Open Access

EDITORIAL

James E. Lock^1,2,3
Congenital Heart Disease, Vol.12, No.6, pp. 697-698, 2017, DOI:10.1111/chd.12548
Abstract This article has no abstract. More >

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REVIEW

Gruschen R. Veldtman¹, Alexander R. Opotowsky², Samuel G. Wittekind¹, Jack Rychik³, Daniel J. Penny⁴, Mark Fogel³, Bradley S. Marino⁵, Marc Gewillig⁶
Congenital Heart Disease, Vol.12, No.6, pp. 699-710, 2017, DOI:10.1111/chd.12526
Abstract Although medium-term survival following Fontan operations in the modern era has improved dramatically, late cardiovascular and extracardiac morbidity are common and are associated with impaired quality of life and premature late mortality. This serves as a reminder of the extraordinary adaptations required of the cardiovascular system when the systemic arterial, systemic venous and pulmonary circulations are placed in series coupled to a single ventricular pump. This article reviews the key features and principles that govern interactions between the ventricle, systemic arterial circulation, the systemic venous and pulmonary circulatory compartments, the microcirculation, and lymphatic circulations. The More >

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ARTICLE

Zhiyong Lin, Hanwei Ge, Jiyang Xue, Guowei Wu, Jie Du, Xingti Hu, Qifeng Zhao
Congenital Heart Disease, Vol.12, No.6, pp. 711-720, 2017, DOI:10.1111/chd.12503
Abstract Objective: This study aims to assess and compare the early and long-term effects of extracardiac conduit (EC) and lateral tunnel (LT) in patients with a functional single ventricle through metaanalysis.
Design: A systematic search was performed in PubMed, Embase, Cochrane Library, CNKI, VIP, CBM, and WanFang databases for papers that were published until August 1, 2016. Cochrane systematic review method was used for paper screening and information retrieve, and RevMan 5.3 software was applied for the meta-analysis.
Results: Data for 10 studies with a total of 3814 patients were retrieved. The advantages of EC comparing to LT include:… More >

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ARTICLE

Hitesh Agrawal^1,2, Oriana K. Wright³, Kathleen E. Carberry^1,4, S. Kristen Sexson Tejtel^1,2, Carlos M. Mery^1,5, Silvana Molossi^1,2
Congenital Heart Disease, Vol.12, No.6, pp. 721-725, 2017, DOI:10.1111/chd.12473
Abstract Background: Long-term outcome data on patients with anomalous aortic origin of coronary arteries (AAOCA) is sparse and they are often managed in a nonuniform manner. There is subjective perception of anxiety and unmet needs in these patients and families.
Methods: An online survey of 13 questions was sent to 74 families of patients with AAOCA between May and October 2015. Descriptive statistics were performed.
Results: A total of 31 (47%) families responded. Of these, 27 expressed the need to interact with other patients/families with AAOCA. The majority were interested in either face-to-face meetings (77%) or online support groups… More >

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ARTICLE

Athanasios Koutsoukis¹, Xavier Halna du Fretay², Patrick Dupouy³, Phalla Ou⁴, Jean-Pierre Laissy⁴, Jean-Michel Juliard⁵, Fabien Hyafil⁶, Pierre Aubry⁵
Congenital Heart Disease, Vol.12, No.6, pp. 726-732, 2017, DOI:10.1111/chd.12504
Abstract Objective: The diagnosis of anomalous connections of the coronary arteries (ANOCOR) requires an appropriate identification for the management of the patients involved. We studied the observer variability in the description and classification of ANOCOR between a nonexpert group of physicians and a group of expert physicians, using the ANOCOR cohort.
Patients and design: Consecutive patients identified by 71 referring cardiologists were included in the ANOCOR cohort. Anomalous connection was diagnosed by invasive and/or computed tomography coronary angiography. Angiographic images were reviewed by an angiographic committee with experience in this field. Both investigators and angiographic committee filled out… More >

