Congenital Heart Disease

Publisher: Wiley

Journal description

Congenital Heart Disease: Clinical Studies from Fetus to Adulthood is a new, clinical journal focusing on congenital heart disease in children and adults. Though the number of infants born with heart disease each year is relatively small (approximately 1% of the population), advances in the treatment of such malformations have led to increased life spans for this population. Consequently, in the United States today most patients treated for congenital heart disease are over the age of 20. What are the special needs of adults with congenital heart disease? What are the latest developments in the care of the fetus, infants, and children? Who should treat these patients? How should they be treated? Congenital Heart Disease focuses on these questions and more. Conceived as a forum for the most up-to-date information on congenital heart disease, the journal is led by Editor-in-Chief Douglas S. Moodie, MD, Chairman of the Department of Pediatrics at Ochsner Clinic in New Orleans, as well as an international editorial board. Congenital Heart Disease publishes articles on heart disease as it relates to the following areas: Clinical pediatric and adult cardiology; Cardiac imaging; Preventive cardiology; Diagnostic and interventional cardiac catheterization; Electrophysiology; Surgery; Long-term follow-up, particularly as it relates to older children and adult congenital heart disease; Exercise and exercise physiology in the congenital patient; Post-op and critical care; Common disorders such as syncope, chest pain, murmurs, as well as acquired disorders such as Kawasaki syndrome. The journal includes clinical studies, invited editorials, state-of-the-art reviews, case reports, articles focusing on the history and development of congenital heart disease, and CME material. Occasional issues focus on special topics. Congenital Heart Disease was created for pediatric cardiologists; adult cardiologists who care for patients with congenital heart disease; pediatric and pediatric cardiology nurses; surgeons; radiologists; anesthesiologists; critical care physicians and nurses; and adult support staff involved in the care of patients with congenital heart disease.

Current impact factor: 1.20

Impact Factor Rankings

2015 Impact Factor Available summer 2015
2013 / 2014 Impact Factor 1.202
2012 Impact Factor 1.013
2011 Impact Factor 0.901

Impact factor over time

Impact factor

Additional details

5-year impact 0.00
Cited half-life 3.30
Immediacy index 0.14
Eigenfactor 0.00
Article influence 0.00
Website Congenital Heart Disease website
Other titles Congenital heart disease (Online)
ISSN 1747-0803
OCLC 76166284
Material type Document, Periodical, Internet resource
Document type Internet Resource, Computer File, Journal / Magazine / Newspaper

Publisher details


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    • Publisher last contacted on 07/08/2014
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  • Classification
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Publications in this journal

