Open Access

EDITORIAL

Robert H. Anderson
Congenital Heart Disease, Vol.13, No.2, pp. 161-162, 2018, DOI:10.1111/chd.12609
Abstract This article has no abstract. More >

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Rohit S. Loomba¹, Robert H. Anderson²
Congenital Heart Disease, Vol.13, No.2, pp. 163-166, 2018, DOI:10.1111/chd.12593
Abstract Impact factor has been used as a metric by which to gauge scientific journals for several years. A metric meant to describe the performance of a journal overall, impact factor has also become a metric used to gauge individual performance as well. This has held true in the field of pediatric cardiology where many divisions utilize impact factor of journals that an individual has published in to help determine the individual’s academic achievement. This subsequently can impact the individual’s promotion through the academic ranks. We review the purpose of impact factor, its strengths and weaknesses, More >

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981

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ARTICLE

Jeffery B. Anderson¹, Devyani Chowdhury², Jean A. Connor³, Curt J. Daniels⁴, Craig E. Fleishman⁵, Michael Gaies⁶, Jeffrey Jacobs^7,8, John Kugler⁹, Nicolas Madsen¹, Robert H. Beekman⁶, Stacey Lihn¹⁰, Kay Stewart-Huey¹¹, Robert Vincent¹¹, Robert Campbell¹¹
Congenital Heart Disease, Vol.13, No.2, pp. 167-180, 2018, DOI:10.1111/chd.12575
Abstract Pediatric cardiovascular services are responding to the dynamic changes in the medical environment, including the business of medicine. The opportunity to advance our pediatric cardiology field through collaboration is now realized, permitting us to define meaningful quality metrics and establish national benchmarks through multicenter efforts. In March 2016, the American College of Cardiology hosted the first Adult Congenital/Pediatric Cardiology Section Congenital Heart Community Day. This was an open participation meeting for clinicians, administrators, patients/parents to propose metrics that optimize patient care and outcomes for a state-of-the-art congenital heart center of the 21st century. Care center… More >

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976

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REVIEW

Laura S. Kverneland^1,2, Peter Kramer², Stanislav Ovroutski²
Congenital Heart Disease, Vol.13, No.2, pp. 181-193, 2018, DOI:10.1111/chd.12570
Abstract Almost fifty years after its first clinical application, the modified Fontan operation is among the most frequently performed procedures in congenital heart disease surgery in children today. The objective of this review is to systematically summarize the international evolution of outcomes in regard to morbidity and mortality of patients with Fontan palliation. All studies published over the past five decades with more than 100 Fontan patients included were screened. In eligible studies, information concerning preoperative patients’ characteristics, Fontan modifications employed, early mortality, long-term survival and frequency of relevant complications was extracted. Ultimately, thirty-one studies published… More >

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1128

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REVIEW

Jing Wu¹, Li Kuang¹, Lijuan Fu²
Congenital Heart Disease, Vol.13, No.2, pp. 194-202, 2018, DOI:10.1111/chd.12586
Abstract Objective: The aim of this study was to evaluate the effects of inspiratory muscle training (IMT) in chronic heart failure (CHF) patients.
Design: We searched MEDLINE, EMBASE, Cochrane Library, CINHAL, and CBMdisc to collect controlled trials on the application of inspiratory muscle training in CHF patients from the establishment of these databases to November 2016. Two reviewers independently screened literature according to the inclusion and exclusion criteria, extracted data, and assessed the quality of literature. Meta-analysis was conducted by software RevMan5.3.
Results: Eight studies involving 302 patients were identified. Meta-analysis indicated that IMT significantly improved PI_max, V_E/VCO₂ slope and More >

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1066

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REVIEW

So Yeon Kim^1,2, Diana S. Wolfe², Cynthia C. Taub²
Congenital Heart Disease, Vol.13, No.2, pp. 203-209, 2018, DOI:10.1111/chd.12546
Abstract Aims: Pregnancy in patients with Marfan’s syndrome (MFS) carries an increased risk of cardiovascular complications, resulting in increased maternal and fetal mortality and morbidity. Literature on MFS pregnant patients is relatively sparse, and there has yet to be a concrete consensus on the management of this unique patient population. The purpose of our paper is to provide a literature review of case reports and studies on MFS during pregnancy (published between 2005 and 2015) and to explore cardiovascular outcomes of patients with MFS.
Methods and Results: Of the 852 women in our review, there were 1112 pregnancies,… More >