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1184

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ARTICLE

Brian A. Boe¹, Mark D. Norris², Jeffrey D. Zampi², Albert P. Rocchini², Gregory J. Ensing²
Congenital Heart Disease, Vol.12, No.6, pp. 733-739, 2017, DOI:10.1111/chd.12514
Abstract Objective: We sought to identify a time during cardiac ejection when the instantaneous pressure gradient (IPG) correlated best, and near unity, with peak-to-peak systolic ejection gradient (PPSG) in patients with congenital aortic stenosis. Noninvasive echocardiographic measurement of IPG has limited correlation with cardiac catheterization measured PPSG across the spectrum of disease severity of congenital aortic stenosis. A major contributor is the observation that these measures are inherently different with a variable relationship dependent on the degree of stenosis.
Design: Hemodynamic data from cardiac catheterizations utilizing simultaneous pressure measurements from the left ventricle (LV) and ascending aorta (AAo)… More >

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ARTICLE

Joseph M. Krepp¹, Mary J. Roman², Richard B. Devereux², Adrienne Bruce¹, Siddharth K. Prakash³, Shaine A. Morris⁴, Dianna M. Milewicz³, Kathryn W. Holmes⁵, William Ravekes⁵, Ralph V. Shohet⁶, Reed E. Pyeritz⁷, Cheryl L. Maslen⁸, Barbara L. Kroner⁹, Kim A. Eagle¹⁰, Liliana Preiss⁹, GenTAC Investigators^*, Federico M. Asch¹
Congenital Heart Disease, Vol.12, No.6, pp. 740-745, 2017, DOI:10.1111/chd.12520
Abstract Background: Unicuspid aortic valve (UAV) is a rare disorder, often difficult to distinguish from bicuspid aortic valve (BAV). BAV and UAV share valve pathology such as the presence of a raphe, leaflet fusion, aortic stenosis, aortic regurgitation, and/or ascending aortic dilatation, but a comprehensive echocardiographic comparison of patients with UAV and BAV has not been previously performed.
Methods: We investigated UAV and BAV patients at an early stage of disease included in GenTAC, a national registry of genetically related aortic aneurysms and associated cardiac conditions. Clinical and echocardiographic data from the GenTAC Registry were compared between 17… More >

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1242

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ARTICLE

Eun Young Choi¹, Jinyoung Song², Heirim Lee², Chang Ha Lee³, Jun Huh², I.-Seok Kang², Ji Hyuk Yang⁴, Tae Gook Jun⁴
Congenital Heart Disease, Vol.12, No.6, pp. 746-750, 2017, DOI:10.1111/chd.12507
Abstract Background: Balloon dilatation of a bioprosthetic valve in the pulmonary position could be performed to delay valve replacement. We proposed to identify the long-term effectiveness of such a procedure.
Methods: We reviewed the medical records of 49 patients who underwent balloon valvuloplasty between January 2000 and December 2015. The primary goal was to determine the time interval until the following surgical or catheter intervention.
Results: The mean age at bioprosthetic valve insertion was 5.7 years old, and the mean age for ballooning was 11.7 years. The mean interval after pulmonary valve replacement was 71.6 months. The mean ratio… More >

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1214

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ARTICLE

Jimmy C. Lu¹, Manish Bansal², Sarina K. Behera³, Jeffrey R. Boris⁴, Brian Cardis⁵, John S. Hokanson⁶, Bahram Kakavand⁷, Roy Jedeikin⁸
Congenital Heart Disease, Vol.12, No.6, pp. 751-755, 2017, DOI:10.1111/chd.12509
Abstract Objective: As part of the American College of Cardiology Adult Congenital and Pediatric Cardiology Section effort to develop quality metrics (QMs) for ambulatory pediatric practice, the chest pain subcommittee aimed to develop QMs for evaluation of chest pain.
Design: A group of 8 pediatric cardiologists formulated candidate QMs in the areas of history, physical examination, and testing. Consensus candidate QMs were submitted to an expert panel for scoring by the RAND-UCLA modified Delphi process. Recommended QMs were then available for open comments from all members.
Patients: These QMs are intended for use in patients 5–18 years old, referred… More >

