet volume (MPV), platecrit, and platelet distribution width (PDW) are markers of platelet activation. Previous studies have found that platelet activation occurs in patients with pulmonary arterial hypertension. Platelet indices including MPV, PDW, and platecrit have not been studied in children with congenital heart disease associated pulmonary arterial hypertension (APAH-CHD) who survived and those who died.
    Objective: The objective of this study to investigate the value of platelet indices with clinical and hemodynamic indicators predicting the disease severity and survival in children with APAH-CHD.
    Methods: This was a nested case-control study. MPV, platecrit, and PDW levels… More >

  • Open Access

    ARTICLE

    Pulmonary hypertension: Barrier or just a bump in the road in transplanting adults with congenital heart disease

    Jonathan N. Menachem1, Edo Y. Birati2, Payman Zamani2, Anjali T. Owens2, Pavan Atluri3, Christian A. Bermudez3, David Drajpuch2, Stephanie Fuller4, Yuli Y. Kim2, Christopher E. Mascio4, Vikram Palanivel2, J. Eduardo Rame2, Joyce Wald2, Michael A. Acker3, Jeremy A. Mazurek2

    Congenital Heart Disease, Vol.13, No.4, pp. 492-498, 2018, DOI:10.1111/chd.12606

    Abstract Background: Heart failure (HF) is the leading cause of death in adults with congenital heart disease (ACHD). Identification of disease progression and timing of referral for advanced therapies is often delayed. However, increased awareness and understanding of ACHD and improvements in the approach to treatment have led to improved outcomes. Pulmonary hypertension (PH) is a common barrier to HT. In ACHD, the approach to PH and HT is quite complicated, given the anatomic heterogeneity and lower prevalence and experience. However, in some cases, PH is a result of elevated systemic filling pressures and low output.
    Methods: We… More >

  • Open Access

    ARTICLE

    NT-pro BNP—A marker for worsening respiratory status and mortality in infants and young children with pulmonary hypertension

    Shahnawaz M. Amdani1, Muhammad Umair M. Mian2, Ron L. Thomas3, Robert D. Ross4

    Congenital Heart Disease, Vol.13, No.4, pp. 499-505, 2018, DOI:10.1111/chd.12601

    Abstract Aim: To evaluate predictors of morbidity and mortality in pediatric patients with pulmonary hypertension (PH), laboratory and echocardiographic measures of PH were analyzed.
    Methods: A retrospective review of all infants and children< 2 years of age with PH from January 2011 to August 2016 was conducted. Correlations were determined using Spearman’s rank correlation coefficients. Differences in characteristics between survivors and nonsurvivors were analyzed and Kaplan-Meier survival curves were generated.
    Results: Of 56 patients, the majority were extremely premature; of African American ethnicity; and had bronchopulmonary dysplasia. Patients who died were more likely to have underlying congenital heart disease;… More >

  • 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

    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

    ARTICLE

    Arrhythmia burden and related outcomes in Eisenmenger syndrome

    Shankar Baskar1, Philippa Horne2, Samantha Fitzsimmons3, Philip R. Khoury1, Joseph Vettukattill4, Koichiro Niwa5, Teiji Agaki6, Mark Spence7, Hisanori Sakazaki8, Gruschen Veldtman1

    Congenital Heart Disease, Vol.12, No.4, pp. 512-519, 2017, DOI:10.1111/chd.12481

    Abstract Background: Patients with Eisenmenger syndrome have a shorter lifespan than the general population. A significant proportion develop arrhythmia and some, sudden death.
    Objective: The aims of this study were to characterize the frequency, type and effects of arrhythmias in adult patients with Eisenmenger’s syndrome and to identify risk factors for arrhythmias.
    Methods: This retrospective study included patients aged ≥18 years of age with Eisenmenger’s syndrome from three institutions. Arrhythmias were noted from electrocardiograms and Holter study reviews.
    Results: A total of 167 patients, 96 females, 63 males (gender not available in 9 patients) were included in this study. The… More >

  • Open Access

    ARTICLE

    Hemodynamic, biological, and right ventricular functional changes following intraatrial shunt repair in patients with flow-induced pulmonary hypertension

    Chih-Hsin Hsu1,2, Jun-Neng Roan1,3, Jieh-Neng Wang4, Chien-Chi Huang5, Chao-Jung Shih3, Jyh-Hong Chen2,6, Jing-Ming Wu4, Chen-Fuh Lam7,8

    Congenital Heart Disease, Vol.12, No.4, pp. 533-539, 2017, DOI:10.1111/chd.12479

    Abstract Objectives: Atrial septal defects may result in pulmonary hypertension and right heart remodeling. We analyzed improvements in patients with flow-induced pulmonary hypertension and the activation of endothelial progenitor cells after flow reduction.
    Design: This prospective cohort study included 37 patients who were admitted for an occluder implantation. Blood samples were collected before and after the procedure. We determined the number of endothelial progenitor cells in outgrowth colonies and serum Hsp27 concentrations. Daily performance and cardiothoracic ratio were reevaluated later.
    Results: Closure of the defect significantly reduced the pulmonary pressure and B-type natriuretic peptide levels. The cardiothoracic ratio and… More >

  • Open Access

    RESIDENT’S CORNER

    Selective arterial embolization of angiomyolipoma leading to pulmonary hypertension

    Franklin Lee, David S. Aaronson, Sarah Blaschko, Phil Barbosa, Alex K. Wu, Michael Eisenberg, Bradley A. Erickson, Jack W. McAninch

    Canadian Journal of Urology, Vol.17, No.3, pp. 5232-5235, 2010

    Abstract Objectives: To report two cases of secondary pulmonary hypertension resulting from microsphere extravasation following selective arterial embolization of renal angiomyolipoma, its diagnosis, and management.
    Methods: We reviewed the cases of two patients at the University of California, San Francisco, treated with selective arterial embolization for management of their angiomyolipoma (AML) using Tris-Acryl Gelatin Microspheres.
    Results: Both patients were women, ages 51 and 77. Indications for treatment were the following: Patient 1 was treated for a large asymptomatic AML. Patient 2 was treated for a symptomatic, bleeding AML. Both patients developed progressive hypoxia following selective arterial embolization using Tris-Acryl More >

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