Open Access

EDITORIAL

Douglas S. Moodie,
Congenital Heart Disease, Vol.13, No.4, pp. 491-491, 2018, DOI:10.1111/chd.12656
Abstract This article has no abstract. More >

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718

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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 describe the approach… More >

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796

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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; have a… More >

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880

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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 measured in… More >

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825

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Natasha L. Lopez, Charitha Gowda, Carl H. Backes, Deipanjan Nandi, Holly Miller-Tate, Samantha Fichtner, Robin Allen, Jamie Stewart, Clifford L. Cua
Congenital Heart Disease, Vol.13, No.4, pp. 512-518, 2018, DOI:10.1111/chd.12602
Abstract Introduction: Neonates with hypoplastic left heart syndrome (HLHS) are at increased risk for necrotizing enterocolitis (NEC). Initial hospital outcomes are well described, but minimal midterm data exist. Goal of this study was to compare outcomes of HLHS infants with NEC (HLHS-NEC) to HLHS without NEC (HLHS-nNEC) during the interstage period.
Methods: Data were reviewed from 55 centers using the NPC-QIC database. Case-control study with one HLHS-NEC matched to HLHS-nNEC neonates in a 1:3 ratio based on institutional site, type of surgical repair, and gestational age ±1 week was performed. Baseline demographics as well as outcome data were recorded. The t… More >

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827

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Parthak Prodhan¹, Xinyu Tang², Jeffrey Gossett², Brandon Beam³, Janet Simsic⁴, Nancy Ghanayem⁵, Nahed O. ElHassan⁶
Congenital Heart Disease, Vol.13, No.4, pp. 519-527, 2018, DOI:10.1111/chd.12610
Abstract Objective: Different feeding strategies have been suggested to improve growth and survival of infants with hypoplastic left heart syndrome following stage 1 palliation. The study objective was to assess hospital mortality following stage 1 palliation among infants with hypoplastic left heart syndrome who had two feeding modalities, gastrostomy tube vs no gastrostomy tube.
Design: Retrospective study design.
Setting: Multicenter pediatric heath information system database.
Patient: About 4287 patients with hypoplastic left heart syndrome who underwent stage 1 Norwood procedure from 2004 through 2013. Infants who had gastrostomy tube with or without fundoplication procedure were identified and their clinical characteristics were… More >

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754

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Elizabeth C. Caris¹, Nicole Dempster², Gil Wernovsky³, Yongjie Miao², Melissa Moore-Clingenpeel², Trent Neely², Rachel Fonseca², Holly Miller-Tate², Robin Allen², Samantha Fichtner², Jamie Stewart², Clifford L. Cua²
Congenital Heart Disease, Vol.13, No.4, pp. 528-532, 2018, DOI:10.1111/chd.12619
Abstract Objectives: Siblings of children with chronic medical conditions endorse a lower quality of life compared to age-matched peers. Caregiver and sibling-self report of adjustment are often discordant. Congenital heart disease significantly affects family life. To date, there have been no studies addressing the functioning of siblings of children with hypoplastic left heart syndrome, one of the most severe forms of congenital heart disease. The goal of this study was to assess the impact of hypoplastic left heart syndrome on sibling’s quality of life as well as the caregiver’s perception of this effect.
Study Design: Cross-sectional study using a web-based survey… More >

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723

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Doris P. Yimgang¹, John D. Sorkin², Charles F. Evans³, Danielle S. Abraham¹, Geoffrey L. Rosenthal^1,4
Congenital Heart Disease, Vol.13, No.4, pp. 533-540, 2018, DOI:10.1111/chd.12622
Abstract Introduction: Angiotensin converting enzyme inhibitors are commonly prescribed medications after the Norwood procedure. There are little data that can be used to determine if angiotensin converting enzyme inhibitors improve interstage outcomes in children with single ventricle defects. The objective of this study was to investigate the relationship between angiotensin converting enzyme inhibitors and interstage failure among infants born with hypoplastic left heart syndrome.
Methods: We conducted a retrospective cohort study using data from the National Pediatric Cardiology Quality Improvement Collaborative database (collected between 2008 and 2015). We used logistic regression models to assess the exposure-outcome associations and propensity score matching… More >