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1048

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ARTICLE

Jonathan N. Johnson^1,2, Cindy S. Barrett³, Wayne H. Franklin⁴, Eric M. Graham⁵, Nancy J. Halnon⁶, Brandy A. Hattendorf⁷, Catherine D. Krawczeski⁸, James J. McGovern⁹, Matthew J. O’Connor¹⁰, Amy H. Schultz¹¹, Jeffrey M. Vinocur¹², Devyani Chowdhury¹³, Jeffrey B. Anderson¹⁴
Congenital Heart Disease, Vol.12, No.6, pp. 756-761, 2017, DOI:10.1111/chd.12519
Abstract Introduction: In 2012, the American College of Cardiology’s (ACC) Adult Congenital and Pediatric Cardiology Council established a program to develop quality metrics to guide ambulatory practices for pediatric cardiology. The council chose five areas on which to focus their efforts; chest pain, Kawasaki Disease, tetralogy of Fallot, transposition of the great arteries after arterial switch, and infection prevention. Here, we sought to describe the process, evaluation, and results of the Infection Prevention Committee’s metric design process.
Methods: The infection prevention metrics team consisted of 12 members from 11 institutions in North America. The group agreed to work… More >

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1169

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ARTICLE

Juan Villafane¹, Thomas C. Edwards², Karim A. Diab³, Gary M. Satou⁴, Elizabeth Saarel⁵, Wyman W. Lai⁶, Gerald A. Serwer⁷, Peter P. Karpawich⁸, Russell Cross⁹, Russell Schiff¹⁰, Devyani Chowdhury¹¹, Thomas J. Hougen¹²
Congenital Heart Disease, Vol.12, No.6, pp. 762-767, 2017, DOI:10.1111/chd.12523
Abstract Objective: The objective of this study was to develop quality metrics (QMs) relating to the ambulatory care of children after complete repair of tetralogy of Fallot (TOF).
Design: A workgroup team (WT) of pediatric cardiologists with expertise in all aspects of ambulatory cardiac management was formed at the request of the American College of Cardiology (ACC) and the Adult Congenital and Pediatric Cardiology Council (ACPC), to review published guidelines and consensus data relating to the ambulatory care of repaired TOF patients under the age of 18 years. A set of quality metrics (QMs) was proposed by the… More >

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ARTICLE

Rahul H. Rathod^1,2, Brittney Jurgen^1,2, Rose A. Hamershock³, Kevin G. Friedman^1,2, Audrey C. Marshall^1,2, Mihail Samnaliev⁴, Dionne A. Graham³, Kathy Jenkins^1,2, James E. Lock^1,2, Andrew J. Powell^1,2
Congenital Heart Disease, Vol.12, No.6, pp. 768-776, 2017, DOI:10.1111/chd.12508
Abstract Background: Standardized Clinical Assessment and Management Plans (SCAMPs) are a quality improvement initiative designed to reduce unnecessary utilization, decrease practice variation, and improve patient outcomes. We created a novel methodology, the SCAMP managed episode of care (SMEOC), which encompasses multiple encounters to assess the impact of the arterial switch operation (ASO) SCAMP on total costs.
Methods: All ASO SCAMP patients (dates March 2009 to July 2015) were compared to a control group of ASO patients (January 2001 to February 2009). Patients were divided into “younger” (<2 years) and “older” (2–18 years) subgroups. Utilization included all cardiology visits,… More >

