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  • Open Access

    ARTICLE

    Arteriovenous fistula creation for hypoxia after single ventricle palliation: A single‐institution experience and literature review

    Andrew D. Spearman1, Steven J. Kindel1, Ronald K. Woods2, Salil Ginde1,3

    Congenital Heart Disease, Vol.14, No.6, pp. 1199-1206, 2019, DOI:10.1111/chd.12828

    Abstract Background: Hypoxia is a common and sometimes severe morbidity of single ven‐ tricle congenital heart disease (CHD). Creation of an arteriovenous fistula (AVF) is occasionally performed for patients after superior or total cavopulmonary connec‐ tion (SCPC or TCPC) in an attempt to improve oxygen saturations. Despite previ‐ ous reports, AVF creation is a rare palliation with inadequately defined benefits and risks. We sought to determine changes in peripheral oxygen saturation (SpO2) and risk of adverse event after AVF creation in children with single ventricle CHD at our institution.
    Methods: We conducted a retrospective chart review of patients… More >

  • Open Access

    ARTICLE

    Postoperative and long‐term outcomes in children with Trisomy 21 and single ventricle palliation

    Jennifer K. Peterson1, Shaun P. Setty1,2, Jessica H. Knight3, Amanda S. Thomas4, James H. Moller5, Lazaros K. Kochilas4,6

    Congenital Heart Disease, Vol.14, No.5, pp. 854-863, 2019, DOI:10.1111/chd.12823

    Abstract Objective: Patients with Trisomy 21 (T21) and single ventricle (SV) physiology present unique challenges compared to euploidic counterparts. This study reports postoperative and long‐term outcomes in patients with T21 and SV palliation.
    Design: This retrospective cohort study from the Pediatric Cardiac Care Consortium (PCCC) included patients with T21 (<21 years old) that underwent surgical palliation for SV between 1982 and 2008 and control patients without known genetic anom‐ aly following Fontan palliation for similar diagnoses. Kaplan‐Meier survival plots were created based on death events obtained from the PCCC and by linkage with the National Death Index (NDI)… More >

  • Open Access

    ARTICLE

    Echocardiographic assessment of single‐ventricle diastolic function and its correlation to short‐term outcomes after the Fontan operation

    Erin K. Davis1, Salil Ginde1, Jessica Stelter2, Peter Frommelt1, Garick D. Hill3

    Congenital Heart Disease, Vol.14, No.5, pp. 720-725, 2019, DOI:10.1111/chd.12814

    Abstract Background/Hypothesis/Objectives: Postoperative complications after the Fontan operation for single ventricle heart disease are common and include persistent pleural drainage and prolonged length of hospital stay (LOS). Diastolic ventricular dysfunction may increase risk for postoperative complications by raising central venous pressures. We sought to determine the relationship between preoperative echocardiographic measurements of diastolic function, including myocardial deformation imaging, on (a) preoperative invasive catheterization measurements and (b) postoperative outcomes after the Fontan procedure.
    Design/Methods: All patients that underwent Fontan procedure from 2011 to 2017 were included. Echocardiograms performed within 6 months prior to Fontan operation were evaluated. Measurements of… More >

  • Open Access

    EDITORIAL

    Perspective. Digoxin for interstage single ventricle patients: What could possibly go wrong?

    George F. Van Hare

    Congenital Heart Disease, Vol.14, No.3, pp. 321-323, 2019, DOI:10.1111/chd.12760

    Abstract This article has no abstract. More >

  • Open Access

    Feeding methods for infants with single ventricle physiology are associated with length of stay during stage 2 surgery hospitalization

    Jeannine M. Hoch1, Oluwatosin Fatusin2, Gayane Yenokyan3, W. Reid Thompson2, Maureen A. Lefton‐Greif4

    Congenital Heart Disease, Vol.14, No.3, pp. 438-445, 2019, DOI:10.1111/chd.12742

    Abstract Background: Tube feedings are often needed to achieve the growth and nutrition goals associated with decreased morbidity and mortality in patients with single ventricle anat‐ omy. Variability in feeding method through the interstage period has been previously described, however, comparable information following stage 2 palliation is lacking.
    Objectives: To identify types of feeding methods following stage 2 palliation and their influence on length of stay.
    Design: Secondary analysis of the National Pediatric Cardiology Quality Improvement Collaborative registry was performed on 932 patients. Demographic data, medical characteristics, postoperative complications, type of feeding method, and length of stay for stage… More >

  • Open Access

    ARTICLE

    Focal liver lesions following Fontan palliation of single ventricle physiology: A radiology‐pathology case series

    Emily M. Engelhardt1, Andrew T. Trout2,3, Rachel M. Sheridan4, Gruschen R. Veldtman5, Jonathan R. Dillman2,3