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742

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Justin Georgekutty¹, Amir Kazerouninia², YunFei Wang³, Peter R. Ermis⁴, Dhaval R. Parekh⁴, Wayne J. Franklin⁴, Wilson W. Lam⁴
Congenital Heart Disease, Vol.13, No.4, pp. 541-547, 2018, DOI:10.1111/chd.12603
Abstract Objective: Adult Fontan patients are at increased risk for thrombosis and thromboembolic complications leading to increased morbidity and mortality. Most are prescribed antiplatelet or anticoagulant therapy for thromboprophylaxis; novel oral anticoagulants (NOACs) are uncommonly used given lack of data on their use in this population and generalized concerns regarding Fontan patients’ abnormal coagulation. We report the largest single-center experience with the use of NOACs for treatment and prophylaxis of thrombosis and thromboembolism in adult Fontan patients.
Results: A retrospective chart review identified 21 patients (11 female, 10 male), median age 33 years (18-50) at first initiation, who were prescribed a… More >

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864

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Albert C. Hergenroeder¹, Douglas S. Moodie², Daniel J. Penny², Constance M. Wiemann¹, Blanca Sanchez-Fournier¹, Lauren K. Moore², Jane Head³
Congenital Heart Disease, Vol.13, No.4, pp. 548-553, 2018, DOI:10.1111/chd.12604
Abstract Objective: To describe changes in functional status between the last pediatric and first adult congenital heart disease (CHD) clinic visits in patients with moderate to severe CHD after implementing a healthcare transition (HCT) planning program.
Design: Quasi-experimental design. Patients were followed prospectively following the implementation of the intervention; Control patients transitioned from the Pediatric CHD Clinic into Adult CHD Clinic before the intervention.
Setting: Texas Children’s Hospital (TCH).
Patients: Sixteen to 25-year-olds, cognitively normal, English speaking patients with moderate to severe CHD who transitioned from the Pediatric to the Adult CHD clinic.
Interventions: An EMR-based transition planning tool (TPT) was… More >

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856

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Nancy McClung^1,2, Jill Glidewell¹, Sherry L. Farr¹
Congenital Heart Disease, Vol.13, No.4, pp. 554-562, 2018, DOI:10.1111/chd.12605
Abstract Objective: To examine the financial burdens and mental health needs of families of children with special healthcare needs (CSHCN) with congenital heart disease (CHD).
Methods: Data from the 2009–2010 National Survey of Children with Special Health Care Needs (NS-CSHCN) were used to examine parent-reported financial burdens (out-of-pocket expenses, financial problems, employment impact, caregiving hours) and family members’ need for mental health services in families of CSHCN with CHD. Multivariable logistic regression was used to compare financial burdens and family members’ need for mental health services among CSHCN with and without CHD. Among CSHCN with CHD, multivariable logistic regression, stratified by… More >

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782

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Kevin C. Harris¹, Christine Voss¹, Kathryn Rankin², Basmina Aminzadah², Ross Gardner¹, Andrew S. Mackie²
Congenital Heart Disease, Vol.13, No.4, pp. 563-570, 2018, DOI:10.1111/chd.12612
Abstract Objective: Individuals with congenital heart disease (CHD) may be at higher risk of acquired cardiovascular disease than the general population due to their underlying physiology and/or surgical sequelae. We sought to assess the prevalence of cardiovascular disease risk factors in youth and adults with CHD.
Methods: We assessed cardiovascular health as per the Cardiovascular Health in Ambulatory Care Research Team (CANHEART) health index in patients with CHD aged 15+ years who attended cardiology outpatient clinics. Participants self-reported smoking behavior, fruit and vegetable consumption, physical activity, and whether they had diabetes and hypertension. Individual health indices were categorized into ideal/not ideal,… More >

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795

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Michele M. Pasierb, Josiah M. Penalver, Margaret M. Vernon, Bhawna Arya
Congenital Heart Disease, Vol.13, No.4, pp. 571-577, 2018, DOI:10.1111/chd.12611
Abstract Background: Accurate prenatal diagnosis of congenital heart disease (CHD) allows for appropriate delivery and postnatal management. Geographic constraints limit access to fetal cardiology subspecialists. In our approach, general pediatric cardiologists are first line in regional prenatal cardiac screening. We aim to demonstrate the utility of this approach in diagnosing CHD requiring cardiac interventions within 30 days of life.
Methods: This is a retrospective review of fetal echocardiograms performed at Seattle Children’s Hospital regional cardiology sites (SCH-RC) from December 2008 to December 2015. Referrals to Seattle Children’s Hospital Prenatal Program (SCH-PNP) were evaluated for referral timing, indication, diagnostic accuracy, and postnatal… More >

