Open Access

EDITORIAL

Jane W. Newburger
Congenital Heart Disease, Vol.14, No.2, pp. 126-127, 2019, DOI:10.1111/chd.12773
Abstract This article has no abstract. More >

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REVIEW

Georges Ephrem¹, Camden Hebson², Anitha John³, Estella Moore⁴, Maan Jokhadar⁴, Ryan Ford⁵, Gruschen Veldtman⁶, Yoav Dori⁷, Michelle Gurvitz^8,9, Brian Kogon¹⁰, Adrienne Kovacs¹¹, Meghan Roswick, Michael McConnell¹², Wendy M. Book⁴, Fred Rodriguez III^4,12
Congenital Heart Disease, Vol.14, No.2, pp. 128-137, 2019, DOI:10.1111/chd.12685
Abstract The initial “Frontiers in Fontan Failure” conference in 2015 in Atlanta, Georgia, provided an opportunity for experts in the field of pediatric cardiology and adult congenital heart disease to focus on the etiology, physiology, and potential interventions for patients with “Failing Fontan” physiology. Four types of “Fontan Failure” were described and then published by Dr Book et al. The acknowledgment that even Dr Fontan himself realized that the Fontan proce‐ dure “imposed a gradually declining functional capacity and premature late death after an initial period of often excellent palliation.” The purpose of the second “Frontiers… More >

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1158

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ARTICLE

Emmanuel Akintoye¹, Gruschen R. Veldtman², William R. Miranda³, Heidi M. Connolly³, Alexander C. Egbe³
Congenital Heart Disease, Vol.14, No.2, pp. 138-139, 2019, DOI:10.1111/chd.12690
Abstract The purpose of this study was to determine the optimal age for performing Fontan operation using data from the National Inpatient Sample. Our results showed that although the Fontan operation was most commonly performed at age 2 in the United States, age 3 is the optimum age for this procedure as evident by lower rate of in‐hospital mortality, procedure‐related complications, and rate of nonroutine home discharge when procedure is performed at age 3 years. More >

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1076

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ARTICLE

Neil C. Zaki^1,2, Michael S. Kelleman¹, W. James Parks^1,3, Timothy C. Slesnick^1,3, Michael E. McConnell^1,3, Matthew E. Oster^1,3
Congenital Heart Disease, Vol.14, No.2, pp. 140-146, 2019, DOI:10.1111/chd.12692
Abstract Objective: Gated cardiac MRI offers the most detailed and accurate noninvasive method of assessing cardiac anatomy, particularly in patients with complex congenital heart disease. The proposed benefits of using cMRI as a routine screening tool in the Fontan population include early recognition of asymptomatic, postoperative anatomic and physiologic changes. In 2011, we therefore instituted at our center a recommended practice of cMRI screening in patients with Fontan physiology at 3 and 8 years postFontan operation. The purpose of this study was to determine the impact of this standardized practice of cMRI screening on the clinical management… More >

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ARTICLE

Alessia Callegari^1,2,4, Rhoia Neidenbach², Ornella Milanesi¹, Biagio Castaldi¹, Martin Christmann⁴, Masamichi Ono³, Jan Müller², Peter Ewert², Alfred Hager²
Congenital Heart Disease, Vol.14, No.2, pp. 147-155, 2019, DOI:10.1111/chd.12694
Abstract Aim: The Fontan circulation is highly dependent on ventilation, improving pulmonary blood flow and cardiac output. A reduced ventilatory function is reported in these patients. The extent of this impairment and its relation to exercise capacity and qual‐ ity of life is unknown and objective of this study.
Methods: This multicenter retrospective/cross‐sectional study included 232 patients (140 females, age 25.6 ± 10.8 years) after Fontan palliation (19.8% atrioventricular connection; 20.3% atriopulmonary connection; 59.9% total cavopulmonary connec‐ tion). Resting spirometry, cardiopulmonary exercise tests, and quality‐of‐life assess‐ ment (SF‐36 questionnaire) were performed between 2003 and 2015.
Results: Overall, mean forced… More >