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1134

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ARTICLE

Wen-Yi Luo¹, Zhuo-Ming Xu¹, Li Hong², Qian-Yue Wu¹, Yue-Yue Zhang¹
Congenital Heart Disease, Vol.12, No.6, pp. 777-782, 2017, DOI:10.1111/chd.12489
Abstract Background: Food allergy is a rapidly growing public health concern because of its increasing prevalence, as well as life-threatening potential. There is limited knowledge on the nutritional status for the pediatric congenital heart disease (CHD) patients with food allergy.
Objective: This study investigated both clinical and nutritional outcomes according to the CHD infants with food allergy.
Methods: Forty CHD infants with food allergy and 39 controls were recruited in Shanghai, China. The height and weight for age and weight for height were converted to z-scores to evaluate their effects on nutritional status before and after CHD operation.
Results: Cow’s… More >

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1128

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ARTICLE

Monique M. Gardner, Meryl S. Cohen
Congenital Heart Disease, Vol.12, No.6, pp. 783-786, 2017, DOI:10.1111/chd.12506
Abstract Background: Noncompaction is a poorly understood form of cardiomyopathy that typically affects the left ventricle and may be associated with congenital heart disease. Right ventricular noncompaction (RVNC) may occur when the left ventricle is affected but is rarely seen in isolation. RVNC may have clinical significance affecting surgical and long-term outcomes. We describe the diagnosis and clinical course in three patients at our institution.
Methods: We performed a retrospective review of patients diagnosed with RVNC over a 12- month period at our institution and reviewed their imaging and clinical course.
Results: Three patients were identified. All had diagnosis… More >

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ARTICLE

Constanze Pfitzer^1,2,3, Paul C. Helm⁴, Hannah Ferentzi^1,5, Lisa-Maria Rosenthal¹, Ulrike M. M. Bauer^4,6, Felix Berger^1,3,7, Katharina R. L. Schmitt^1,3
Congenital Heart Disease, Vol.12, No.6, pp. 787-793, 2017, DOI:10.1111/chd.12515
Abstract Objective: To assess the prevalence of congenital heart disease (CHD) in Germany in relation to phenotypes, severity and gender.
Design: Cross-sectional registry study.
Setting: We analyzed data from patients with CHD born between 1996 and 2015.
Patients: A total of 26 630 patients, registered with the NRCHD, were born between 1996 and 2015. 10 927 patients were excluded from the current analysis due to prior registration with the NRCHD under the German PAN Prevalence Study, which showed a potential bias in the inclusion of this patient population (proportion of mild cardiac lesions was comparatively high due to improved diagnostic… More >

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ARTICLE

Dmitry Tumin^1,2, Helen Chou¹, Don Hayes Jr^1,3,4, Joseph D. Tobias^1,2,5, Mark Galantowicz^6,7, Patrick I. McConnell^6,7
Congenital Heart Disease, Vol.12, No.6, pp. 794-799, 2017, DOI:10.1111/chd.12513
Abstract Objective: Adults with congenital heart disease may require heart transplantation for end-stage heart failure. Whereas heart transplantation potentially allows adults with congenital heart disease to resume their usual activities, employment outcomes in this population are unknown. Therefore, we investigated the prevalence and predictors of work participation after heart transplantation for congenital heart disease.
Design: Retrospective review of a prospective registry.
Setting: United Network for Organ Sharing registry of transplant recipients in the United States.
Patients: Adult recipients of first-time heart transplantation with a primary diagnosis of congenital heart disease, performed between 2004 and 2015.
Interventions: None.
Outcome measures: Employment status reported… More >

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ARTICLE

Tugcin Bora Polat

Congenital Heart Disease, Vol.12, No.6, pp. 800-807, 2017, DOI:10.1111/chd.12512
Abstract Objectives: To retrospectively review the outcome of stent placement in neonates with a vertical ductus, present a technique of ductal stenting via the axillary artery and compare it to ductal stening via the femoral venous access.
Design: Nineteen patients with duct-dependent pulmonary circulations through a vertical ductus arteriosus were treated with stent implantation. Those patients were retrospectively included in the study. In the first nine of these cases, stent delivery was done transvenously. In the latter ten cases, we favored the axillary artery access to the transvenous approach for stenting the vertical ductus arteriosus. Wire-target technique was… More >

