Open Access

EDITORIAL

Congenital Heart Disease, Vol.13, No.6, pp. 883-883, 2018, DOI:10.1111/chd.12734
Abstract This article has no abstract. More >

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Robin H.S. Chen¹, Adolphus K.T. Chau¹, Pak Cheong Chow¹, Tak Cheung Yung¹, Yiu Fai Cheung^1,2, Kin Shing Lun¹
Congenital Heart Disease, Vol.13, No.6, pp. 884-891, 2018, DOI:10.1111/chd.12658
Abstract Objective: Transcatheter valve perforation for pulmonary atresia intact ventricular septum is the standard of care for patients with mild right ventricular hypoplasia. However, its role in moderate right ventricular hypoplasia has been less well defined. We sought to report the long‐term outcome of patients with moderate hypoplastic right ventricle who had undergone the procedure.
Design, Settings, and Patients: We performed a retrospective analysis on patients who had undergone transcatheter pulmonary valve perforation from January 1996 to January 2015 at our institution. The procedures would be carried out irrespective of the right ventricular size, as long as there were no absolute… More >

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1021

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Shiraz A. Maskatia¹, Christopher J. Petit^2,3, Curtis D. Travers², David J. Goldberg⁴, Lindsay S. Rogers⁴, Andrew C. Glatz⁴, Athar M. Qureshi⁶, Bryan H. Goldstein⁵, Jingning Ao^2,3, Ritu Sachdeva^2,3
Congenital Heart Disease, Vol.13, No.6, pp. 892-902, 2018, DOI:10.1111/chd.12671
Abstract Background: In patients with pulmonary atresia, intact ventricular septum (PA/IVS) following right ventricular (RV) decompression, RV size and morphology drive clinical outcome. Our objectives were to (1) identify baseline and postdecompression echo‐ cardiographic parameters associated with 2V circulation, (2) identify echocardio‐ graphic parameters associated with RV growth and (3) describe changes in measures of RV size and changes in RV loading conditions.
Methods: We performed a retrospective analysis of patients who underwent RV de‐ compression for PA/IVS at four centers. We analyzed echocardiograms at baseline, postdecompression, and at follow up (closest to 1‐year or prior to Glenn circulation).
Results: Eighty‐one… More >

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Keri M. Shafer^1,2, Alexander R. Opotowsky^1,2, Jonathan Rhodes¹
Congenital Heart Disease, Vol.13, No.6, pp. 903-910, 2018, DOI:10.1111/chd.12661
Abstract Objective: Risk prediction using cardiopulmonary exercise testing (CPET) in complex congenital heart disease tends to either focus on single diagnoses or complete cohorts. We aimed to evaluate patients with two distinct anatomies cared for at a single institution over the same time period to determine CPET variables associated with mortality.
Design: All Fontan and tetralogy of Fallot (TOF) subjects with CPET between November 1, 2002 and December 31, 2014 and subsequently died were identified (cases). Cases were matched 1:3 to controls with similar age, underlying anatomy and timing of exercise test.
Results: Of the 42 cases, 27 had a Fontan… More >

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979

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Fabricio González‐Andrade, Daniel Echeverría, Valeria López, Michaela Arellano
Congenital Heart Disease, Vol.13, No.6, pp. 911-918, 2018, DOI:10.1111/chd.12654
Abstract Objective: To assess the pulse oximetry as a method for screening critical congenital heart disease (CHD) in newborns.
Study design: This is an observational, transversal, descriptive simple study. The pre‐ ductal and postductal saturation were taken in term newborns thatfulfilled the criteria of inclusion and exclusion in the Hospital Gineco‐Obstetrico Isidro Ayora (HGOIA) in Quito. These measurements were performed between the 24 and 48 h after birth. Those new‐ borns that saturated less than 90% on initial pulse oxìmetry underwent 3 successive measurements at 1‐h intervals. Those who saturate less than 90% after 3 measurements or have a difference higher… More >