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1162

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REVIEW

Camden L. Hebson¹, Michael E. McConnell², David W. Hannon³
Congenital Heart Disease, Vol.14, No.2, pp. 156-161, 2019, DOI:10.1111/chd.12720
Abstract Dysautonomia is an increasingly recognized yet still poorly understood disease within the field of pediatrics. Symptoms, including dizziness, headaches, fatigue, joint pain, anxiety, and intolerance of heat or cold, are often significant and difficult to sort, especially in terms of their relation to each other. This often leads to referral to multiple subspecialists, who then proceed to treat seemingly familiar symptoms in kind. In the authors’ experience, this leads to more frustration on the part of the patients and their physicians when symptom improvement does not follow (or can even worsen). On the other hand, More >

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ARTICLE

Raffaele Giordano¹, Massimiliano Cantinotti², Giuseppe Comentale¹, Luigi Di Tommaso¹, Gabriele Iannelli¹¹, Emanuele Pilato¹, Gaetano Palma¹
Congenital Heart Disease, Vol.14, No.2, pp. 162-166, 2019, DOI:10.1111/chd.12680
Abstract Background: In this study, we compared our experience about early and midterm follow‐up outcomes for right anterolateral minithoracotomy (RAMT) vs full sternot‐ omy (FS) in surgical aortic valve replacement (AVR) among adolescents with bicuspid aortic valve (BAV).
Methods: Patients were retrospectively enrolled from January 2008 to December 2017. Inclusion criteria were patients with BAV who had to undergo to AVR. They were divided in two groups: RAMT and FS. The choice of RAMT was based on indi‐ vidual surgeon’s preferences or when expressly requested by patient that was in‐ formed of nonconventional approach.
Results: We enrolled 61 patients,… More >

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1206

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ARTICLE

Masaki Sato¹, Kei Inai^1,2, Mikiko Shimizu¹, Hisashi Sugiyama¹, Toshio Nakanishi²
Congenital Heart Disease, Vol.14, No.2, pp. 167-175, 2019, DOI:10.1111/chd.12683
Abstract Objective: The recognition of fluid retention is critical in treating heart failure (HF). Bioelectrical impedance analysis (BIA) is a well-known noninvasive method; however, data on its role in managing patients with congenital heart disease (CHD) are limited. Here, we aimed to clarify the correlation between BIA and HF severity as well as the prognostic value of BIA in adult patients with CHD.
Design: This prospective single-center study included 170 patients with CHD admitted between 2013 and 2015. We evaluated BIA parameters (intra- and extracellular water, protein, and mineral levels, edema index [EI, extracellular water-to-total body water ratio]),… More >

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1127

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ARTICLE

Nayan T. Srivastava^1,2, Roger Hurwitz³, W. Aaron Kay⁴, George J. Eckert⁵, Alisha Kuhlenhoelter⁶, Nicole DeGrave², Eric S. Ebenroth^2,6
Congenital Heart Disease, Vol.14, No.2, pp. 176-184, 2019, DOI:10.1111/chd.12698
Abstract Objective: For over 20 years, we have followed a cohort of patients who underwent the Mustard procedure for d‐transposition of the great arteries. The current study follows the same cohort from our last study in 2007 to reassess their functional ca‐ pacity and quality of life.
Participants: Of the original 45 patients, six patients have required cardiac transplant and 10 patients have died, including two of the transplanted patients. Twenty‐five of the remaining patients agreed to participate in this current study.
Design: Patients underwent comparable testing to the previous studies when possi‐ ble including exercise stress testing, echocardiography,… More >