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1015

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ARTICLE

Amir-Reza Hosseinpour¹, Marie-Hélène Perez², David Longchamp², Jacques Cotting², Nicole Sekarski³, Michel Hurni¹, René Prêtre¹, Stefano Di Bernardo³
Congenital Heart Disease, Vol.13, No.2, pp. 210-216, 2018, DOI:10.1111/chd.12545
Abstract Objective: Congenital cardiac malformations with high pulmonary blood flow and pressure due to left-to-right shunts are usually repaired in early infancy for both the benefits of early relief of heart failure and the fear that the concomitant pulmonary hypertension may become irreversible unless these defects are corrected at an early age. Age, however, has been a poor predictor of irreversibility of pulmonary hypertension in our experience, which is presented here.
Design: A retrospective observational study. We defined “late” as age ≥2 years. We examined clinical, echocardiographic, and hemodynamic data from all patients aged ≥2 years with such… More >

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951

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ARTICLE

Horacio G. Carvajal^1,2, Kathryn J. Lindley³, Trupti Shah¹, Anoop K. Brar¹, Philip M. Barger³, Joseph J. Billadello³, Pirooz Eghtesady¹
Congenital Heart Disease, Vol.13, No.2, pp. 217-221, 2018, DOI:10.1111/chd.12554
Abstract Objective: The effects of pregnancy on autograft dilatation and neoaortic valve function in patients with a Ross procedure have not been studied. We sought to evaluate the effect of pregnancy on autograft dilatation and valve function in these patients with the goal of determining whether pregnancy is safe after the Ross procedure.
Design: A retrospective chart review of female patients who underwent a Ross procedure was conducted.
Patients: Medical records for 51 patients were reviewed. Among the 33 patients who met inclusion criteria, 11 became pregnant after surgery and 22 did not.
Outcome Measures: Echocardiographic reports were used to… More >

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970

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ARTICLE

Karen Hinsley, Margaret Evans-Langhorst, Courtney Porter, Stephanie Chandler, Christina VanderPluym, John Triedman, Vassilios J. Bezzerides
Congenital Heart Disease, Vol.13, No.2, pp. 222-225, 2018, DOI:10.1111/chd.12551
Abstract Objective: This quality improvement study was implemented to demonstrate consistent and reliable post procedure anticoagulation for patients undergoing left-sided ablations. We evaluated the safety and efficacy of anticoagulation practice during a transition from anticoagulation with overnight infusion of unfractionated heparin to a single subcutaneous injection of low molecular weight heparin.
Methods: Outcomes for patients who received unfractionated heparin from January 2014 to October 2014, were compared with outcomes of patients who received low molecular weight heparin from October 2014 to October 2015. Complications prepractice and postpractice change were documented and compared to establish confidence in the practice… More >

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952

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ARTICLE

David Aurigemma, John W. Moore, Gabrielle Vaughn, Nasser Moiduddin, Howaida G. El-Said
Congenital Heart Disease, Vol.13, No.2, pp. 226-231, 2018, DOI:10.1111/chd.12552
Abstract Background: Right ventricular outflow tract (RVOT) stents have been used as palliation in patients with severe tetralogy of Fallot (TOF). Radiofrequency perforation of the RVOT has also been described in patients with pulmonary atresia (PA)/ventricular septal defect (VSD). However, RVOT stenting in conjunction with radiofrequency perforation as a means for establishing reliable pulmonary blood flow in patients with PA/VSD has not previously been reported.
Objectives: Our aim is to report our experience with using perforation of plate-like pulmonary valve atresia combined with stenting of RVOT as an alternative and equally efficacious intervention for infants with PA/VSD, as… More >

