Open Access

EDITORIAL

Douglas S. Moodie
Congenital Heart Disease, Vol.12, No.1, pp. 5-5, 2017, DOI:10.1111/chd.12444
Abstract This article has no abstract. More >

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Camden Hebson^1,2, Wendy Book¹, Robert W. Elder³, Ryan Ford⁴, Maan Jokhadar¹, Kirk Kanter⁵, Brian Kogon⁵, Adrienne H. Kovacs⁶, Rebecca D. Levit¹, Michael Lloyd¹, Kevin Maher², Preeti Reshamwala⁴, Fred Rodriguez III^1,2, Rene Romero⁷, Thor Tejada¹, Anne Marie Valente⁸, Gruschen Veldtman⁹, Michael McConnell^1,2
Congenital Heart Disease, Vol.12, No.1, pp. 6-16, 2017
Abstract “Frontiers in Fontan Failure” was the title of a 2015 conference sponsored by Children’s Healthcare of Atlanta and Emory University School of Medicine. In what is hoped to be the first of many such gatherings, speakers and attendees gathered to discuss the problem of long-term clinical deterioration in these patients. Specific focuses included properly defining the problem and then discussing different treatment strategies, both medical and surgical. The health of the liver after Fontan palliation was a particular point of emphasis, as were quality of life and future directions. More >

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1094

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Kavitha N. Pundi¹, Krishna N. Pundi², Jonathan N. Johnson^1,3, Joseph A. Dearani⁴, Zhuo Li, BS⁵, David J. Driscoll¹, Philip L. Wackel¹, Christopher J. McLeod³, Frank Cetta^1,3, Bryan C. Cannon^1,3
Congenital Heart Disease, Vol.12, No.1, pp. 17-23, 2017
Abstract Objectives: We sought to examine the incidence and predictors of arrhythmias and sudden cardiac death (SCD) after Fontan operation.
Background: Arrhythmias and SCD have been reported following operations for congenital heart disease, but the incidence and risk factors have not been well defined in patients after a Fontan operation.
Methods: We reviewed records of all patients who had a Fontan operation from 1973 to 2012 (n 5 1052) at our institution. A questionnaire was mailed to patients who were not known to be deceased at the initiation of the study. Late arrhythmias were classified as bradyarrhythmias or tachyarrhythmias requiring treatment… More >

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988

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Alexander C. Egbe^*, Heidi M. Connolly^*, Joseph T. Poterucha^*,†, Carole A. Warnes^*
Congenital Heart Disease, Vol.12, No.1, pp. 24-31, 2017
Abstract Objectives. There is a paucity of data about mixed aortic valve disease (MAVD) in patients with bicuspid/unicuspid aortic valve (BAV). This study sought to describe the outcomes of patients with moderate/severe MAVD.
Methods. We queried our database for patients with BAV and moderate/severe MAVD seen between 1994 and 2013. We excluded patients with baseline New York Heart Association (NYHA) III/IV symptoms, left ventricular ejection fraction <50%, aortic dimension >50 mm, and significant disease of other valves. The purpose of the study was to determine the freedom from NYHA III/IV symptoms and aortic valve replacement (AVR).
Results. We identified 138 patients… More >

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973

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Lianne M. Geerdink^1,2, Gideon J. du Marchie Sarvaas³, Irene M. Kuipers⁴, Willem A. Helbing⁵, Tammo Delhaas⁶, Henriette ter Heide⁷, Lieke Rozendaal⁸, Chris L. de Korte⁹, Sandeep K. Singh¹⁰, Tjark Ebels¹¹, Mark G. Hazekamp¹², Felix Haas¹³, Ad J. J. C. Bogers¹⁴, Livia Kapusta^1,15
Congenital Heart Disease, Vol.12, No.1, pp. 32-39, 2017
Abstract Objective: Surgical outcomes of pediatric patients with Ebstein’s anomaly are often described as part of all-age-inclusive series. Our objective is to focus on patients treated surgically in childhood (0-18 y). We study the intended treatment (biventricular or 1.5 ventricle repair or univentricular palliation), freedom from unplanned reoperation and survival of this specific age group, in a nationwide study.
Design: Records of all Ebstein’s anomaly patients born between 1980 and 2013 were reviewed. Demographic variables, intraoperative procedures and postoperative outcomes were analyzed.
Results: Sixty-three patients underwent 109 operations. Median follow-up after diagnosis was 121 months (range 0-216 months). Twenty-nine (46%) patients… More >

