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  • Open Access

    ARTICLE

    Fate of the Fontan connection: Mechanisms of stenosis and management

    Donald J. Hagler1,2, William R. Miranda2, Brielle J. Haggerty1, Jason H. Anderson1, Jonathan N. Johnson1, Frank Cetta1,2, Sameh M. Said1,3, Nathaniel W. Taggart1

    Congenital Heart Disease, Vol.14, No.4, pp. 571-581, 2019, DOI:10.1111/chd.12757

    Abstract Background: Stenosis of the venous connections and conduits is a well‐known late complication of the Fontan procedure. Currently, data on the outcomes of percuta‐ neous intervention for the treatment of extra‐ or intracardiac conduits and lateral tunnel baffles obstruction are limited. In an attempt to better define the nature and severity of the stenosis and the results of catheter interventional management, we reviewed Fontan patients with obstructed extra‐ or intracardiac conduits and lateral tunnel baffles.
    Methods: Retrospective review of all Fontan patients who had cardiac catheteriza‐ tion from January 2002 to October 2018 was performed. Hemodynamic and… More >

  • Open Access

    ARTICLE

    Two‐year evolution of latent rheumatic heart disease in Malawi

    Amy Sanyahumbi1, Andrea Beaton2, Danielle Guffey3, Mina C. Hosseinipour4, Melissa Karlsten1, Charles G. Minard3, Daniel J. Penny1, Craig A. Sable5, Peter N. Kazembe6

    Congenital Heart Disease, Vol.14, No.4, pp. 614-618, 2019, DOI:10.1111/chd.12756

    Abstract Background: In asymptomatic children, screening echocardiography has been used to attempt to diagnose rheumatic heart disease (RHD) at an early stage (latent RHD). World Heart Federation guidelines have standardized categorization of “definite,” “borderline,” or no RHD by echo findings. The progression of RHD diagnosed through echo screening is not known. In 2014, we screened 1450 schoolchildren in Malawi.
    Objective: Our objective was to evaluate 2‐year RHD evolution among those diag‐ nosed through screening.
    Methods: Two‐year follow‐up echocardiograms of those diagnosed with latent RHD were read by a primary, secondary, then third reader if there was disagreement. Progression or… More >

  • Open Access

    ARTICLE

    Systolic/diastolic ratio correlates with end diastolic pressures in pediatric patients with single right ventricles

    Clifford L. Cua1, Melissa Moore‐Clingenpeel1, Nazia Husain2, Ralf Holzer3, John P. Cheatham1, Janaki Gokhale4

    Congenital Heart Disease, Vol.14, No.4, pp. 609-613, 2019, DOI:10.1111/chd.12755

    Abstract Background: Increased ventricular end‐diastolic pressure (VEDP) is a known risk fac‐ tor for morbidity and mortality in patients with single right ventricle (RV) physiology. Previous studies have shown mixed results correlating echocardiographic measure‐ ments with catheter‐derived VEDP in this population. Goal of this study was to eval‐ uate if echocardiographic systolic/diastolic ratio (S/D) correlated with VEDP.
    Methods: Patients with single RV physiology who underwent simultaneous echocar‐ diography and catheterization were evaluated. Systolic and diastolic durations were measured using tricuspid inflow durations from Doppler analysis to calculate the S/D ratio. VEDP was obtained from the catheterization report.
    Results: Twenty‐seven… More >

  • Open Access

    ARTICLE

    Obesity trends in children, adolescents, and young adults with congenital heart disease

    Jeremy M. Steele1,2, Tamar J. Preminger4, Francine G. Erenberg2, Lu Wang3, Katherine Dell2, Tarek Alsaied1, Kenneth G. Zahka2

    Congenital Heart Disease, Vol.14, No.4, pp. 517-524, 2019, DOI:10.1111/chd.12754

    Abstract Objectives: To determine the prevalence, age of onset, and risk factors for overweight and obesity in children with congenital heart disease (CHD).
    Study Design: Children with CHD who were seen at our institution from 1996 to 2017 were studied. Patients were full-time residents of the United States and were receiving all cardiac care at our institution. Patients were categorized by age and CHD diagnosis. The date of last normal weight for age and the date of first recorded weight in the range of overweight and obese were documented.
    Results: Nine hundred sixty-eight patients with CHD were included. The More >

  • Open Access

    EDITORIAL

    A fond farewell

    Douglas S. Moodie

    Congenital Heart Disease, Vol.14, No.6, pp. 884-884, 2019, DOI:10.1111/chd.12865

    Abstract This article has no abstract. More >

  • Open Access

    ARTICLE

    The incidence of arrhythmias during exercise stress tests among children with Kawasaki disease: A single-center case series

    Varun Aggarwal1,2, Kristen Sexson-Tejtal1, Wilson Lam1, Santiago O. Valdes1, Caridad M. de la Uz1, Jeffrey J. Kim1, Christina Y. Miyake1

    Congenital Heart Disease, Vol.14, No.6, pp. 1032-1036, 2019, DOI:10.1111/chd.12864

