Home / Journals / CHD / Vol.12, No.6, 2017
  • Open Access

    EDITORIAL

    Are the spin doctors winning?

    James E. Lock1,2,3
    Congenital Heart Disease, Vol.12, No.6, pp. 697-698, 2017, DOI:10.1111/chd.12548
    Abstract This article has no abstract. More >

  • Open Access

    REVIEW

    Cardiovascular adaptation to the Fontan circulation

    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 overarching aim is to provide… More >

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    ARTICLE

    Comparison of extracardiac conduit and lateral tunnel for functional single-ventricle patients: A meta-analysis

    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: lower 30… More >

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    ARTICLE

    Family perception of unmet support needs following a diagnosis of congenital coronary anomaly in children: Results of a survey

    Hitesh Agrawal1,2, Oriana K. Wright3, Kathleen E. Carberry1,4, S. Kristen Sexson Tejtel1,2, Carlos M. Mery1,5, Silvana Molossi1,2
    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 (71%). Regarding… More >

  • Open Access

    ARTICLE

    Interobserver variability in the classification of congenital coronary abnormalities: A substudy of the anomalous connections of the coronary arteries registry

    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 a questionnaire to… More >

  • Open Access

    ARTICLE

    Temporal relationship between instantaneous pressure gradients and peak-to-peak systolic ejection gradient in congenital aortic stenosis

    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) in patients with… More >

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    ARTICLE

    Bicuspid and unicuspid aortic valves: Different phenotypes of the same disease? Insight from the GenTAC Registry

    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 patients with UAV… More >

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    ARTICLE

    The effect of balloon valvuloplasty for bioprosthetic valve stenosis at pulmonary positions

    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 of balloon… More >

  • Open Access

    ARTICLE

    Development of quality metrics for ambulatory pediatric cardiology: Chest pain

    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 for initial… More >

  • Open Access

    ARTICLE

    Development of quality metrics for ambulatory pediatric cardiology: Infection prevention

    Jonathan N. Johnson1,2, 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 on specific infection… More >

  • Open Access

    ARTICLE

    Development of quality metrics for ambulatory care in pediatric patients with tetralogy of Fallot

    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 WT. The metrics… More >

  • Open Access

    ARTICLE

    Impact of standardized clinical assessment and management plans on resource utilization and costs in children after the arterial switch operation

    Rahul H. Rathod1,2, Brittney Jurgen1,2, Rose A. Hamershock3, Kevin G. Friedman1,2, Audrey C. Marshall1,2, Mihail Samnaliev4, Dionne A. Graham3, Kathy Jenkins1,2, James E. Lock1,2, Andrew J. Powell1,2
    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, tests, and procedures.… More >

  • Open Access

    ARTICLE

    Nutritional outcomes in infants with food allergy after cardiac surgery

    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 milk… More >

  • Open Access

    ARTICLE

    Clinical findings in right ventricular noncompaction in hypoplastic left heart syndrome

    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 of RVNC… More >

  • Open Access

    ARTICLE

    Changing prevalence of severe congenital heart disease: Results from the National Register for Congenital Heart Defects in Germany

    Constanze Pfitzer1,2,3, Paul C. Helm4, Hannah Ferentzi1,5, Lisa-Maria Rosenthal1, Ulrike M. M. Bauer4,6, Felix Berger1,3,7, Katharina R. L. Schmitt1,3
    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 capability… More >

  • Open Access

    ARTICLE

    Employment after heart transplantation among adults with congenital heart disease

    Dmitry Tumin1,2, Helen Chou1, Don Hayes Jr1,3,4, Joseph D. Tobias1,2,5, Mark Galantowicz6,7, Patrick I. McConnell6,7
    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… More >

  • Open Access

    ARTICLE

    Stenting the vertical ductus arteriosus via axillary artery access using “wire-target” technique

    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 used to attain… More >

  • Open Access

    ARTICLE

    Outcomes of Blalock-Taussig shunts in current era: A single center experience

    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 had a… More >

  • Open Access

    ARTICLE

    Inaccuracy of a continuous arterial pressure waveform monitor when used for congenital cardiac catheterization

    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 >

  • Open Access

    ARTICLE

    Congenital heart disease and cardiac procedural outcomes in patients with trisomy 21 and Turner syndrome

    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. Single ventricular anatomy is associated… More >

  • Open Access

    ARTICLE

    Epigenetics for the pediatric cardiologist

    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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