Open Access

EDITORIAL

Douglas S. Moodie,
Congenital Heart Disease, Vol.12, No.2, pp. 123-123, 2017, DOI:10.1111/chd.12464
Abstract This article has no abstract. More >

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REVIEW

Moira B. Hilscher¹, Jonathan N. Johnson^2,3, Frank Cetta^2,3, David J. Driscoll^2,3, John J. Poterucha¹, William Sanchez¹, Heidi M. Connolly³, Patrick S. Kamath¹
Congenital Heart Disease, Vol.12, No.2, pp. 124-132, 2017, DOI:10.1111/chd.12446
Abstract The physiological consequences of the Fontan circulation impose risk for hepatic dysfunction and may culminate in hepatic fibrosis, cirrhosis, and hepatocellular carcinoma. Consensus regarding appropriate surveillance modalities to diagnose liver disease in Fontan patients is lacking, in part due to the relative lack of strong evidence and prospective studies in this patient population. The goal of this paper is to critically review the current evidence and provide recommendations for the surveillance of hepatic complications in the post-Fontan patient population. More >

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1094

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REVIEW

R. Thomas Collins II^1,2, Margaret G. Collins³, Michael L. Schmitz^2,4, Justin T. Hamrick⁴
Congenital Heart Disease, Vol.12, No.2, pp. 133-142, 2017, DOI:10.1111/chd.12447
Abstract Williams syndrome (WS) is a congenital, multisystem disorder affecting the cardiovascular, connective tissue, and central nervous systems in 1 in 10 000 live births. Cardiovascular involvement is the most common cause of morbidity and mortality in patients with WS, and noninvasive and invasive procedures are common. Sudden cardiovascular collapse in patients with WS is a well-known phenomenon, especially in the peri-procedural period. Detailed guidelines for peri-procedural management of patients with WS are limited. The goal of this review is to provide thoughtful, safe and effective management strategies for the peri-procedural care of patients with WS with careful consideration of hemodynamic… More >

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787

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ARTICLE

Anushree Agarwal, Fatima Samad, Lindsey Kalvin, Michelle Bush, A. Jamil Tajik
Congenital Heart Disease, Vol.12, No.2, pp. 143-152, 2017, DOI:10.1111/chd.12453
Abstract Introduction: Congenitally corrected transposition of the great arteries (ccTGA) is a rare congenital disease that frequently remains undiagnosed until adulthood, especially when there is an absence of other congenital anomalies. Adults with ccTGA may remain asymptomatic and their diagnosis could be missed on initial evaluation, or it could be diagnosed incidentally as an evaluation of murmur. We aim to report the different presentations of ccTGA in eight adult patients and review the key features required to suspect the diagnosis during an initial visit.
Cases: We present some illustrative cases of ccTGA patients who had diverse presentations ranging from being completely… More >

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748

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ARTICLE

Matthew J. Lewis¹, Elizabeth Hecht², Jonathan Ginns¹, Joshua Benton², Martin Prince², Marlon S. Rosenbaum¹
Congenital Heart Disease, Vol.12, No.2, pp. 153-158, 2017, DOI:10.1111/chd.12422
Abstract Background: The progression of hepatic disease in adult Fontan patients is not well understood. They reviewed the experience with serial cardiac MRIs (CMR) in adult Fontan patients to determine if hepatic anatomic markers of prolonged Fontan exposure were present and if clinical predictors of progressive hepatic congestion could be identified.
Methods and Results: A retrospective cohort study of all adult Fontan patients who had undergone at least two CMRs was performed. Hepatic dimensions, inferior vena cava (IVC) size, right hepatic vein (RHV) size and spleen diameter were determined from images acquired at the time of clinically guided CMR. Two radiologists… More >

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782

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ARTICLE

Yuli Y. Kim¹, Wei He², Thomas E. MacGillivray³, Oscar J. Benavidez²
Congenital Heart Disease, Vol.12, No.2, pp. 159-165, 2017, DOI:10.1111/chd.12433
Abstract Objective: Despite their clinical importance, 30-day readmission after adult congenital heart surgery has been understudied. They sought to determine the frequency of unplanned readmissions after adult congenital heart surgery and to identify any potential associated risk factors.
Design: Retrospective cohort study using State Inpatient Databases for Washington, New York, Florida, and California from 2009 to 2011.
Setting: Federal and nonfederal acute care hospitals.
Patients: Admissions of patients age 18–49 years with International Classification of Diseases, Ninth Revision, Clinical Modification codes indicating adult congenital heart surgery.
Outcome Measures: Readmission was defined as any nonelective hospitalization for a given patient ≤30 days… More >