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1196

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ARTICLE

Jennifer L. Lapum¹, Suzanne Fredericks¹, Barbara Bailey², Terrence M. Yau^3,4, Jennifer Graham⁵, Ariane J. Marelli^6,7
Congenital Heart Disease, Vol.14, No.2, pp. 185-192, 2019, DOI:10.1111/chd.12716
Abstract Objective: The number of adults with congenital heart disease (CHD) has increased substantially because of medical advances that are extending life expectancy beyond childhood. A historical investigation is timely not only because of this significant demographic shift, but also because stakeholder engagement is increasingly important in shaping research directions. The study questions were: From the perspective of stakeholders, what is known about the medical treatment for the adult CHD cohort in Canada and how has it changed over time?
Design/Methods: We harnessed the use of historical research methods and conducted interviews with 21 key informants in order… More >

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1113

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ARTICLE

Joseph T. Poterucha¹, Saraschandra Vallabhajosyula², Alexander C. Egbe², Joseph S. Krien³, Devon O. Aganga⁴, Kimberly Holst⁵, Adele W. Golden⁶, Joseph A. Dearani⁵, Sheri S. Crow⁴
Congenital Heart Disease, Vol.14, No.2, pp. 193-200, 2019, DOI:10.1111/chd.12717
Abstract Background: High levels of vasoactive inotrope support (VIS) after congenital heart surgery are predictive of morbidity in pediatric patients. We sought to discern if this relationship applies to adults with congenital heart disease (ACHD).
Methods: We retrospectively studied adult patients (≥18 years old) admitted to the intensive care unit after cardiac surgery for congenital heart disease from 2002 to 2013 at Mayo Clinic. Vasoactive medication dose values within 96 hours of admis‐ sion were examined to determine the relationship between VIS score and poor out‐ come of early mortality, early morbidity, or complication related morbidity.
Results: Overall, 1040… More >

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1092

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ARTICLE

Shankar Baskar, Andrew N. Redington, Philip R. Khoury, Timothy K. Knilans, David S. Spar, Richard J. Czosek
Congenital Heart Disease, Vol.14, No.2, pp. 201-206, 2019, DOI:10.1111/chd.12684
Abstract Background: Traditional indices to evaluate biventricular (BiV) pacing are load dependent, fail to assess dynamic changes, and may not be appropriate in patients with congenital heart disease (CHD). We therefore measured the force‐frequency relationship (FFR) using tissue Doppler‐de‐ rived isovolumic acceleration (IVA) to assess the dynamic adaption of the myocardium and its vari‐ ability with different ventricular pacing strategies.
Methods: This was a prospective pilot study of pediatric and young adult CHD patients with biventricular or multisite pacing systems. Color‐coded myocardial velocities were recorded at the base of the systemic ventricular free wall. IVA was calculated at… More >

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1072

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ARTICLE

Matthew Lewis¹, William Whang², Angelo Biviano², Kathleen Hickey², Hasan Garan², Marlon Rosenbaum¹
Congenital Heart Disease, Vol.14, No.2, pp. 207-212, 2019, DOI:10.1111/chd.12695
Abstract Background: Catheter ablation is commonly performed to treat atrial arrhythmias in adult congenital heart disease (ACHD). Despite the frequency of ablations in the ACHD population, predictors of recurrence remain poorly defined.
Objective: We sought to determine predictors of arrhythmia recurrence in ACHD patients following catheter ablation for atrial arrhythmias.
Methods: We performed a retrospective study of all catheter ablations for atrial arrhythmias performed in ACHD patients between January 12, 2005 and February 11, 2015 at our institution. Prespecified exposures of interest and time from ablation to recurrence were determined via chart review.
Results: Among 124 patients (mean age: 45… More >

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ARTICLE

Jun Oyamada¹, Chisato Shimizu¹, Jihoon Kim², Matthew R. Williams^1,3, Eileen Png⁴, Martin L. Hibberd⁴, Adriana H. Tremoulet^1,3, James C. Perry^1,3, Jane C. Burns^1,3
Congenital Heart Disease, Vol.14, No.2, pp. 213-220, 2019, DOI:10.1111/chd.12696
Abstract Background: We previously described the association of genetic variants in calcium channel genes and susceptibility to Kawasaki disease (KD), an acute, self‐limited vas‐ culitis, and the most common cause of acquired cardiac disease in children. Abnormal repolarization of cardiomyocytes and changes in T wave morphology have been re‐ ported in KD but have not been studied systematically.
Methods: We analyzed acute and convalescent ECG T wave morphology in two inde‐ pendent cohorts of KD subjects and studied the association between bifid T waves and genetic variants in previously reported genes with SNVs associated with cardiac repolarization.
Results: Bifid… More >