    Abstract Objective: Based on 2017 guidelines, participation in competitive sports with prior history of Kawasaki Disease (KD) requires those with coronary artery aneurysms (CAA) z score ≥ 5 to undergo evaluation for evidence of inducible ischemia or arrhythmias. The use of exercise stress testing (EST) to evaluate arrhythmias among KD patients has never been reported. This retrospective single-center case series study sought to describe the presence of inducible arrhythmias during EST in KD patients with or without CAA.
    Methods: Single-center retrospective review of medical records of patients diagnosed with KD between 1989-2015 at Texas Children’s Hospital, Houston, Texas… More >

  • Open Access

    ARTICLE

    Infundibular sparing versus transinfundibular approach to the repair of tetralogy of Fallot

    Mary K. Olive1, Charles D. Fraser2, Shelby Kutty3, Emmett D. McKenzie4, James M. Hammel5, Rajesh Krishnamurthy6, Nicolas A. Dodd7, Shiraz A. Maskatia8

    Congenital Heart Disease, Vol.14, No.6, pp. 1149-1156, 2019, DOI:10.1111/chd.12863

    Abstract Introduction: The right ventricular infundibular sparing approach (RVIS) to the repair of tetralogy of Fallot (TOF) avoids a full-thickness ventricular incision, typically utilized in the transinfundibular (TI) method.
    Methods: We performed a retrospective, age-matched cohort study of patients who underwent RVIS at Texas Children’s Hospital or TI at Children’s Hospital Medical Center in Nebraska and subsequently underwent cardiac magnetic resonance imaging (CMR). We compared right ventricular end-diastolic and systolic volumes indexed to body surface area (RVEDVi and RVESVi) and right ventricular ejection fraction (RVEF) as primary endpoints. Secondary endpoints were indexed left ventricular diastolic and systolic volume… More >

  • Open Access

    ARTICLE

    Baseline tubular biomarkers in young adults with congenital heart disease as compared to healthy young adults: Detecting subclinical kidney injury

    Dana Y. Fuhrman1,2, Lan Nguyen3, Morgan Hindes3, John A. Kellum2

    Congenital Heart Disease, Vol.14, No.6, pp. 963-967, 2019, DOI:10.1111/chd.12862

    Abstract Background: There are significant implications for kidney disease in young adults with congenital heart disease. Prior investigations have not focused on the use of urinary tubular biomarkers for the early identification of kidney disease in this growing patient group.
    Objective: Determine if young adults with congenital heart disease have differences in the baseline concentration of urinary tubular biomarkers when compared to healthy young adults.
    Design/Methods: In a pilot case control study, 30 patients from 18 to 35 years of age with congenital heart disease and a normal serum creatinine were recruited during a routine follow-up visit. In the… More >

  • Open Access

    ARTICLE

    Echocardiography vs cardiac magnetic resonance imaging assessment of the systemic right ventricle for patients with d-transposition of the great arteries status post atrial switch

    Margaret M. Samyn1,2, Ke Yan1, Conor Masterson3, Benjamin H. Goot1,2, David Saudek1,2, Julie Lavoie2, Aaron Kinney2, Mary Krolikowski1, Kan Hor4,5, Scott Cohen1,2

    Congenital Heart Disease, Vol.14, No.6, pp. 1138-1148, 2019, DOI:10.1111/chd.12861

    Abstract Objective: Patients with Dextro-transposition of the great arteries status post atrial switch (dTGA s/p atrial switch) are “at-risk” for systemic right ventricular (RV) dysfunction. Due to complex RV geometry, echocardiography (Echo) does not allow accurate determination of ejection fraction (EF), but cardiac magnetic resonance imaging (CMR) allows quantitative right ventricular assessment. Measures of ventricular deformation may be precursors to global ventricular dysfunction. The primary aim of this study was to characterize imaging and clinical findings for adult patients with dTGA s/p atrial switch.
    Design: This was a retrospective cohort study of patients with dTGA s/p atrial switch… More >

  • Open Access

    ARTICLE

    Pulmonary artery pulsatility index predicts prolonged inotrope/ pulmonary vasodilator use after implantation of continuous flow left ventricular assist device

    Varun Aggarwal1,2, Sebastian C. Tume3, Marco Rodriguez1, Iki Adachi4, Antonio G. Cabrera1, Hari Tunuguntla1, Athar M. Qureshi1

    Congenital Heart Disease, Vol.14, No.6, pp. 1130-1137, 2019, DOI:10.1111/chd.12860

    Abstract Objective: Predictors of right ventricle (RV) dysfunction after continuous‐flow left ventricular assist device (CF‐LVAD) implantation in children are not well described. We explored the association of preimplantation Pulmonary Artery Pulsatility index (PAPi) and other hemodynamic parameters as predictors of prolonged postoperative inotropes/pulmonary vasodilator use after CF‐LVAD implantation.
    Design: Retrospective chart review.
    Setting: Single tertiary care pediatric referral center.
    Patients: Patients who underwent CF‐LVAD implantation from January 2012 to October 2017.
    Interventions: Preimplantation invasive hemodynamic parameters were analyzed to evaluate the association with post‐CF‐LVAD need for prolonged (>72 hours) use of inotropes/pulmonary vasodilators.
    Measurements and main results: Preimplantation cardiac catheterization data was… More >

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