  • [Show abstract] [Hide abstract]
    ABSTRACT: Left axis deviation (LAD) on the electrocardiogram (ECG) is associated with congenital heart disease (CHD), prompting the clinician to order further testing when evaluating a patient with this finding. The purpose is to (1) compare the physical examination (PE) by a pediatric cardiologist to echocardiogram (ECHO) findings in patients with LAD on resting ECG and (2) assess cost of performing ECHO on all patients with LAD on ECG. An IRB approved, retrospective cohort study was performed on patients with LAD (QRS axis ≥0° to -90°) on ECG between 01/02 and 12/12. age >0.25 and <18 years, non-postoperative, and PE and ECHO by pediatric cardiologist. A decision tree model analyzed cost of ECHO in patients with LAD and normal/abnormal PE. Cost of complete ECHO ($239.00) was obtained from 2014 Medicare reimbursement rates. A total of 146 patients met inclusion criteria with 46.5% (68) having normal PE and ECHO, 1.4% (2) having normal PE and abnormal ECHO, 47.3% (69) having abnormal PE and ECHO, and 4.8% (7) having an abnormal PE and normal ECHO. Sensitivity and specificity of PE for detecting abnormalities in this population was 97% and 90%. Positive and negative predictive value of PE was 91% and 97.5%. In patients with normal PE, the cost to identify an ECHO abnormality was $8365, and $263 for those with abnormal PE. In presence of LAD on ECG, the sensitivity, specificity, and positive and negative predictive values of PE by a pediatric cardiologist are excellent at identifying CHD. Performing an ECHO on patients with LAD on ECG is only cost effective in the presence of an abnormal PE. In the presence of normal PE, there is a possibility of missing incidental structural cardiac disease in approximately 2% if an ECHO is not performed. © 2015 Wiley Periodicals, Inc.
    Congenital Heart Disease 08/2015; DOI:10.1111/chd.12294
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    ABSTRACT: Critically ill children with congenital heart disease (CHD) are at risk for metabolic bone disease (MBD) and bone fractures. Our objective was to characterize a cohort of CHD patients with fractures and describe a Fragile Bone Protocol (FBP) developed to reduce fractures. Patients who developed fractures in the Cardiac Intensive Care Unit (CICU) of Boston Children's Hospital from 3/2008 to 6/2014 were identified via quality improvement and radiology databases. The FBP (initiated July 2011) systematically identifies patients at risk for MBD and prescribes special handling precautions. Twenty-three fractures were identified in 15 children. Median age at fracture identification was 6.2 months, with a median duration of hospitalization before fracture diagnosis of 2.7 months. Six patients (40%) had single ventricle CHD. Hyperparathyroidism and low 25-OH vitamin D levels were present in 77% and 40% of those tested, respectively. Compared with patients not diagnosed with fractures, fracture patients had increased exposure to possible risk factors for MBD and had elevated parathyroid and decreased calcitriol levels.Six patients (40%) did not survive to hospital discharge, compared with an overall CICU mortality rate of 2.6% (P < .01). The fracture case rate before implementation of the FBP was 2.6 cases/1000 admissions and was 0.7/1000 after implementation of the FBP (P = .04). Critically ill CHD patients are at risk for fractures. They represent a complex group who frequently has hyperparathyroidism and decreased calcitriol levels, and each may predispose to fractures. FBPs consisting of identification and careful patient handling should be considered in at-risk patients. © 2015 Wiley Periodicals, Inc.
    Congenital Heart Disease 08/2015; DOI:10.1111/chd.12293
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    ABSTRACT: In order to determine the feasibility of tracking quality of care in adults with congenital heart disease (ACHD), we aimed to estimate the availability of relevant data in electronic medical records (EMR) used in North American ACHD centers. Previously proposed quality indicators (QIs) were reviewed to consider what types of data would be required for each. ACHD program directors were surveyed about the nature of electronic data in existing EMRs. From the survey, the availability of data types needed for the denominator and numerator of each QI were estimated, and an overall estimate of data availability was calculated for each QI. These estimates were adjusted by the sensitivity of identifying the patients through administrative codes. Analysis was repeated for scenarios in which various data type estimates were hypothetically dropped by half to determine the overall impact of each data type. A total of 64 ACHD program directors responded to the survey. Of 55 QIs, average estimated data availability was 67%. QIs for tetralogy of Fallot had the highest estimated data availability (mean 88%), whereas those for atrial septal defect were lowest (mean 23%), reflecting both the need for interpretation of imaging studies and the lower reliability of billing codes for identification of ACHD patients. QIs with highest estimates were based largely on administrative data, which had the biggest impact on overall estimates. QIs needing interpretation of imaging findings had the lowest estimates, as well as certain overuse measures. For a wide range of ACHD programs, data for proposed QIs based on administrative data are most likely to be obtainable through EMR. Data related to imaging interpretation or overuse measures are least likely. Our findings can inform future efforts to establish registry efforts or data reporting tools to track these indicators. © 2015 Wiley Periodicals, Inc.