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1093

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ARTICLE

Navaneetha Sasikumar, Antony Hermuzi, Chun-Po Steve Fan, Kyong-Jin Lee, Rajiv Chaturvedi, Edward Hickey, Osami Honjo, Glen S. Van Arsdell, Christopher A. Caldarone, Arnav Agarwal, Lee Benson
Congenital Heart Disease, Vol.12, No.6, pp. 808-814, 2017, DOI:10.1111/chd.12516
Abstract Objectives: Mortality associated with the modified Blalock–Taussig shunt (MBTS) remains high despite advanced perioperative management. This study was formulated to provide data on (1) current indications, (2) outcomes, and (3) factors affecting mortality and morbidity.
Design: A retrospective single center chart review identified 95 children (excluding hypoplastic left heart lesions) requiring a MBTS. Mortality and major morbidity were analyzed using the Kaplan Meier method and risk factor analysis using Cox’s proportional hazard regression.
Results: Median age was 8 (0–126) days, weight 3.1(1.7–5.4) kg. Seventy-three percent were neonates, 58% duct dependent and 73% had single ventricle physiology. Ninety-seven percent… More >

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1125

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ARTICLE

Michael D. Seckeler¹, Katri Typpo², Jendar Deschenes², Ruth Higgins³, Ricardo Samson¹, Peter Lichtenthal⁴
Congenital Heart Disease, Vol.12, No.6, pp. 815-819, 2017, DOI:10.1111/chd.12517
Abstract Objective: To determine the accuracy of a continuous cardiac output monitor (FloTrac sensor) for measuring cardiac index in children with congenital heart disease undergoing cardiac catheterization. Cardiac index is a critical hemodynamic parameter measured during catheterizations in children with congenital heart disease. This has been challenging to measure accurately and many clinicians rely on predictive equations for calculating cardiac index.
Design: Prospective, nonrandomized trial.
Setting: Tertiary care congenital heart center.
Patients: Consecutive participants ≤18 years old undergoing clinically indicated cardiac catheterizations from September 2014 through August 2015.
Interventions: Oxygen consumption was measured using the Vmax Encore 229 monitor attached to… More >

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ARTICLE

Raysa Morales-Demori
Congenital Heart Disease, Vol.12, No.6, pp. 820-827, 2017, DOI:10.1111/chd.12521
Abstract Congenital heart disease (CHD) is present in approximately 50% of patients with trisomy 21 (T21) and Turner syndrome (TS). According to the American Academy of Pediatrics, every patient with these genetic disorders should have a postnatal echocardiogram. T21 is usually associated with atrioventricular (30%–60%), atrial (16%-21%), or ventricular septal defects (14%–27%). TS is usually associated with left-sided heart disease. However, the spectrum of CHD in these genetic disorders is wider than those mentioned lesions. More cardiac surgical procedures are offered to these patients and that has influenced positively their life expectancy for some CHD conditions. More >

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ARTICLE

Andrew D. Spearman
Congenital Heart Disease, Vol.12, No.6, pp. 828-833, 2017, DOI:10.1111/chd.12543
Abstract A genetic basis of congenital heart disease (CHD) has been known for decades. In addition to the sequence of the genome, the contribution of epigenetics to pediatric cardiology is increasingly recognized. Multiple epigenetic mechanisms, including DNA methylation, histone modification, and RNA-based regulation, are known mediators of cardiovascular disease, including both development and progression of CHD and its sequelae. Basic understanding of the concepts of epigenetics will be essential to all pediatric cardiologists in order to understand mechanisms of pathophysiology, pharmacotherapeutic concepts, and to understand the role of epigenetics in precision medicine. More >

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