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1024

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Hoi Lam She^*, Arno A.W. Roest^†, Emmeline E. Calkoen^†, Pieter J. van den Boogaard^‡, Rob J. van der Geest^‡, Mark G. Hazekamp^§, Albert de Roos^‡, Jos J.M. Westenberg^‡
Congenital Heart Disease, Vol.12, No.1, pp. 40-48, 2017
Abstract Purpose. To evaluate the inflow pattern and flow quantification in patients with functional univentricular heart after Fontan’s operation using 4D flow magnetic resonance imaging (MRI) with streamline visualization when compared with the conventional 2D flow approach.
Method. Seven patients with functional univentricular heart after Fontan’s operation and twenty-three healthy controls underwent 4D flow MRI. In two orthogonal two-chamber planes, streamline visualization was applied, and inflow angles with peak inflow velocity (PIV) were measured. Transatrioventricular flow quantification was assessed using conventional 2D multiplanar reformation (MPR) and 4D MPR tracking the annulus and perpendicular to the streamline inflow at PIV, and they… More >

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Christoph Gräni, Dominik C. Benz, Mathias Possner, Olivier F. Clerc, Fran Mikulicic, Jan Vontobel, Julia Stehli, Tobias A. Fuchs, Aju P. Pazhenkottil, Oliver Gaemperli, Philipp A. Kaufmann, Ronny R. Buechel
Congenital Heart Disease, Vol.12, No.1, pp. 49-57, 2017
Abstract Objective: To provide data on the value of fused cardiac hybrid imaging with coronary computed tomography angiography (CCTA) and positron emission tomography myocardial perfusion imaging (PET-MPI) in patients with complex coronary artery anomalies (CCAA).
Design/setting: This is a retrospective, single-center study.
Patients: Seven consecutive patients with CCAA (mean 57 ± 7 y, 86% were male) who underwent clinically indicated hybrid CCTA/PET-MPI between 2005 and 2015 in our clinic were included. The findings from both modalities and fused cardiac hybrid imaging were evaluated in these patients.
Results: Out of the seven patients with CCAA, two patients had Bland–White–Garland anomaly, two patients… More >

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1109

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Richard J. Dobson¹, Ify Mordi², Mark H. Danton¹, Niki L. Walker¹, Hamish A. Walker¹, Nikolaos Tzemos²
Congenital Heart Disease, Vol.12, No.1, pp. 58-66, 2017
Abstract Objective: Myocardial fibrosis has been associated with poorer outcomes in tetralogy of Fallot, however only a handful of studies have assessed its significance in the current era. Our aim was to quantify the amount of late gadolinium enhancement in both the LV and RV in a contemporary cohort of adults with surgically repaired tetralogy of Fallot, and assess the relationship with adverse clinical outcomes.
Design: Single centre cohort study
Setting: National tertiary referral center
Patients: One hundred fourteen patients with surgically repaired tetralogy of Fallot with median age 29.5 years (range 17.5-64.2). Prospective follow-up for mean 2.4 years (SD 1.29).
… More >

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Jonathan Komisar¹, Shubhika Srivastava², Miwa Geiger², John Doucette³, Helen Ko², Jay Shenoy², Rajesh Shenoy²
Congenital Heart Disease, Vol.12, No.1, pp. 67-73, 2017
Abstract Background: Antenatal diagnosis of congenital heart defects (CHD) can impact outcomes in neonates with severe CHD. Obstetric screening guidelines and the indications for fetal echocardiography (FE) have evolved in an attempt to improve the early prenatal detection of CHD. Analyzing yield for specific indications will help clinicians better stratify at-risk pregnancies.
Methods: Retrospective cohort study of all FE performed between 2000 and 2010 at a single tertiary care academic medical center in New York City. A total of 9878 FE met inclusion criteria for our study. In cases of multiple gestations (MG), each fetus was counted as a separate study.
… More >

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951

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Nicola Maschietto¹, Luca Semplicini², Giulio Ceolotto², Arianna Cattelan², Helen Poser³, Ilaria Iacopetti³, Gabriele Gerardi³, Giulia Maria De Benedictis³, Tommaso Pilla³, Daniele Bernardini³, Luca Aresu⁴, Stefania Rizzo⁵, Cristina Basso⁵, Andrea Semplicini², Ornella Milanesi¹
Congenital Heart Disease, Vol.12, No.1, pp. 74-83, 2017
Abstract Background: Stent implantation is the treatment of choice for adolescents and adults with aortic coarctation (CoAo). Despite excellent short-term results, 20%–40% of the patients develop arterial hypertension later in life, which was attributed to inappropriate response of the aortic baroreceptors to increased stiffness of the ascending aorta (ASAO), either congenital or induced by CoAo repair. In particular, it has been hypothesized that stent itself may cause or sustain hypertension. Therefore, we aimed to study the hemodynamic and structural impact following stent implantation in the normal aorta of a growing animal.
Methods: Eight female sheep completed the study and a stent… More >