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944

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Matthew W. Buelow^1,2, Nancy Rudd¹, Jena Tanem¹, Pippa Simpson³, Peter Bartz^1,2, Garick Hill⁴
Congenital Heart Disease, Vol.13, No.6, pp. 919-926, 2018, DOI:10.1111/chd.12655
Abstract Background: Single ventricle heart disease with aortic arch hypoplasia has high mor‐ bidity and mortality, with the greatest risk after stage 1 palliation. Residual lesions often require catheter‐based or surgical reintervention to minimize risk. We sought to describe the types, frequency, and risk factors for re‐intervention between stage 1 and stage 2 palliation, utilizing the National Pediatric Cardiology Quality Improvement Collaborative (NPC‐QIC) registry.
Methods: The NPC‐QIC registry, consisting of patients discharged after stage 1 pal‐ liation, was queried. Hybrid stage 1 palliation patients were excluded from this study. The primary risk factor was shunt type and the primary outcome was… More >

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868

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Martin A. Chacon‐Portillo^1,2, Rodrigo Zea‐Vera^1,2, Huirong Zhu³, Heather A. Dickerson^4,5, Iki Adachi^1,2, Jeffrey S. Heinle^1,2, Charles D. Fraser^1,2, Carlos M. Mery^1,2
Congenital Heart Disease, Vol.13, No.6, pp. 927-934, 2018, DOI:10.1111/chd.12664
Abstract Objective: There are limited studies analyzing pulsatile Glenn as a long‐term pallia‐ tion strategy for single ventricle patients. This study sought to determine their out‐ comes at a single institution.
Design: A retrospective review was performed.
Setting: Study performed at a single pediatric hospital.
Patients: All single ventricle patients who underwent pulsatile Glenn from 1995 to 2016 were included.
Outcome measures: Pulsatile Glenn failure was defined as takedown, transplant, or death. Further palliation was defined as Fontan, 1.5, or biventricular repair. Risk fac‐ tors were assessed by Cox multivariable competing risk analyses.
Results: Seventy‐eight patients underwent pulsatile Glenn at age… More >

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928

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Nikki M. Singh¹, Rohit S. Loomba², Todd M. Gudausky¹, Michael E. Mitchell³
Congenital Heart Disease, Vol.13, No.6, pp. 935-943, 2018, DOI:10.1111/chd.12670
Abstract Introduction: In patients with tetralogy of Fallot (TOF), use of transannular patch (TAP) may be required in order to relieve significant right ventricular outflow tract obstruction, subsequently resulting in pulmonary insufficiency (PI). The monocusp valve has been used to temporarily reduce insufficiency in hopes to improve short and midterm outcomes. The purpose of this study was to assess for potential benefits of the monocusp valve in this subset of patients.
Design: Between 2005 and 2016, 119 patients with TOF with pulmonary stenosis who underwent repair with TAP were analyzed, 43 (36.1%) had a monocusp valve placed. Immediate outcomes were assessed… More >

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Benjamin M. Moore^1,2, Rachael L. Cordina^1,2, Mark A. McGuire^1,2, David S. Celermajer^1,2
Congenital Heart Disease, Vol.13, No.6, pp. 944-951, 2018, DOI:10.1111/chd.12657
Abstract Objective: Amiodarone is a highly effective antiarrhythmic therapy, however its tox‐ icity profile often limits treatment. This is particularly relevant in adults with congeni‐ tal heart disease (CHD), who are often young and in whom other antiarrhythmic agents commonly fail or are contraindicated. We sought to determine incidence and predictors of adverse effects caused by amiodarone in adult CHD (ACHD).
Design: A retrospective review of patients with moderate to complex ACHD treated with amiodarone at our center between 2000 and 2017 was performed. Incidence and predictors of adverse effects were described. Efficacy of amiodarone therapy in controlling the clinical arrhythmia… More >

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882

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Adikan Bhasipol¹, Nopawan Sanjaroensuttikul², Prapaporn Pornsuriyasak³, Sukit Yamwong¹, Tarinee Tangcharoen¹
Congenital Heart Disease, Vol.13, No.6, pp. 952-958, 2018, DOI:10.1111/chd.12659
Abstract Objective: We aimed to study the efficiency and safety of once‐a‐week outpatient rehabilitation followed by home program with tele‐monitoring in patients with com‐ plex cyanotic congenital heart disease.
Design: Prospective nonrandomized study.
Method: Patients who have been diagnosed either Eisenmenger’s syndrome or inop‐ erable complex cyanotic heart disease and able to attend 12‐week cardiac rehabilita‐ tion program were included. Training with treadmill walking and bicycling under supervision at cardiac rehabilitation unit once‐a‐week in the first 6 weeks followed by home‐based exercise program (bicycle and walking) with a target at 40%‐70% of maximum heart rate (HRmax) at pretraining peak exercise for… More >