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767

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Natasha G. Boyes¹, Michael K. Stickland², Stephanie Fusnik¹, Elizabeth Hogeweide¹, Josie T.J. Fries¹, Mark J. Haykowsky³, Chantelle L. Baril¹, Shonah Runalls¹, Ashok Kakadekar⁴, Scott Pharis⁴, Charissa Pockett⁴, Timothy J. Bradley⁴, Kristi D. Wright⁵, Marta Erlandson¹, Corey R. Tomczak¹
Congenital Heart Disease, Vol.13, No.4, pp. 578-583, 2018, DOI:10.1111/chd.12614
Abstract Children with congenital heart disease are at risk for developing increased arterial stiffness and this may be modulated by physical activity. Objective: To compare arterial stiffness in high- and low-physically active children with congenital heart disease and healthy age- and sex-matched controls.
Patients: Seventeen children with congenital heart disease (12 ± 2 years; females = 9), grouped by low- and high-physical activity levels from accelerometry step count values, and 20 matched controls (11 ± 3 years; females = 9) were studied.
Outcome Measures: Carotid-radial pulse wave velocity was assessed with applanation tonometry to determine arterial stiffness. Body composition and 6-min… More >

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769

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Hieu Lan Nguyen^1,2, Quang Tan Phan^3,4, Linh Huynh Dinh², Hieu Ba Tran², Hoyoun Won⁴, Julian Johny Thottian^4,5, Dung Doan Duc¹, Truong Nguyen Quang³, Sang Wook Kim⁴
Congenital Heart Disease, Vol.13, No.4, pp. 584-593, 2018, DOI:10.1111/chd.12613
Abstract Objective: To evaluate the efficacy and safety of Nit-Occlud Le VSD Coil versus Duct Occluders ^ for percutaneous closure of perimembranous ventricular septal defect (pmVSD).
Introduction: VSD closure using conventional pmVSD occluders has been largely abandoned because of an unacceptable high rate of complete heart block (CHB). The advantages of Duct Occluders and VSD Coil are supposed to reduce the drawbacks of previous devices, especially CHB complications.
Method: Patients underwent percutaneous pmVSD closure were divided into Coil group (using VSD Coil, n = 71) and DO group (using Duct Occluders, n = 315). Patient demographics, clinical presentations, echocardiography measurements, procedure… More >

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889

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Martin Christmann^1,2, Alexandra Wipf^1,2, Hitendu Dave^2,3, Daniel Quandt^1,2, Oliver Niesse^1,2, Markus Deisenberg^2,4, Martin Hersberger^2,5, Oliver Kretschmar^1,2, Walter Knirsch^1,2
Congenital Heart Disease, Vol.13, No.4, pp. 594-601, 2018, DOI:10.1111/chd.12615
Abstract Objective: To find risk factors for a complicated early postoperative course after arterial switch operation (ASO) in neonates with d-transposition of the great arteries (dTGA). In addition to anatomical and surgical parameters, the predictive value of early postoperative troponin T (TnT) values in correlation to the early postoperative course after ASO is analyzed.
Methods: Seventy-nine neonates (57 (72%) male) with simple dTGA treated by ASO between 2009 and 2016 were included in the analysis. A complicated early postoperative course (30 days) was defined by one of the following criteria: (A) moderate to severe cardiac dysfunction without rhythm disturbances, (B) rhythm… More >

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776

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Christopher R. Broda¹, Hamsini Sriraman², Devanshi Wadhwa², YunFei Wang¹, Hari Tunuguntla¹, Ayse Akcan-Arikan^3,4, Peter R. Ermis¹, Jack F. Price¹
Congenital Heart Disease, Vol.13, No.4, pp. 602-607, 2018, DOI:10.1111/chd.12617
Abstract Purpose: Elevated central venous pressure (CVP) has deleterious effects on several organ systems in patients with Fontan circulation. However, the relationship between CVP and estimated glomerular filtration rate (eGFR) has not been assessed in patients with Fontan circulation.
Methods: Patients with Fontan circulation whose hemodynamics were assessed by catheterization between 1987 and 2015 and had a serum creatinine measured within 72 hours prior to the procedure were included for analysis. Patients with primary kidney disease were excluded. Renal function was calculated by “bedside Schwartz” equation in children (< 18 years) and Modification of Diet in Renal Disease equation in adults.… More >