    Congenital Heart Disease 08/2015; DOI:10.1111/chd.12289
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    ABSTRACT: The objective of this study was to characterize the natural history of metabolic uncoupling (type B hyperlactemia and hyperglycemia) following cardiopulmonary bypass (CPB), and to determine the impact of insulin therapy on time to lactate normalization in patients without low cardiac output. The design used was a retrospective cohort study. The study was set in a pediatric cardiac intensive care unit in a tertiary-care urban children's hospital. All patients were aged ≤21 years admitted between 2007 and 2013 following cardiac surgery involving CPB with empiric intraoperative corticosteroids. Eligibility criteria: simultaneous hyperlactemia (≥3.5 mEq/L) and hyperglycemia (≥200 mg/dL) within 48 hours after bypass. Exclusion criteria were evidence of low cardiac output state, diabetes or postoperative steroid administration. Characteristics were compared between those treated with insulin and those who were not (controls). Outcome measures used were time from admission to onset of hyperglycemia and hyperlactemia and time to resolution. Clinical outcomes included duration of mechanical ventilation, length of stay, unplanned readmission/reoperation, hypoglycemia and death. Of the 1345 patients receiving CPB, 132 (9.8%) met inclusion criteria. Seventy-eight (59%) were treated with insulin, leaving 54 controls. Patient characteristics, surgical complexity and time to onset of hyperglycemia and hyperlactemia were similar between groups. The insulin group had a shorter duration of hyperglycemia. There was no significant difference between groups in time to lactate normalization, ventilator days, length of stay, readmission and reoperation rates. Hypoglycemia (<60 mg/dL) occurred in three patients. In children with metabolic uncoupling after CPB, insulin use did not shorten the time to lactate normalization or alter clinical outcomes. These findings suggest that type B hyperlactemia with hyperglycemia after CPB will resolve spontaneously and does not warrant specific treatment. © 2015 Wiley Periodicals, Inc.
    Congenital Heart Disease 07/2015; DOI:10.1111/chd.12285
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    ABSTRACT: Understanding value provides an important context for improvement. However, most health care models fail to measure value. Our objective was to categorize inpatient encounters within an academic congenital heart program based on clinical outcome and the cost to achieve the outcome (value). We aimed to describe clinical and nonclinical features associated with value. We defined hospital encounters based on outcome per resource utilized. We performed principal component and cluster analysis to classify encounters based on mortality, length of stay, hospital cost and revenue into six classes. We used nearest shrunken centroid to identify discriminant features associated with the cluster-derived classes. These features underwent hierarchical clustering and multivariate analysis to identify features associated with each class. We analyzed all patients admitted to an academic congenital heart program between September 1, 2009, and December 31, 2012. A total of 2658 encounters occurred during the study period. Six classes were categorized by value. Low-performing value classes were associated with greater institutional reward; however, encounters with higher-performing value were associated with a loss in profitability. Encounters that included insertion of a pediatric ventricular assist device (log OR 2.5 [95% CI, 1.78 to 3.43]) and acquisition of a hospital-acquired infection (log OR 1.42 [95% CI, 0.99 to 1.87]) were risk factors for inferior health care value. Among the patients in our study, institutional reward was not associated with value. We describe a framework to target quality improvement and resource management efforts that can benefit patients, institutions, and payers alike. © 2015 Wiley Periodicals, Inc.
    Congenital Heart Disease 07/2015; DOI:10.1111/chd.12290