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999

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Adam McKillop¹, Brian W. McCrindle^1,2, Gina Dimitropoulos³, Adrienne H. Kovacs³
Congenital Heart Disease, Vol.13, No.2, pp. 232-240, 2018, DOI:10.1111/chd.12553
Abstract Objective: A physically active lifestyle can help maintain positive physical and psychosocial health outcomes among adults with congenital heart disease (CHD). This study explored the physical activity perceptions and behaviors among young adults with CHD.
Design: This was a cross-sectional, mixed-methods study that included objectively measured physical activity assessment (accelerometer), individual semistructured interviews, and psychosocial questionnaires.
Results: Fifteen participants (67% male; 21 ± 3 years old) with moderate (n = 10) or complex (n = 5) CHD were recruited from an outpatient adult CHD clinic. Participants accumulated 26 ± 16 minutes of moderate-to-vigorous physical activity per day, and… More >

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1032

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ARTICLE

Weiguang Yang¹, Frank L. Hanley², Frandics P. Chan³, Alison L. Marsden^1,4, Irene E. Vignon-Clementel⁵, Jeffrey A. Feinstein^1,4
Congenital Heart Disease, Vol.13, No.2, pp. 241-250, 2018, DOI:10.1111/chd.12556
Abstract Background: Up to 90% of individuals with Alagille syndrome have congenital heart diseases. Peripheral pulmonary artery stenosis (PPS), resulting in right ventricular hypertension and pulmonary flow disparity, is one of the most common abnormalities, yet the hemodynamic effects are illdefined, and optimal patient management and treatment strategies are not well established. The purpose of this pilot study is to use recently refined computational simulation in the setting of multiple surgical strategies, to examine the influence of pulmonary artery reconstruction on hemodynamics in this population.
Materials and Methods: Based on computed tomography angiography and cardiac catheterization data, preoperative… More >

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1066

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Amanda L. Phillips¹, Muhammad Yasir Qureshi^1,2, Benjamin W. Eidem^1,2,3, Frank Cetta^1,2,3
Congenital Heart Disease, Vol.13, No.2, pp. 251-253, 2018, DOI:10.1111/chd.12560
Abstract Background: Lambl’s excrescences (LE) are fibrous extensions that can be found along the lines of closure of the aortic valve. Due to improvements in ultrasound technology, LE are frequently imaged during transthoracic echocardiography (TTE) in adults.
Objective: The purpose of this study was to determine the prevalence of LE among children from two eras (2004–2006 and 2011–2012) and the effect of technological advancements on LE detection.
Methods: TTE from 700 subjects (age 18 years old or younger) were reviewed. All parasternal long and short axis images of the aortic valve were reviewed by a board certified echocardiographer, and… More >

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1042

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ARTICLE

Camilla Sandberg^1,2, Magnus Hedström¹, Karin Wadell², Mikael Dellborg³, Anders Ahnfelt³, Anna-Klara Zetterström⁴, Amanda Öhrn⁴, Bengt Johansson¹
Congenital Heart Disease, Vol.13, No.2, pp. 254-262, 2018, DOI:10.1111/chd.12562
Abstract Objective: The beneficial effects of exercise training in acquired heart failure and coronary artery disease are well known and have been implemented in current treatment guidelines. Knowledge on appropriate exercise training regimes for adults with congenital heart disease is limited, thus further studies are needed. The aim of this study was to examine the effect of home-based interval exercise training on maximal endurance capacity and peak exercise capacity.
Design: Randomized controlled trial.
Methods: Twenty-six adults with complex congenital heart disease were recruited from specialized units for adult congenital heart disease. Patients were randomized to either an intervention group—12… More >

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1146

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Peter Bak¹, Cristel S. Hjortshøj², Peter Gæde³, Lars Idorn⁴, Lars Søndergaard², Annette S. Jensen²
Congenital Heart Disease, Vol.13, No.2, pp. 263-270, 2018, DOI:10.1111/chd.12565
Abstract Objective: Cyanotic congenital heart disease is a systemic disease, with effects on multiple organ systems. A high prevalence of subclinical hypothyroidism (SCH) has been reported in a small cohort of cyanotic congenital heart disease patients. Subclinical hypothyroidism has been associated with various adverse cardiovascular effects, as well as an increased risk of progression to overt hypothyroidism. The aim of this study was to examine the prevalence of SCH in cyanotic congenital heart disease patients, consider possible etiologies, and evaluate thyroid function over time.
Methods: First, 90 clinically stable cyanotic congenital heart disease patients were examined with blood… More >

