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 >
Open Access
ARTICLE
Robin H.S. Chen1, Adolphus K.T. Chau1, Pak Cheong Chow1, Tak Cheung Yung1, Yiu Fai Cheung1,2, 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 >
Open Access
ARTICLE
Shiraz A. Maskatia1, Christopher J. Petit2,3, Curtis D. Travers2, David J. Goldberg4, Lindsay S. Rogers4, Andrew C. Glatz4, Athar M. Qureshi6, Bryan H. Goldstein5, Jingning Ao2,3, Ritu Sachdeva2,3
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 >
Open Access
ARTICLE
Keri M. Shafer1,2, Alexander R. Opotowsky1,2, 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 >
Open Access
ARTICLE
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 >
Open Access
ARTICLE
Matthew W. Buelow1,2, Nancy Rudd1, Jena Tanem1, Pippa Simpson3, Peter Bartz1,2, 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 >
Open Access
ARTICLE
Martin A. Chacon‐Portillo1,2, Rodrigo Zea‐Vera1,2, Huirong Zhu3, Heather A. Dickerson4,5, Iki Adachi1,2, Jeffrey S. Heinle1,2, Charles D. Fraser1,2, Carlos M. Mery1,2
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 >
Open Access
ARTICLE
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 >
Open Access
ARTICLE
Benjamin M. Moore1,2, Rachael L. Cordina1,2, Mark A. McGuire1,2, David S. Celermajer1,2
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 >
Open Access
ARTICLE
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 >
Open Access
ARTICLE
Martina Nassif1, Reindert P. van Steenwijk2, Jacqueline M. Hogenhout2, Huangling Lu1, Rianne H.A.C.M. de Bruin‐Bon B Health1, Alexander
Hirsch1,3, Peter J. Sterk MD, PhD2 | Berto J. Bouma MD, PhD1 | Bart Straver
MD, PhD4 | Jan G.P. Tijssen1, Barbara J.M. Mulder1,5, 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 >
Open Access
ARTICLE
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 >
Open Access
ARTICLE
Pablo Marino1,2, Gabriella de Oliveira Lopes1,3, 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 >
Open Access
ARTICLE
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 >
Open Access
ARTICLE
Eliana Al Haddad1*, Damien J. LaPar1*, 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 >
Open Access
ARTICLE
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 >
Open Access
ARTICLE
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 >
Open Access
ARTICLE
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 >
Open Access
ARTICLE
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 >
Open Access
ARTICLE
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 >
Open Access
ARTICLE
Shae Anderson1,2, Courtney E. McCracken2, Ritu Sachdeva1,2
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 >
Open Access
ARTICLE
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 >
Open Access
ARTICLE
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 >
Open Access
ARTICLE
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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