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    ABSTRACT: So-called heterotaxy affects lateralization of the thoracic and abdominal organs. Congenital malformations may be present in one of several organ systems. Cardiac involvement includes both structural and conduction abnormalities. Data regarding arrhythmias in heterotaxy come from case reports and small case series. We pooled available data to further characterize arrhythmias in heterotaxy. A systematic review of the literature for manuscripts describing arrhythmias in heterotaxy patients was conducted. Databases including PubMed, EMBASE, and Ovid were searched. Studies describing arrhythmias in patients with heterotaxy were included if they were in English and presented characteristics of the arrhythmias. Arrhythmia characteristics were abstracted and are presented as pooled data. Freedom from arrhythmia by age was then analyzed using Kaplan-Meier analysis. A total of 19 studies with 121 patients were included in the pooled analysis. Those with right isomerism were found to be more likely to have atrial flutter, atrial tachycardia, junctional tachycardia, and ventricular tachycardia. Those with left isomerism were more likely to have atrioventricular block, intraventricular conduction delay, sick sinus syndrome, and atrioventricular nodal reentry tachycardia. Median age of onset for all arrhythmias was 4 years with no difference by specific arrhythmia or isomerism. Those with right and left isomerism are at risk for different arrhythmias but are likely to develop arrhythmias at the same age. Those with left isomerism are more likely to require pacemaker placement due to atrioventricular block. Understanding these differences allows for focused surveillance of development of these arrhythmias. © 2015 Wiley Periodicals, Inc.
    Congenital Heart Disease 07/2015; DOI:10.1111/chd.12288
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    ABSTRACT: Initial palliative treatment of congenital aortic valve stenosis consists of either surgical commissurotomy or transcatheter balloon valvuloplasty. The basis for selection of primary approach usually is ill defined. It was hypothesized that aortic valve characteristics may be helpful in directing choice of intervention. This study assesses the impact of valve morphology on time to reintervention after catheter- or surgical-based therapy. A retrospective review of preprocedural echocardiographic aortic outflow characteristics was compared with outcomes of primary surgical or balloon valve interventions. Characteristics studied included (1) annular dimension; (2) leaflet number; (3) leaflet excursion; and (4) degree of leaflet coaptation. Patients included those <20 years with a primary diagnosis of aortic stenosis (AS) and no other hemodynamically significant lesions at our institutions from 2000 to 2011. A total of 102 patients were included, 31 classified as having critical and 71 as having noncritical aortic stenosis. Of the patients, 79 were male, and 50 underwent primary catheter intervention. Echo parameters were compared with procedural outcome as defined by death or need for reintervention. Receiver operator curves were utilized to determine the point within each morphologic feature where the greatest difference occurred. This was utilized as the distinguishing point within each the morphologic group. Analysis was conducted separately for critical and noncritical aortic stenosis. Kaplan-Meier analysis demonstrated no significant difference in time to reintervention or death whether initial palliation consisted of surgical commissurotomy or balloon valvuloplasty with respect to any of the morphologic characteristics studied. Patients with AS do equally well with surgical commissurotomy or balloon valvuloplasty as initial palliation. This holds true for those with either critical or noncritical aortic stenosis. Valve morphology did not help in selection of initial palliative strategy. Current technologies should enable an improved selection of initial palliative approach through thoughtful, randomized trials. © 2015 Wiley Periodicals, Inc.
    Congenital Heart Disease 07/2015; DOI:10.1111/chd.12278
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    ABSTRACT: While well characterized in adult patients, the pattern of hypertrophy and the extent of myocardial scarring in hypertrophic cardiomyopathy (HCM) are insufficiently known. The aim of this study was to assess the hypertrophy patterns and the prevalence and clinical significance of scars in the hearts of young patients with HCM. A retrospective analysis of the imaging findings of 38 children (aged 12.83 ± 2 years, 30 males) with HCM who underwent cardiac magnetic resonance imaging (CMR) was performed. In addition to left ventricular mass and volumes, the examinations were assessed for the pattern of hypertrophy and presence of late gadolinium enhancement (LGE). A myocardial signal intensity ≥6 standard deviations above the mean of normal myocardium defined positive LGE. Left ventricular mass index averaged 110 ± 34 g/m(2) . Nineteen children (50%) had diffuse septal, 13 (34%) diffuse concentric and 6 (16%) isolated basal hypertrophy. Seven children (18%) had LGE. Patients with LGE had a greater left ventricular mass index than those without (136 ± 34 g/m(2) vs. 104 ± 31 g/m(2) , P = .025). The only two patients who presented with an episode of aborted sudden cardiac death had LGE (P = .03). The most common hypertrophy pattern in children with HCM was diffuse septal hypertrophy. The incidence of LGE observed is lower than that reported in adults. The presence of LGE appears to confer a risk for adverse events. © 2015 Wiley Periodicals, Inc.