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996

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ARTICLE

Yu Zhu^1*, Yong Chen^1*, Yu Feng², Di Yu¹, Xuming Mo¹
Congenital Heart Disease, Vol.13, No.2, pp. 271-281, 2018, DOI:10.1111/chd.12567
Abstract We conducted this meta-analysis to address the open question of a possible association between maternal body mass index (BMI) and congenital heart defects (CHDs) in infants. We conducted a comprehensive computerized search of PubMed, Web of Science, Medline, and Embase databased (January 1980 through August 2017). We assessed the association between maternal BMI and the risk for congenital heart defects in their offspring. Study-specific relative risk estimates were polled according to random-effect or fixed-effect models. From 2567 citations, a total of 13 case-control studies and 4 cohort studies were selected for a meta-analysis, including more… More >

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1095

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ARTICLE

Xinyan Li¹, Xiaoxia Qiu¹, Huan Huang¹, Yili Zhao², Xueqin Li¹, Meng Li¹, Xiaoxian Tian¹
Congenital Heart Disease, Vol.13, No.2, pp. 282-287, 2018, DOI:10.1111/chd.12568
Abstract Objective: To evaluate the efficacy of using fetal heart size measurements derived from axial echocardiography to predict homozygous α-thalassemia-1.
Design: Prospective diagnostic study.
Setting: The carrier rate of α-thalassemia-1 (–/αα) in China’s Guangxi Zhuang Autonomous Region is approximately 15%. If both parents are carriers, the risk of homozygous a-thalassemia-1 in one pregnancy is 25%.
Patients: Singleton mid-pregnancies at risk of homozygous α-thalassemia-1 were enrolled.
Outcome Measures: Fetal heart measurements, including heart diameter (HD), heart length (HL), heart circumference (HC), and heart area (HA), were measured. The z-scores for these heart parameters were then calculated separately based on previously constructed z-score… More >

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1224

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ARTICLE

Clotilde Kingsley¹, Saad Ahmad², John Pappachan¹, Sujata Khambekar¹, Thomas Smith¹, Diane Gardiner¹, James Shambrook¹, Shankar Baskar³, Ryan Moore³, Gruschen Veldtman³
Congenital Heart Disease, Vol.13, No.2, pp. 288-294, 2018, DOI:10.1111/chd.12569
Abstract Background: The right ventricular (RV) contractile reserve is a measure of the dynamic function of the RV and is a sensitive indicator of volume load. This can be measured noninvasively using the tricuspid annular plane systolic excursion (TAPSE) during exercise. We studied the RV contractile reserve of patients after tetralogy of Fallot (TOF) repair with varying degree of RV dilation and pulmonary regurgitation (PR), and compared them to a control group.
Methods: Twenty-six patients who had undergone TOF repair (mean age 29 ± 10 years) were identified and stratified into three group based on the presence and… More >

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Fariborz Soheili^1,2, Zahra Jalili³, Mahtab Rahbar⁴, Zahed Khatooni¹, Amir Mashayekhi⁵, Hossein Jafari⁶
Congenital Heart Disease, Vol.13, No.2, pp. 295-304, 2018, DOI:10.1111/chd.12571
Abstract Background: The mutations in GATA4 gene induce inherited atrial and ventricular septation defects, which is the most frequent forms of congenital heart defects (CHDs) constituting about half of all cases.
Method: We have performed High resolution melting (HRM) mutation scanning of GATA4 coding exons of nonsyndrome 100 patients as a case group including 39 atrial septal defects (ASD), 57 ventricular septal defects (VSD) and four patients with both above defects and 50 healthy individuals as a control group. Our samples are categorized according to their HRM graph. The genome sequencing has been done for 15 control samples and… More >

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987

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Jia-Wang Xiao, Qi-Guang Wang, Duan-Zhen Zhang, Chun-Sheng Cui, Xiumin Han, Po Zhang, Chuangju Hou, Xian-Yang Zhu
Congenital Heart Disease, Vol.13, No.2, pp. 305-310, 2018, DOI:10.1111/chd.12572
Abstract Objective: To evaluate the clinical efficacy, safety, and long-term outcomes of percutaneous closure (PC) and surgical repair of ruptured sinus of Valsalva aneurysm (RSVA).
Methods: Eighty-five consecutive patients with RSVA were included in this study. Patients were considered candidates for PC if they met the criterion, surgical repair was performed on patients who were unsuitable or failed PC. Of them, 30 patients underwent PC, while the other 55 patients had surgical repair.
Results: RSVA was successfully occluded in 29 of 30 patients who were treated by PC. The mean narrowest diameter at the ruptured site was 6.45 ±… More >