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ARTICLE

Patrick D. Evers^1,*, Tarek Alsaied^1,2,*, Jeffrey B. Anderson¹, James F. Cnota¹, Allison A. Divanovic¹
Congenital Heart Disease, Vol.14, No.2, pp. 221-229, 2019, DOI:10.1111/chd.12713
Abstract Objective: Maternal anti-Ro/SSA and anti-La/SSB antibodies can lead to fetal complete heart block (CHB). Current guidelines recommend weekly echocardiographic screening between 16 and 28 weeks gestation. Given the cost of screening and the rarity of conduction abnormalities in fetuses of mothers with low anti-Ro levels (<50 U/mL), we sought to identify a strategy that optimizes resource utilization.
Design: Decision analysis cost-utility modeling was performed for three screening paradigms: “standard screening” (SS) in which mid-gestation mothers are screened weekly, “limited screening” (LS) in which fetal echocardiograms are avoided unless the fetus develops bradycardia, and “targeted screening by maternal… More >

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ARTICLE

Soham Dasgupta¹, Shae Anderson¹, Michael Kelleman², Ritu Sachdeva¹
Congenital Heart Disease, Vol.14, No.2, pp. 230-235, 2019, DOI:10.1111/chd.12687
Abstract Introduction: In the pediatric Appropriate Use Criteria (AUC), abnormal electrocardiogram (ECG) in an asymptomatic patient has been rated as an “Appropriate” indication for transthoracic echocardiogram (TTE). We hypothesized that the yield of abnormal findings on TTE for this indication will be low.
Methods: All asymptomatic patients (≤ 18 years) from January 1, 2015 to December 31, 2017 who underwent initial outpatient evaluation at our center and had a TTE ordered for an abnormal ECG, were included. Clinic records were reviewed to obtain ECG and TTE findings.
Results: Of the 199 study patients, 13 (6.5%) had abnormal findings. Incomplete… More >

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ARTICLE

Samantha C. Butler^1,4, Anjali Sadhwani^1,4, Christian Stopp², Jayne Singer^1,3,4, David Wypij^2,5,6, Carolyn Dunbar‐Masterson², Janice Ware^1,3,4, Jane W. Newburger^2,5
Congenital Heart Disease, Vol.14, No.2, pp. 236-245, 2019, DOI:10.1111/chd.12686
Abstract Objective: Mortality rates for children with congenital heart disease (CHD) have significantly declined, resulting in a growing population with associated neurodevelopmental disabilities. American Heart Association guidelines recommend systematic developmental screening for children with CHD. The present study describes results of inpatient newborn neurodevelopmental assessment of infants after open heart surgery.
Outcome measures: We evaluated the neurodevelopment of a convenience sample of high‐risk infants following cardiac surgery but before hospital discharge using an adaptation of the Newborn Behavioral Observation. Factor analysis examined relationships among assessment items and consolidated them into domains of development.
Results: We assessed 237 infants at… More >

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1228

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ARTICLE

Mariana M. Clavé¹, Nair Y. Maeda², Ana M. Thomaz¹, Sergio P. Bydlowski³, Antonio A. Lopes¹
Congenital Heart Disease, Vol.14, No.2, pp. 246-255, 2019, DOI:10.1111/chd.12688
Abstract Background: Ideally, vasodilator therapies for pulmonary arterial hypertension (PAH) should have a favorable impact on markers of vascular dysfunction, in addition to their known effects on hemodynamics, cardiac function, and patient’s physical capacity.
Methods: We analyzed circulating (plasma) markers of endothelial and platelet activation/dys‐ function (enzyme‐linked immunoassays) in the specific setting of advanced PAH associated with congenital heart disease, during the course of sildenafil and tadalafil therapies. Thirty‐one patients were enrolled (age 10‐54 years), most of them with chronic hypoxemia and elevated hematocrit. Drugs were administered orally for 6 months (sildenafil [n = 16], 20 mg t.i.d.;… More >