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987

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Benjamin H. Goot, Sonali Patel, Brian Fonseca
Congenital Heart Disease, Vol.12, No.1, pp. 84-90, 2017
Abstract Objective: When imaging the lower airway by MRI, the traditional technique turbo spin echo (TSE) results in high quality 2D images, however planning and acquisition times are lengthy. An alternative, delayed volume interpolated breath-holds examination (VIBE), is a 3D gradient echo technique that produces high spatial resolution imaging of the airway in one breath-hold. The objective of this study is to retrospectively evaluate the accuracy of lower airway measurements obtained by delayed VIBE when compared to TSE.
Design: Patients with congenital heart disease who underwent a cardiac MRI (CMR) that included a delayed VIBE sequence from 5/2008 to 9/2013 were… More >

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996

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Leda Klouda¹, Wayne J. Franklin¹, Anita Saraf^1,2, Dhaval R. Parekh¹, David D. Schwartz³
Congenital Heart Disease, Vol.12, No.1, pp. 91-98, 2017
Abstract Objective: Congenital heart disease (CHD) can affect the developing central nervous system, resulting in neurocognitive and behavioral deficits. Preoperative neurological abnormalities as well as sequelae of the open heart operations required to correct structural abnormalities of the heart contribute to these deficits. There are few studies examining the neurocognitive functioning of adults with CHD. This study sought to investigate multiple domains of neurocognitive functioning in adult survivors of CHD who had childhood cardiac surgery with either moderate or severe disease complexity.
Design: A total of 48 adults (18–49 years of age) who had undergone cardiac surgery for CHD prior to… More >

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952

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Raya Safa, Ronald Thomas, Peter P. Karpawich
Congenital Heart Disease, Vol.12, No.1, pp. 99-104, 2017
Abstract Background: The early ECG repolarization QRS pattern (ERp), with J-point elevation of 0.1 mV in two contiguous inferior and/or lateral leads, can be associated with ventricular arrhythmias among adults. The significance of an ERp in the young is unknown.
Objective: The purpose of this study was to assess the prevalence of ERp among young patients (pts), describe and correlate the characteristics with clinical presentations and any arrhythmias.
Methods: This was a 1 y retrospective review of ECGs obtained from patients referred specifically for documented arrhythmias, possible arrhythmia-related symptoms or sports clearance. ECGs were analyzed for ERp (J-point, ascending/horizontal patterns, location)… More >

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1131

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Diego A. Lara¹, Mary K. Ethen², Mark A. Canfield², Wendy N. Nembhard³, Shaine A. Morris¹
Congenital Heart Disease, Vol.12, No.1, pp. 105-112, 2017
Abstract Background: Hypoplastic left heart syndrome (HLHS) is strongly associated with Turner syndrome (TS); outcome data when these conditions coexist is sparse. We aimed to investigate long-term survival and causes of death in this population.
Methods: The Texas Birth Defects Registry was queried for all live born infants with HLHS during 1999–2007. We used Kaplan–Meier and Cox regression analyses to compare survival among patients with HLHS with TS (HLHS/TS＋) to patients who had HLHS without genetic disorders or extracardiac birth defects (HLHS/TS–).
Results: Of the 542 patients with HLHS, 11 had TS (2.0%), 71 had other extracardiac birth defects or genetic… More >

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1000

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Giovanni Biglino^1,2, Claudio Capelli^2,3, Despina Koniordou³, Di Robertshaw², Lindsay-Kay Leaver², Silvia Schievano^2,3, Andrew M. Taylor^2,3, Jo Wray²
Congenital Heart Disease, Vol.12, No.1, pp. 113-118, 2017
Abstract Background: Nurse education and training are key to providing congenital heart disease (CHD) patients with consistent high standards of care as well as enabling career progression. One approach for improving educational experience is the use of 3D patient-specific models.
Objectives: To gather pilot data to assess the feasibility of using 3D models of CHD during a training course for cardiac nurses; to evaluate the potential of 3D models in this context, from the nurses’ perspective; and to identify possible improvements to optimise their use for teaching.
Design: A cross-sectional survey.
Setting: A national training week for cardiac nurses.
Participants: One… More >

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