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1012

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Michael V. Di Maria¹, Matthew Mulvahill², James Jaggers³, David Dunbar Ivy¹, Adel K. Younoszai¹
Congenital Heart Disease, Vol.13, No.2, pp. 311-318, 2018, DOI:10.1111/chd.12574
Abstract Objective: Infants with single ventricle physiology typically undergo cardiac catheterization prior to superior cavopulmonary anastomosis (SCPA) to assess operative suitability. Predictors of poor outcome at sea level include elevated pulmonary artery pressure (mPAP), indexed pulmonary vascular resistance (PVRi), age <3 months, significant atrioventricular valve regurgitation, among others. Increased altitude has vasoconstrictive effects on the pulmonary vasculature, which may affect pre-SCPA hemodynamics and outcomes. The goal of this study was to determine the predictive value of pre-SCPA catheterization data with regard to reaching Fontan palliation at altitude.
Design: A retrospective review revealed 150 patients who underwent pre-SCPA catheterization… More >

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964

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Djoeke Wolff¹, Henricus B. M. van de Wiel², Mirthe E. de Muinck Keizer¹, Joost P. van Melle³, Petronella G. Pieper³, Rolf M. F. Berger¹, Tjark Ebels⁴, Willebrord C. M. Weijmar Schultz⁵
Congenital Heart Disease, Vol.13, No.2, pp. 319-326, 2018, DOI:10.1111/chd.12576
Abstract Objective: To get an impression of the quality of life (QOL) and sexual well-being in the Fontan population, and to generate hypotheses for future research.
Methods: For this cross-sectional pilot study, questionnaires regarding health-related QOL, sexual function and fertility/pregnancy were completed by 21 patients with a Fontan circulation >16 years old, followed at the University Medical Center Groningen, the Netherlands. Semi-structured qualitative interviews were conducted in 8 patients.
Results: Fontan patients scored significantly lower on general health than their healthy peers (t (19)=-3.0, P = .008), whereas their scores on other QOL domains and sexual well-being were comparable… More >

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938

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ARTICLE

Angeline D. Opina, Constance Cephus, Yunfei Wang, Samuel Younan, Douglas Moodie
Congenital Heart Disease, Vol.13, No.2, pp. 327-333, 2018, DOI:10.1111/chd.12580
Abstract Objective: Studies have shown improvement in lipid levels after institution of lifestyle changes in children enrolled in closely monitored programs. There programs are difficult to mimic in real world clinics. We aim to determine if diet and exercise result in improvement in lipid levels in patients seen in a designated lipid clinic in a real life setting.
Design: Retrospective review of patients followed for dyslipidemia at the Texas Children’s Hospital Lipid Clinic from May 1, 2012 to May 1, 2015. Patients included were seen more than once, had repeat lipid testing, and abnormal baseline lipid levels. Multivariate… More >

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883

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ARTICLE

Kriti Puri¹, Peter Kazembe², Treasure Mkaliainga³, Msandeni Chiume³, Antonio G. Cabrera¹, Amy Sims Sanyahumbi¹
Congenital Heart Disease, Vol.13, No.2, pp. 334-341, 2018, DOI:10.1111/chd.12573
Abstract Malawi is one of the poorest nations in the world, ranked 151st among 195 countries by the World Bank, with an under-5-year mortality rate of 63 per 1000 live births. There are no previous studies describing the spectrum of inpatient pediatric cardiology consultations in sub-Saharan Africa. A descriptive cohort study was performed at Kamuzu Central Hospital (KCH), a tertiary care hospital in Lilongwe, Malawi. Demographic, anthropometric, and clinical information for all cardiology consults patients aged 0–18 years admitted to the children’s wards over a period of 1 month was reviewed. Seventy-three consults and 69 echocardiograms… More >

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