    Congenital Heart Disease 07/2015; DOI:10.1111/chd.12286
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    ABSTRACT: A recent review supports a strategy of deferring treatment of patent ductus arteriosus (PDA) in the preterm neonate until at least the second week after birth. In light of previous suggestion that later initiation of treatment may be less efficacious for closing PDAs it is reasonable to question if delayed treatment may be less effective. We conducted a single center retrospective review of a neonatal intensive care unit database of infants ≤37 weeks gestation with the diagnosis of PDA and treated with indomethacin from 1999 to 2007. We determined gestational age (GA), timing of indomethacin initiation, and status of the PDA at hospital discharge. Treatment failure was defined as neonates requiring further intervention to close their PDA or those who died without echo-proven PDA closure. Of the 341 infants meeting the study criteria, 77 (23%) had defined treatment failure. The failure group had a younger median GA of 25 weeks (interquartile range [IQR], 24-26) vs. 28 weeks (IQR, 26-30) for the successful group (P < .0001). The failure group had a median treatment initiation on day of life (DOL) 4 (IQR, 1-8) compared with DOL 3 (IQR, 1-6) for those in the successful group (P = .15). Taken as a whole, infants treated after DOL 5 were significantly more likely to have treatment failure (30.1% vs. 19.3% for those treated DOL 1-5, P = .03). Our study confirms that younger GA at birth is correlated with increased likelihood of failed PDA closure. We also show a trend indicating that later initiation of treatment may decrease the chances of successfully closing a PDA. Future examination of PDA management should consider the potential unintended consequences that may accompany a delayed treatment strategy. © 2015 Wiley Periodicals, Inc.
    Congenital Heart Disease 07/2015; DOI:10.1111/chd.12287
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    ABSTRACT: The purpose of this study was to explore the transition process in congenital heart disease (CHD) care through the perceived needs and concerns of adolescents (pretransition) and the experiential insight from adults (post-transition), in order to inform future transition initiatives and information delivery methods. In this cross-sectional study, adolescents and adults with moderate or complex CHD participated in semistructured telephone interviews. Interview transcripts were coded using NVivo qualitative data software. Single large urban tertiary care CHD center PATIENTS: Adolescents (between 16 and 20 years of age) and adults (21-40 years of age) with moderate or complex CHD. Patients with global developmental delay or known chromosomal abnormalities were excluded. Twenty adolescents and 20 adults participated in the study; each interview allowed for participants to discuss issues relevant to them. Among adolescents, half wanted more CHD information and half were concerned about becoming adults with CHD. All adolescents had access to the Internet via a mobile phone, but only 55% had searched online for CHD information. Adolescents were interested in mentorship with other adolescents (90%) and adults (60%) with CHD either in person or via protected social media. Among adults, 55% were satisfied with the amount of information they had received as adolescents. Areas in which adults would have preferred additional information include longer-term implications of CHD, employment, insurance, family planning, and mental health. There are similarities and differences in the perceived transition needs of a diverse group of adolescents and adults with CHD. Both cohorts desire additional information during the transition process, including interest in use of a transition checklist to learn about managing their CHD. Adolescents are interested in interactions with other teens and adult mentors with CHD and are open to Internet-based interventions. Web-based initiatives could deliver CHD information and provide a platform for social media mentorship. © 2015 Wiley Periodicals, Inc.
    Congenital Heart Disease 07/2015; DOI:10.1111/chd.12283