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885

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Martina Nassif¹, Reindert P. van Steenwijk², Jacqueline M. Hogenhout², Huangling Lu¹, Rianne H.A.C.M. de Bruin‐Bon B Health¹, Alexander Hirsch^1,3, Peter J. Sterk MD, PhD2 | Berto J. Bouma MD, PhD1 | Bart Straver MD, PhD4 | Jan G.P. Tijssen¹, Barbara J.M. Mulder^1,5, Robbert J. de Winter¹
Congenital Heart Disease, Vol.13, No.6, pp. 959-966, 2018, DOI:10.1111/chd.12665
Abstract Objective: The association between secundum atrial septal defects (ASD) and asthma‐ like dyspnea with consequent long‐term pulmonary inhalant use, is poorly understood in adult ASD patients. Airway hyperresponsiveness is suggested to be the underlying mechanism of cardiac asthma from mitral valve disease and ischemic cardiomyopathy. We hypothesized that airway hyperresponsiveness may also be found in adult ASD patients. Our aim was to study airway responsiveness in adult ASD patients before percutaneous closure and at short‐and long‐term postprocedural follow‐up.
Methods: This prospective study included 31 ASD patients (65% female, mean age 49 ± 15y) who underwent spirometry and bronchoprovocation testing pre‐and… More >

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Joshua Saef¹, Sandeep Sodhi¹, Kristen M. Tecson², Vanessa al Rashida¹, Jong Mi Ko², Kamila S. White³, Philip A. Ludbrook¹, Ari M. Cedars⁴
Congenital Heart Disease, Vol.13, No.6, pp. 967-977, 2018, DOI:10.1111/chd.12668
Abstract Objective: Growth in the adults with congenital heart disease (ACHD) population represents a challenge to the health care infrastructure. As patients with chronic disease are increasingly held accountable for their own care, contributors to disease‐ specific health knowledge, which are known to correlate with patients’ participation in care, merit investigation to design patient‐focused interventions.
Design: We conducted a single‐site, cross‐sectional study of ACHD patients. Investigators retrospectively gathered clinical data as well as psychometric and health status assessments completed at the time of enrollment.
Outcome Measures: We investigated the impact of clinical and psychological varia‐ bles on Leuven Knowledge Questionnaire for… More >

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Pablo Marino^1,2, Gabriella de Oliveira Lopes^1,3, Juliana Pereira Borges³, Maria Carolina Terra Cola¹, Daniel Arkader Kopiler¹, Eduardo Tibirica¹
Congenital Heart Disease, Vol.13, No.6, pp. 978-987, 2018, DOI:10.1111/chd.12660
Abstract Objective: Adults with congenital heart disease share some features with those with chronic heart failure. Although microvascular endothelial dysfunction has been de‐ scribed in chronic heart failure, evaluation of the microcirculation in adults with con‐ genital heart disease is lacking. The present study aimed to investigate systemic microvascular reactivity in adults with congenital heart disease.
Interventions: The patients initially underwent cardiopulmonary exercise testing. Then, the cutaneous microvascular reactivity was evaluated in these patients using a laser speckle contrast imaging system coupled with skin iontophoresis of endothelial‐de‐ pendent (acetylcholine) or ‐independent (sodium nitroprusside) vasodilators and pos‐ tocclusive reactive hyperemia (PORH) and… More >

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892

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Lidia R. Bons¹, Lucas Uchoa de Assis¹, Silvy Dekker¹, Robert M. Kauling¹, Judith A.A.E. Cuypers¹, Hence J.M. Verhagen², Ricardo P.J. Budde³, Jolien W. Roos‐Hesselink¹
Congenital Heart Disease, Vol.13, No.6, pp. 988-996, 2018, DOI:10.1111/chd.12663
Abstract Objective: The aim is to present current clinical practice of thoracic aorta screening in a tertiary referral center. We identified how often imaging techniques were used for screening and established the value of transthoracic echocardiography (TTE) in com‐ parison with computed tomography (CT) to detect aortic dilation. We also investigated which additional abnormalities of the heart, aorta or smaller arteries were discovered.
Design: All patients ≥15 years who visited our tertiary center in 2012‐2016 for first thoracic aortic screening were retrospectively included. Diameters of the sinus of Valsalva (SoV) and maximum ascending aorta (AA) were compared between TTE and CT.… More >

