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 >
Open Access
REVIEW
Moira B. Hilscher1, Jonathan N. Johnson2,3, Frank Cetta2,3, David J. Driscoll2,3, 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 >
Open Access
REVIEW
R. Thomas Collins II1,2, Margaret G. Collins3, Michael L. Schmitz2,4, 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 >
Open Access
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 >
Open Access
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 >
Open Access
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 >
Open Access
ARTICLE
Dawn Ilardi1,2, Kim E. Ono1,2, 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 >
Open Access
ARTICLE
Marco Astengo1,2*, Caroline Berntsson3*, Åse A. Johnsson3,4, Peter Eriksson1,2, Mikael Dellborg1,2
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 >
Open Access
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 >
Open Access
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 >
Open Access
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 >
Open Access
ARTICLE
Jacqueline H. Sanz1,2, Madison M. Berl1,2, Anna C. Armour1, Jichuan Wang3,4, Yao I. Cheng3, Mary T. Donofrio5,6
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 >
Open Access
ARTICLE
Sean M. Lang1,2,3, Elijah Bolin1,2,3, Joshua A. Daily1,2,3, Xinyu Tang1,2,3, R. Thomas Collins II1,2,3
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 >
Open Access
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 >
Open Access
ARTICLE
Kari L. Turkowski1, David J. Tester2,3, J. Martijn Bos2,4, Kristina H. Haugaa5, Michael J. Ackerman2,3,4
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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