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ARTICLE

Ersin Çagrı Şimşek¹, Selcen Yakar Tülüce², Kamil Tülüce³, Sadık Volkan Emren², Serap Çuhadar⁴, Cem Nazlı²
Congenital Heart Disease, Vol.14, No.2, pp. 256-263, 2019, DOI:10.1111/chd.12718
Abstract Objective: The bicuspid aortic valve (BAV) is the most common congenital heart dis‐ ease. The process of aortic dilatation is not completely clear in patients with the BAV. Apelin is a peptide found at high levels in vascular endothelial cells which has a role in vascular regulation and cardiovascular function. The aim of this study was to de‐ termine the relationship between serum apelin levels and ascending aortic dilatation in adult patients with BAV.
Design: This cross‐sectional study included 62 patients with isolated BAV and to an age, gender, and body mass index‐matched control group of 58… More >

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ARTICLE

Kailyn Anderson¹, James Cnota^2,3, Jeanne James^4,5, Erin M. Miller^2,3, Ashley Parrott³, Valentina Pilipenko^1,2, Kathryn Nicole Weaver^1,2, Amy Shikany³
Congenital Heart Disease, Vol.14, No.2, pp. 264-273, 2019, DOI:10.1111/chd.12721
Abstract Objective: To evaluate the prevalence of Noonan spectrum disorders (NSD) in a pediatric population with valvar pulmonary stenosis (vPS) and identify the clinical characteristics that differentiate those with NSD from those without NSD.
Design: A retrospective chart review of 204 patients diagnosed with vPS between 9/1/2012 and 12/1/2016 at a pediatric medical center was performed. The quantitative features of vPS, genetic diagnosis information, and phenotypic characteristics of Noonan syndrome were collected. Chi‐square test, Fisher’s exact test, t test, Wilcoxon rank‐sum test, and ANOVA were used for comparisons among the groups. Logistic regression was used to test for… More >

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1105

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ARTICLE

Vanessa Marie Hormaza¹, Mark Conaway², Daniel Scott Schneider¹, Jeffrey Eric Vergales¹
Congenital Heart Disease, Vol.14, No.2, pp. 274-279, 2019, DOI:10.1111/chd.12722
Abstract Objective: Limited information is known on how right ventricular function affects outcomes after stage 2 palliation. We evaluated the impact of different right ventricular indices prior to stage 2 palliation on morbidity and mortality.
Design: Retrospective study design.
Setting: Pediatric Heart Network Single Ventricle Reconstruction Trial Public Data Set.
Patient: Any variant of stage 1 palliation and all anatomic hypoplastic left heart syndrome variants in the trial were evaluated. Echocardiograms prior to stage 2 palliation were analyzed and compared between those who failed and those who survived.
Intervention: None.
Outcome measures: Mortality was defined as death, listed for transplant, or transplanted… More >

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1084

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ARTICLE

Charlotte A. Houck^1,2, Reinder Evertz³, Christophe P. Teuwen¹, Jolien W. Roos‐Hesselink¹, Janneke A. E. Kammeraad⁴, Anthonie L. Duijnhouwer³, Natasja M. S. de Groot³, Ad J. J. C. Bogers²
Congenital Heart Disease, Vol.14, No.2, pp. 280-287, 2019, DOI:10.1111/chd.12724
Abstract Objective: Outcomes after surgical repair of complete atrioventricular septal defect (cAVSD) have improved. With advancing age, the risk of development of dysrhythmias may increase. The aims of this study were to (1) examine development of sinus node dysfunction (SND), atrial and ventricular tachyarrhythmias, and (2) study progression of atrioventricular conduction abnormalities in young adult patients with repaired cAVSD.
Study design: In this retrospective multicenter study, 74 patients (68% female) with a cAVSD repaired in childhood were included. Patients’ medical files were evaluated for occurrence of SND, atrioventricular conduction block (AVB), atrial and ventricular tachyarrhythmias.
Results: Median age at… More >