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804

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ARTICLE

Dawn Ilardi^1,2, Kim E. Ono^1,2, Rebecca McCartney³, Wendy Book⁴, Anthony Y. Stringer²
Congenital Heart Disease, Vol.12, No.2, pp. 166-173, 2017, DOI:10.1111/chd.12434
Abstract Objective: Adults with congenital heart disease (CHD) are at increased risk of psychological disorders and cognitive deficiencies due to structural/acquired neurological abnormalities and neurodevelopmental disorders as children. However, limited information is known about the neuropsychological functioning of adults with CHD. This study screened neuropsychological abilities and explored group differences related to cardiac disease severity and neurological risk factors in adults with CHD.
Design: Participants completed brief neuropsychological testing. Information about neurobehavioral and psychological symptoms, employment, education, and disability were also collected from the patient and a family member.
Results: Forty-eight participants with adult CHD completed neuropsychological testing. Visuospatial skills and… More >

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715

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ARTICLE

Marco Astengo^1,2*, Caroline Berntsson^3*, Åse A. Johnsson^3,4, Peter Eriksson^1,2, Mikael Dellborg^1,2
Congenital Heart Disease, Vol.12, No.2, pp. 174-180, 2017, DOI:10.1111/chd.12424
Abstract Objective: Coarctation of the aorta (CoA) is a common condition. Adult patients with newly diagnosed CoA and patients with recurring or residual CoA require evaluation of the severity of aortic obstruction. Cardiac catheterization is considered the gold standard for the evaluation of hemodynamically significant CoA. The European Society of Cardiology (ESC) Guidelines for the management of grown-up congenital heart disease (GUCH) include noninvasive criteria for identifying significant CoA. Our aim was to investigate the ability of the Class I and Class IIa ESC recommendations to identify significant CoA at cardiac catheterization.
Design: Sixty-six adult patients with native or recurrent CoA… More >

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1058

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885

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ARTICLE

Shyam Sathanandam¹, Pooja Kashyap¹, David Zurakowski², Lindsey Bird¹, Vera McGhee³, Jeffrey Towbin¹, Benjamin Rush Waller III¹
Congenital Heart Disease, Vol.12, No.2, pp. 181-187, 2017, DOI:10.1111/chd.12423
Abstract Objectives: The primary aim of this study was to compare thermodilution (TD) cardiac index (TDCi) measured by injecting cold saline (C-TDCi) to saline at room temperature (R-TDCi). The secondary aim was to assess the change in body temperature with cold saline injections in children.
Design: This is a prospective, case control study.
Setting: Cardiac catheterization lab at Le Bonheur Children’s Hospital, Memphis, Tennessee.
Patients: Eighty-six children ≤18 years of age that underwent cardiac catheterization between April 2013 and April 2015, excluding patients with admixing lesions, on inotropic support and with ejection fraction < 30%.
Interventions: A TD catheter in the… More >

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852

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ARTICLE

Safwat A. Aly¹, David Zurakowski², Penny Glass³, Kami Skurow-Todd⁴, Richard A. Jonas⁵, Mary T. Donofrio⁴
Congenital Heart Disease, Vol.12, No.2, pp. 188-195, 2017, DOI:10.1111/chd.12426
Abstract Importance: There are no well-established noninvasive biomarkers for identifying patients at risk for poor outcome after surgery for congenital heart disease. Few studies have assessed prognostic accuracy of cerebral tissue oxygenation index (cTOI) measured by near infrared spectroscopy (NIRS).
Objective: To assess the utility of noninvasive NIRS monitoring as a predictor of outcomes after neonatal cardiac surgery through measurement of cTOI. To examine the utility of noninvasive NIRS monitoring in combination with lactate concentration and inotropic score in prediction of outcomes after neonatal cardiac surgery.
Design: Prospective longitudinal cohort study.
Setting: Operating room and cardiac intensive care unit, Children’s National… More >