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    ABSTRACT: Percutaneous closure of perimembranous ventricular septal defect is one of the greatest challenges in interventional cardiology. The long-term follow-up data are still limited. This report presents our experience in percutaneous closure of perimembranous ventricular septal defect with modified double-disk occluder. The study is a retrospective analysis of cases from the Changhai Hospital clinic database. We reviewed the clinic database from December 2001 to December 2005. We included 117 patients who underwent percutaneous closure of perimembranous ventricular septal defects. Procedural data and complications were assessed by an expert panel of cardiologists. Device implantation was successfully accomplished in 113 of a total 117 patients (96.6%). The mean age was 31.1 ± 12.5 years (range 2-56 years). The patients were followed for a period of 86-134 months. The diameter of defect was 2-15 mm (mean 4.9 ± 2.9 mm). The ventricular septal defect rim below the aortic valve was 1-5 mm. The mean diameter of device used was 6.9 ± 3.7 (range 4-24 mm). Four patients presented complete atrio-ventricular block within 1 week after the procedure and recovered within 3 weeks. No other patient encountered serious adverse events during the follow-up. Percutaneous closure of ventricular septal defect is safe and effective in most selected patients with modified double-disk occluder. Additional studies with larger cohorts and longer follow-up are needed to evaluate its safety. © 2015 Wiley Periodicals, Inc.
    Congenital Heart Disease 07/2015; DOI:10.1111/chd.12284
  • Congenital Heart Disease 07/2015; 10(4):291. DOI:10.1111/chd.12292
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    ABSTRACT: Individuals with L-transposition of the great arteries and two normally sized ventricles are at risk for complete heart block. Little is known about the incidence of complete heart block in those with a single ventricle L-transposition of the great arteries. In this study, we compare the incidence of complete heart block in a modern cohort of patients with L-looped single ventricle anatomy to patients with L-transposition of the great arteries and two ventricles. We conducted a retrospective cohort study of patients with L-transposition of the great arteries who were seen at Yale-New Haven Hospital between 2001 and 2013. Patients were classified as having isolated L-transposition of the great arteries (group I), L-transposition of the great arteries and major cardiac defects with two-ventricle anatomy (group II), or L-transposition of the great arteries and single ventricle anatomy (group III). We recorded the age of onset and the circumstances of CHB in each group. We calculated the incidence rate of complete heart block and compared this between the groups. We identified 64 patients with L-transposition of the great arteries, median age of 21 years (range 6 months-52 years): 21 in group I, 15 in group II, and 28 in group III. In total, 15 subjects developed complete heart block, incidence of 21.9% and rate of 1.3% per person years. Although group III patients were significantly less likely to develop complete heart block than dual ventricle patients (7.1% vs. 33% and 40%, P = .01), this difference is not significant when only spontaneous complete heart block was analyzed (P = .16). All patients with L-transposition of the great arteries have similar risk of spontaneous complete heart block and should be routinely screened for this complication. © 2015 Wiley Periodicals, Inc.
    Congenital Heart Disease 06/2015; DOI:10.1111/chd.12279
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    ABSTRACT: Hypertrophic cardiomyopathy (HCM) is the most common heritable cardiovascular disease and a leading cause of identifiable sudden cardiac death (SCD) in the young. Herein, we sought to determine the genotype-phenotype correlations in a cohort of unrelated, genotyped patients diagnosed with HCM at a young age, as well as to characterize the differences between HCM diagnosed in adulthood and HCM diagnosed at a young age. From 1999 to 2011, 1053 unrelated patients diagnosed with HCM were enrolled in research-based genetic testing. The electronic medical record was reviewed to identify those with HCM diagnosed at ≤21 years (N = 137, mean age at diagnosis 13.2 ± 6 years, 64% male). From this cohort of patients recruited from a tertiary care referral center, the genetic test was positive in 71 (52%), which was significantly higher than patients diagnosed >21 years (31%; P < .001). Genotype-positive patients had increased maximum left ventricular wall thickness (24.9 ± 8.0 vs. 21.6 ± 7.4 mm, P = .01) and higher incidence of reverse-curve ventricular septal morphology (71% vs. 40%, P < .001). Unrelated to genotype status, 26/137 patients (19%) experienced significant HCM-related morbidity/mortality including progressive heart failure symptoms in 12, transplantation in 4, and death in 10. Among patients diagnosed with HCM during the first two decades of life, the yield of genetic testing is significantly higher than when diagnosed at later age. While the phenotype of young HCM patients is worse than patients whose HCM is diagnosed at later age, the phenotypes of genotype-positive and genotype-negative young patients were similar. Independent of genotype, nearly 30% of the patients with follow-up in this study had symptom progression, transplant, or death. © 2015 Wiley Periodicals, Inc.
    Congenital Heart Disease 06/2015; DOI:10.1111/chd.12280