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ARTICLE

Jesse Lee, Doaa Abdullah Shahbah, Howaida El‐Said, Rodrigo Rios, Kanishka Ratnayaka, John Moore
Congenital Heart Disease, Vol.14, No.2, pp. 288-296, 2019, DOI:10.1111/chd.12726
Abstract Background: In the modern era, results of the arterial switch operation (ASO) for transposition of the great arteries are excellent. However, because of the LeCompte maneuver, there may be a propensity for development of pulmonary artery stenosis. We encountered atypical complications of pulmonary artery stenting in patients after the ASO, including aorto‐pulmonary fistula and coronary compression.
Methods: We performed a 10‐year retrospective review of catheterizations per‐ formed in patients after ASO in our institution with a focus on adverse events.
Results: Diagnostic and interventional catheterizations were performed in 47 pa‐ tients. In 29 patients, 37 interventional procedures performed,… More >

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ARTICLE

Brian A. Boe, Sharon L. Cheatham, Aimee K. Armstrong, Darren P. Berman, Joanne L. Chisolm, John P. Cheatham
Congenital Heart Disease, Vol.14, No.2, pp. 297-304, 2019, DOI:10.1111/chd.12728
Abstract Objective: We sought to describe the leaflet morphology variation in the Melody Transcatheter Pulmonary Valve (TPV) and evaluate associated outcomes. The Melody TPV is constructed from harvested bovine jugular venous valves which have been rigorously tested. Natural anatomic leaflet variations are seen in the Melody TPV but have not been evaluated.
Design: A Melody TPV leaflet morphology classification system was devised after reviewing a subset of photographed and implanted TPVs. All images were blindly re‐ viewed by implanters and classified. Midterm hemodynamic outcomes and complica‐ tions of the Melody TPVs were compared by leaflet morphology.
Results: Photographed Melody… More >

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1031

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ARTICLE

Jamie L. Jackson¹, Jennifer Morack², Millie Harris², Jennifer DeSalvo³, Curt J. Daniels⁴, Deena J. Chisolm²
Congenital Heart Disease, Vol.14, No.2, pp. 305-310, 2019, DOI:10.1111/chd.12732
Abstract Objective: The current study aims to identify the rates of lapses in care and loss to follow‐up before age one through age five for white and nonwhite congenital heart disease (CHD) survivors. Nonwhite CHD survivors were hypothesized to experience an earlier lapse in care and be lost to follow‐up than whites.
Design: Patients were from a large pediatric hospital and had (1) at least one outpa‐ tient cardiology clinic visit or cardiac surgery visit before the age of one and (2) a di‐ agnosis of moderate or complex structural CHD. Cardiology outpatient utilization rates were tracked from… More >

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1086

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ARTICLE

Regan E. Giesinger¹, Adrianne R. Bischoff³, Patrick J. McNamara^1,2
Congenital Heart Disease, Vol.14, No.2, pp. 311-316, 2019, DOI:10.1111/chd.12738
Abstract Ligation of a hemodynamically significant ductus arteriosus results in significant changes in loading conditions which have predictable consequences. Postligation cardiac syndrome, defined as hypotension requiring inotropic support and failure of oxygenation and ventilation, may occur 6‐12 hours following ligation due to left ven‐ tricular systolic and diastolic failure, respectively. Afterload is the primary driver of this decompensation. In this review, we describe the pathophysiological changes in loading conditions associated with postligation cardiac syndrome and other contrib‐ utors to cardiovascular dysfunction following ductal ligation. We present strategies for perioperative optimization and a physiology‐based algorithm for postoperative More >

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