    Congenital Heart Disease, Vol.14, No.3, pp. 380-388, 2019, DOI:10.1111/chd.12730

    Abstract Purpose: Patients who have undergone Fontan palliation of single ventricle physiol‐ ogy congenital heart disease are prone to developing focal liver lesions. In our experi‐ ence, the variety of lesions occurring in this population is greater than that described in the literature. The purpose of this study was to describe the breadth of biopsy‐ proven liver lesions in patients post–Fontan palliation of single ventricle physiology cared for at our institution.
    Methods: We retrospectively identified patients who had previously undergone the Fontan operation and had a focal liver lesion biopsied between January 2000 and June 2018. Medical records… More >

  • Open Access

    ARTICLE

    The effect of right ventricular function on survival and morbidity following stage 2 palliation: An analysis of the single ventricle reconstruction trial public data set

    Vanessa Marie Hormaza1, Mark Conaway2, Daniel Scott Schneider1, Jeffrey Eric Vergales1

    Congenital Heart Disease, Vol.14, No.2, pp. 274-279, 2019, DOI:10.1111/chd.12722

    Abstract Objective: Limited information is known on how right ventricular function affects outcomes after stage 2 palliation. We evaluated the impact of different right ventricular indices prior to stage 2 palliation on morbidity and mortality.
    Design: Retrospective study design.
    Setting: Pediatric Heart Network Single Ventricle Reconstruction Trial Public Data Set.
    Patient: Any variant of stage 1 palliation and all anatomic hypoplastic left heart syndrome variants in the trial were evaluated. Echocardiograms prior to stage 2 palliation were analyzed and compared between those who failed and those who survived.
    Intervention: None.
    Outcome measures: Mortality was defined as death, listed for transplant, or transplanted… More >

  • Open Access

    ARTICLE

    Pulsatile Glenn as long‐term palliation for single ventricle physiology patients

    Martin A. Chacon‐Portillo1,2, Rodrigo Zea‐Vera1,2, Huirong Zhu3, Heather A. Dickerson4,5, Iki Adachi1,2, Jeffrey S. Heinle1,2, Charles D. Fraser1,2, Carlos M. Mery1,2

    Congenital Heart Disease, Vol.13, No.6, pp. 927-934, 2018, DOI:10.1111/chd.12664

    Abstract Objective: There are limited studies analyzing pulsatile Glenn as a long‐term pallia‐ tion strategy for single ventricle patients. This study sought to determine their out‐ comes at a single institution.
    Design: A retrospective review was performed.
    Setting: Study performed at a single pediatric hospital.
    Patients: All single ventricle patients who underwent pulsatile Glenn from 1995 to 2016 were included.
    Outcome measures: Pulsatile Glenn failure was defined as takedown, transplant, or death. Further palliation was defined as Fontan, 1.5, or biventricular repair. Risk fac‐ tors were assessed by Cox multivariable competing risk analyses.
    Results: Seventy‐eight patients underwent pulsatile Glenn at age 9… More >

  • Open Access

    ARTICLE

    Incidence and factors influencing the spontaneous closure of Fontan fenestration

    Sudheer R. Gorla1, Nataley K. Jhingoeri1, Abhishek Chakraborty1, Kishore R. Raja1, Ashish Garg1, Satinder Sandhu1, Eliot R. Rosenkranz2, Sethuraman Swaminathan1

    Congenital Heart Disease, Vol.13, No.5, pp. 776-781, 2018, DOI:10.1111/chd.12652

    Abstract Introduction: The Fontan operation is the final stage of single ventricle palliation in patients with complex congenital heart disease. Fenestration in the Fontan conduit, providing an atrial level right to left shunt, has been shown to reduce early postoperative morbidity. However, there is limited data on the long‐term fate of this fenestration. The aim of this study is to define the rate of spontaneous closure of the fenestration in the Fontan conduit and factors predictive of the fate of the fenestration.
    Methods: This was a retrospective study reviewing the medical records of the patients who underwent fenestrated… More >

  • Open Access

    ARTICLE

    Interstage outcomes in single ventricle patients undergoing hybrid stage 1 palliation

    Janet M. Simsic, Christina Phelps, Kristin Kirchner, Kirby‐Rose Carpenito, Robin Allen, Holly Miller‐Tate, Karen Texter, Mark Galantowicz

    Congenital Heart Disease, Vol.13, No.5, pp. 757-763, 2018, DOI:10.1111/chd.12649

    Abstract Objective: Interstage readmissions are common in infants with single ventricle congenital heart disease undergoing staged surgical palliation. We retrospectively examined readmissions during the interstage period.
    Design: Retrospective analysis.
    Setting: The Heart Center at Nationwide Children’s Hospital, Columbus, Ohio.
    Patients: Newborns undergoing hybrid stage 1 palliation from January 2012 to December 2016 who survived to hospital discharge and were followed at our institution.
    Interventions: All patients underwent hybrid stage 1 palliation.
    Outcome Measures: Outcomes included (1) reason for interstage readmission; (2) feeding modality during interstage period; (3) major interstage adverse events; and (4) interstage mortality.
    Results: Study group comprised 57 patients. Five patients only… More >

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