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Eliana Al Haddad^1*, Damien J. LaPar^1*, Jeffrey Dayton², Elizabeth H. Stephens¹, Emile Bacha¹
Congenital Heart Disease, Vol.13, No.6, pp. 997-1004, 2018, DOI:10.1111/chd.12666
Abstract Background: Congenital heart defects affect nearly 1% of all children born per year in the United States, and complete atrioventricular canal (CAVC) accounts for 2%‐9%. While several patch materials have been used for septal defect closure during CAVC repair, clear superiority of one material over another has yet to be established.
Methods: A retrospective review of clinical outcomes following CAVC repair at Morgan Stanley Children’s Hospital/Columbia University was performed on opera‐ tions conducted from March 2010 to September 2017. Univariate and Kaplan‐Meir survival analyses were utilized to evaluate primary outcomes of interest following CAVC repair in the modern surgical era.
… More >

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886

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Phong Teck Lee, Felix Maverick Uy, Jie Sheng Foo, Ju Le Tan
Congenital Heart Disease, Vol.13, No.6, pp. 1005-1011, 2018, DOI:10.1111/chd.12667
Abstract Background: Ventricular septal defect (VSD) is one of the most common congenital heart anomalies in childhood and there is an increasing prevalence of VSDs in the adult population. The long‐term risk of infective endocarditis (IE) is of concern. The aim of this study was to clarify and compare the incidence of IE in adults with re‐ paired and unrepaired VSDs.
Methods: Patients with VSDs were identified using the Adult Congenital Heart Disease registry at the National Heart Centre Singapore. Patients were divided into Group 1 (repaired VSD) and Group 2 (unrepaired VSDs). The electronic medical re‐ cords were searched for… More >

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852

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Mohd Nizam Mat Bah¹, Mohd Hanafi Sapian¹, Mohammad Tamim Jamil¹, Nisah Abdullah¹, Emieliyuza Yusnita Alias¹, Norazah Zahari²
Congenital Heart Disease, Vol.13, No.6, pp. 1012-1027, 2018, DOI:10.1111/chd.12672
Abstract Objectives: There is limited data on congenital heart disease (CHD) from the lower‐ and middle‐income country. We aim to study the epidemiology of CHD with the specific objective to estimate the birth prevalence, severity, and its trend over time.
Design: A population‐based study with data retrieved from the Pediatric Cardiology Clinical Information System, a clinical registry of acquired and congenital heart dis‐ ease for children.
Setting: State of Johor, Malaysia.
Patients: All children (0‐12 years of age) born in the state of Johor between January 2006 and December 2015.
Intervention: None.
Outcome measure: The birth prevalence, severity, and temporal trend… More >

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920

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Shahnawaz M. Amdani, Robert D. Ross, Paul A. Webster, Daniel R. Turner, Thomas J. Forbes, Daisuke Kobayashi
Congenital Heart Disease, Vol.13, No.6, pp. 1028-1037, 2018, DOI:10.1111/chd.12677
Abstract Introduction: Reduction of radiation dosage in the pediatric cardiac catheterization laboratory (PCL) is important to reduce the risk of its stochastic effect in children with congenital heart disease. Lowering the frame rate would reduce radiation dos‐ age possibly at the expense of image quality, potentially resulting in higher fluoro‐ scopic time and procedural complication rate.
Methods: The data were retrospectively analyzed in three eras: era 1 (n = 234), cine‐ angiography 30 frames/sec (f/s) and fluoroscopy 15 pulse/sec (p/s); era 2 (n = 381), cineangiography 30 f/s and fluoroscopy 6 p/s; and era 3 (n = 328), cineangiography 15 f/s… More >

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932

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Nurdan Ozturk Tasar¹, Pelin Kosger², Nevzat Uzuner³, Birsen Ucar²
Congenital Heart Disease, Vol.13, No.6, pp. 1038-1044, 2018, DOI:10.1111/chd.12678
Abstract Objective: We aimed to investigate the effect of ventricular septal defect (VSD) and heart failure on cerebral blood flow (CBF) in children, whether heart failure treatment improves CBF, and if there is any relationship between CBF and serum N‐ terminal pro‐brain natriuretic peptide (NT‐proBNP) level.
Method: Forty children with VSD (13 with heart failure) aged between 1 and 36 months were studied. The control group comprised 25 healthy children in the same age group. Maximum, minimum, and mean blood flow velocities and pulsatility indices of the right and left middle cerebral arteries were assessed using transcranial Doppler ultrasonography. Measurements of… More >