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772

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ARTICLE

Shipra Gupta¹, Ankit Sakhuja², Eric McGrath¹, Basim Asmar¹
Congenital Heart Disease, Vol.12, No.2, pp. 196-201, 2017, DOI:10.1111/chd.12425
Abstract Background: We studied the incidence, trend, underlying conditions, microbiology, and outcomes of infective endocarditis (IE) in children during 11 years using Nationwide Inpatient Sample (NIS) database. This is the largest all-payer inpatient care database in the United States containing data for more than 8 million hospital stays from over 1000 hospitals.
Methods: NIS data from 2000 to 2010 of primary discharge diagnosis of IE in children aged ≤19 years old were studied. Children with underlying congenital heart defects and acquired heart conditions were identified. Microbiological causative agents were recorded. Linear regression was used to assess trend of incidence over time.
… More >

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844

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ARTICLE

Jacqueline H. Sanz^1,2, Madison M. Berl^1,2, Anna C. Armour¹, Jichuan Wang^3,4, Yao I. Cheng³, Mary T. Donofrio^5,6
Congenital Heart Disease, Vol.12, No.2, pp. 202-209, 2017, DOI:10.1111/chd.12427
Abstract Objective: Executive function, a set of cognitive skills important to social and academic outcomes, is a specific area of cognitive weakness in children with congenital heart disease (CHD). We evaluated the prevalence and profile of executive dysfunction in a heterogeneous sample of school aged children with CHD, examined whether children with executive dysfunction are receiving school services and support, and identified risk factors for executive dysfunction at school age.
Design: Ninety-one school aged patients completed questionnaires, including the Behavior Rating Inventory of Executive Function (BRIEF) and a medical history questionnaire. An age- and gendermatched control sample was drawn from a… More >

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858

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ARTICLE

Sean M. Lang^1,2,3, Elijah Bolin^1,2,3, Joshua A. Daily^1,2,3, Xinyu Tang^1,2,3, R. Thomas Collins II^1,2,3
Congenital Heart Disease, Vol.12, No.2, pp. 210-217, 2017, DOI:10.1111/chd.12428
Abstract Objective: Multiple reports have shown echocardiograms for certain indications are neither costeffective nor of high diagnostic yield. Given the ease with which tests can be obtained at a tertiary academic children’s hospital, our aims were to: (1) determine the diagnostic yield of inpatient studies by in-hospital location; (2) evaluate inpatient echocardiograms to determine indications and level of appropriateness; and (3) evaluate the frequency of cardiology involvement prior to those echocardiograms.
Design: All initial inpatient echocardiograms interpreted at our institution from February 2009 to December 2014 were reviewed retrospectively. Patient location was grouped as pediatric intensive care (PICU), emergency department (ED),… More >

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857

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ARTICLE

Haki Jashari¹, Katarina Lannering², Mats Mellander², Pranvera Ibrahimi¹, Annika Rydberg³, Michael Y. Henein¹
Congenital Heart Disease, Vol.12, No.2, pp. 218-225, 2017, DOI:10.1111/chd.12430
Abstract Background and aims: Patients with coarctation of the aorta (CoA) have increased left ventricular (LV) afterload that has been shown to impact the LV and ascending aortic function. We aimed to examine the effect of coarctation on LV function and aorto-septal angle (AoSA) before and after surgical repair.
Methods: We retrospectively studied 21 patients with surgically repaired CoA at a median age of 9 (2-53) days at three time points: (1) just before intervention, (2) at short-term follow-up, and (3) at medium-term follow-up after intervention. AoSA was measured from the parasternal long axis view, at three time points during the… More >

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782

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ARTICLE

Kari L. Turkowski¹, David J. Tester^2,3, J. Martijn Bos^2,4, Kristina H. Haugaa⁵, Michael J. Ackerman^2,3,4
Congenital Heart Disease, Vol.12, No.2, pp. 226-235, 2017, DOI:10.1111/chd.12462
Abstract Background: Arrhythmogenic cardiomyopathy (ACM) is a heritable disease characterized by fibrofatty replacement of cardiomyocytes, has a prevalence of approximately 1 in 5000 individuals, and accounts for approximately 20% of sudden cardiac death in the young (≤35 years). ACM is most often inherited as an autosomal dominant trait with incomplete penetrance and variable expression. While mutations in several genes that encode key desmosomal proteins underlie about half of all ACM, the remainder is elusive genetically. Objective: Here, whole exome sequencing (WES) was performed with genomic triangulation in an effort to identify a novel explanation for a phenotype-positive, genotype-negative multigenerational pedigree with… More >

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846