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757

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Mi Kyoung Song¹, Gi Beom Kim¹, Eun Jung Bae¹, Young Ah Lee¹, Hyun-Young Kim², Seung-Kee Min³, Jung Hee Kim⁴, Jae-Kyung Won⁵
Congenital Heart Disease, Vol.13, No.4, pp. 608-616, 2018, DOI:10.1111/chd.12625
Abstract Objective: Pheochromocytoma and paraganglioma (extra-adrenal pheochromocytoma) are rare neuroendocrine tumors that arise from the neuroendocrine cells. Chronic hypoxia is known as a possible cause, and a strong link between cyanotic congenital heart disease and these tumors has been reported. However, reports of phechromocytoma/paraganglioma in Fontan patients were scarce. We herein report seven cases of phechromocytoma/paraganglioma after Fontan operation at a single tertiary center.
Methods: We retrospectively reviewed medical records and imaging studies who diagnosed as phechromocytoma/paraganglioma after Fontan operation in Seoul National University Children’s Hospital.
Results: Seven patients were identified during follow-up after Fontan operation, and the prevalence was 2.5%… More >

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847

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Alex J. Thompson¹, Joseph A. Dearani², Jonathan N. Johnson^1,3, Hartzell V. Schaff², Eric C. Towe¹, Jared Palfreeman¹, Philip L. Wackel¹, Frank Cetta^1,3
Congenital Heart Disease, Vol.13, No.4, pp. 617-623, 2018, DOI:10.1111/chd.12618
Abstract Background: The transapical approach has been utilized in adult HCM patients with either midventricular obstruction or a small LV cavity; however, there are little data on its use in children.
Methods: We retrospectively reviewed all patients (age <21 years) with HCM who underwent a transapical myectomy from January 2002 to December 2016. Indication for surgery was midventricular obstruction in 19/23 (83%) and small LV cavity in 4 (17%). Preoperative symptoms included: dyspnea (96%), chest pain (65%), presyncope (61%), and syncope (35%). The mean age at the time of operation was 14 ± 4.0 years (range, 4–20).
Results: Overall, 23 patients… More >

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847

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William N. Evans^1,2, Ruben J. Acherman^1,2, Dean Berthoty³, Gary A. Mayman^1,2, Michael L. Ciccolo^1,4, Sergio A. Carrillo^1,4, Humberto Restrepo^1,2
Congenital Heart Disease, Vol.13, No.4, pp. 624-627, 2018, DOI:10.1111/chd.12623
Abstract Patients: We reviewed all patients evaluated at our center with situs solitus, levocardia, and a right aortic arch that were born between January 2000 and January 2018. Results: From our databases, we identified 204 patients. We excluded patients with a double aortic arch from analysis. Of the 204 patients, 103 (50%) were male. Of the 204 patients, 95 (47%) had an isolated right aortic arch. Of the 95 with an isolated right aortic arch, 4 (4%) had chromosome 22q11.2 deletion syndrome, and 89 (94%) had a vascular ring. Of the 204 patients, 109 (53%) had a surgical intracardiac malformation. Of… More >

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766

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Margaret M. Fuchs¹, Heidi M. Connolly¹, Sameh M. Said², Alexander C. Egbe¹
Congenital Heart Disease, Vol.13, No.4, pp. 628-632, 2018, DOI:10.1111/chd.12624
Abstract Objective: To describe outcomes in patients with cor triatriatum sinister (CTS).
Design: Retrospective review of patients with CTS followed at Mayo Clinic Rochester from 1990 to 2016. Clinical notes, operative reports, and baseline imaging studies were reviewed including echocardiogram, magnetic resonance imaging, computed tomography, and cardiac catheterization.
Results: Fifty-seven patients (median age 34 years; men 32 (56%)) were enrolled. Definitive or suspected CTS diagnosis was made by transthoracic echocardiogram in 41 (72%) patients, and additional multimodality imaging was required in 39 (68%) patients. Of these 57 patients, initial diagnosis was made in adulthood in 35 (61%) patients, and 33 of… More >

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727

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Sherry L. Farr¹, Karrie F. Downing^1,2, Tiffany Riehle-Colarusso¹, Ginnie Abarbanell^1,3
Congenital Heart Disease, Vol.13, No.4, pp. 633-639, 2018, DOI:10.1111/chd.12621
Abstract Objective: To examine how cognitive and motor limitations in children with heart disease are associated with education and participation in extracurricular activities.
Design: Using 2009–2010 parent-reported data from the National Survey of Children with Special Health Care Needs (NS-CSHCN), we examined prevalence of five functional limitations (learning/ concentration, communication, self-care, gross motor skills, and fine motor skills) by diagnosed heart disease status using chi-square tests and multivariable logistic regression. Among CSHCN with heart disease, we examined the associations between severity of each functional limitation and missing ≥11 days of school in the past year, receiving special education services, and interference… More >

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