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976

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Shelby C. White¹, Jennifer Sedler², Trahern W. Jones³, Michael Seckeler¹
Congenital Heart Disease, Vol.13, No.6, pp. 1045-1049, 2018, DOI:10.1111/chd.12673
Abstract Objective: Applications of three‐dimensional (3D) printed models in medicine in‐ clude preprocedure planning, patient education, and clinical training. Reproducing complex anatomy as a 3D printed model can be useful for understanding congenital heart defects (CHD). We hypothesized that using 3D printed models during didactic sessions with resident physicians will improve trainees’ understanding of CHD.
Design and intervention: We performed a prospective, randomized educational in‐ tervention for teaching pediatric and pediatric/emergency medicine residents about simple (ventricular septal defect [VSD]) and moderately complex (tetralogy of Fallot [ToF]) CHD. Residents were divided into two groups: intervention and control. Each group completed a subjective… More >

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972

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Shae Anderson^1,2, Courtney E. McCracken², Ritu Sachdeva^1,2
Congenital Heart Disease, Vol.13, No.6, pp. 1050-1057, 2018, DOI:10.1111/chd.12679
Abstract Objective: The educational intervention (EI) through the Pediatric Appropriate Use of Echocardiography (PAUSE) multicenter study resulted in improved appropriate‐ ness of transthoracic echocardiogram (TTE) orders at our center. The current study evaluated if this pattern persisted after cessation of EI and the potential physician characteristics influencing appropriateness.
Design: Outpatients (≤18 years old) seen for initial evaluation during the EI (July to October, 2015) and 6‐month post‐EI (May to August, 2016) phases were included. Comparison was made between TTE rates and appropriateness ratings during EI and post‐EI phase. Association between TTE rate and appropriateness with physician characteristics (age, experience, patient volume,… More >

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874

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Soham Dasgupta¹, Megan Stark², Sonal Bhatt³, Peter Fischbach¹, Shriprasad Deshpande¹
Congenital Heart Disease, Vol.13, No.6, pp. 1058-1063, 2018, DOI:10.1111/chd.12681
Abstract Introduction: A cardiac cause is an extremely rare etiology of pediatric chest pain. Despite its low sensitivity/specificity, exercise stress testing (EST) is widely used to determine the prognosis in patients with suspected/established coronary disease. We aimed to look at the utility of a combined cardiopulmonary EST in the evaluation of pediatric patients with chest pain.
Methods: After institutional review board approval, a retrospective chart review was performed of all pediatric patients who were referred for an EST for chest pain from January 2014 to 2017. Patients with incomplete records, severe congenital heart disease, and a prior EST were excluded.
Results:… More >

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841

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Christian Paech, Franziska Wagner, Sebastian Mensch, Roman Antonin Gebauer
Congenital Heart Disease, Vol.13, No.6, pp. 1064-1068, 2018, DOI:10.1111/chd.12682
Abstract Introduction: Reflex vasovagal—or cardioinhibitory syncope is known to be a major cause of recurrent syncope in children. The mechanism of vasovagal syncope (VVS) is an interaction between a vagally mediated bradycardia or asystole and a more or less manifest vasodilatory component. Although pacing is not advisable as a standard approach in patients with VVS, it remains a treatment option of last resort in excep‐ tionally severe cases, or patients with contraindication or refractoriness to drug ther‐ apy and life style changes. To effectively avoid VVS in these patients, the pacemaker has to both prevent bradycardia and to compensate for the… More >

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853

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Carlos E. B. Branco¹, Roney O. Sampaio¹, Flavio Tarasoutchi¹, Justin P. Zachariah²
Congenital Heart Disease, Vol.13, No.6, pp. 1069-1071, 2018, DOI:10.1111/chd.12676
Abstract In the 2017, “Cairo Accord on Rheumatic Heart Disease—From Molecules to The Global Community” experts from endemic areas enumerated an approach to reduce the population burden of rheumatic heart disease. The 10 key recommendations in‐ clude immediate logistical objectives as well as domains for further study. Echocardiographic population screening programs were relegated to research alone. Given the large body of supporting data, relegating echo screening to an investiga‐ tional modality is an opportunity lost. More >

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