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    ABSTRACT: Background Recent advances in various disciplines of medicine have significantly changed the courses following cardiac surgery in children. On-table extubation (OTE) after open heart surgery in children is evolving.Objective To assess the rate of postoperative complications in children extubated on table after open heart surgery.DesignThis is a retrospective, descriptive study.SettingOperating room (OR) then admitted to the pediatric intensive care unit (PICU).PatientsAll pediatric patients (between 0 and 18 years) undergoing open heart surgery between January 2011 and June 2013.InterventionOn-table extubation.Outcome MeasuresRates of immediate postoperative complications, i.e., re-intubation, significant bleeding, low cardiac output syndrome, and arrhythmia in PICU, were assessed. Data are presented as frequencies and mean ± standard deviation.ResultsA total of 82 patients were included. Mean age at time of operation was 7.25 ± 6.6 years. Fifty-three percent (n = 44) were <5 years old and 64% (n = 53) were men. Ventricular septal defect (47%, n = 39) was the most common lesion, followed by atrial septal defect (36%, n = 30), and tetralogy of Fallot (15%, n = 12), which were repaired. Cardiopulmonary bypass and aortic cross clamp time were 72.3 ± 34.2 and 47.3 ± 27.8 minutes, respectively. The mean inotrope score was 2.66 ± 3.53. There was no mortality in the cohort, whereas 97.8% (n = 80) had no complications during PICU stay. One patient (1.1%) required re-intubation for respiratory failure and one patient (1.1%) had arrhythmia that was medically managed. The mean length of PICU stay was 1.77 ± 0.985 days.Conclusion On-table extubation in children after open heart surgery was feasible and safe in selected group of patients. There was no major complication observed in the PICU.
    Congenital Heart Disease 06/2015; DOI:10.1111/chd.12277
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    ABSTRACT: Epidemiologic studies have reported conflicting results regarding maternal alcohol consumption before and during pregnancy, and the risk of congenital heart defects (CHDs). However, a systematic review and meta-analysis of the association between maternal alcohol consumption and CHDs in offspring has not been conducted. We searched MEDLINE and EMBASE for articles catalogued between their inception and February 16, 2015; we identified relevant published studies that assessed the association between maternal alcohol consumption and CHD risk. Two authors independently assessed the eligibility of the retrieved articles and extracted data from them. Study-specific relative risk estimates were pooled by random-effects or fixed-effects models. From the 1527 references, a total of 19 case-control studies and four cohort studies were enrolled in this meta-analysis. The summary of 23 studies related to CHDs indicated an overall pooled relative risk of 1.13 (95% confidence interval: 0.96, 1.29) among mothers drinking before or during pregnancy. Statistically significant heterogeneity was detected (Q = 196.61, P < .001, I(2) = 88.8%) with no publication bias (Egger's test: P = .157). We conducted stratified and meta-regression analyses to identify the origin of the heterogeneity among studies. In summary, this meta-analysis provided no positive association between maternal alcohol consumption and risk of CHDs. © 2015 Wiley Periodicals, Inc.
    Congenital Heart Disease 06/2015; DOI:10.1111/chd.12271
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    ABSTRACT: Objective Gastrostomy tube (G-tube) placement during three-stage surgical palliation of single-ventricle cardiac physiology has been shown to improve weight gain in this population of infants who often suffer from inadequate feeding. The optimal timing of this intervention is unclear and requires further investigation.DesignA retrospective review of all patients who underwent G-tube placement at any stage of surgical palliation of single-ventricle physiology from January 2005 to December 2012 was performed at a single congenital cardiac surgery center. Analysis of weight gain and survival was undertaken by comparing patients who received the G-tube either less than or greater than 90 days after the first surgical stage.ResultsFifty-four patients were identified that met the criteria, 26 (48%) of which received the G-tube within 90 days of stage 1, while 28 (52%) patients received the tube at greater than 90 days. Percentage of weight gain at time of discharge from stage 1 was significantly higher for group B (A: median 9.9%, interquartile range [IQR] 4.9–29.8; B: median 29.0%, IQR 16.0–44.3; P = .05). However, total hospital length of stay was decreased for the patients who received G-tubes earlier (A: median 60 days, IQR 35–100; B: median 83, IQR 48–184) as was intensive care unit length of stay (A: median 27 days, IQR 13–69; B: median 48, IQR 16–119) by nearly half, although not statistically significant (P = .47). Survival to time of discharge from stage 1 surgery was not significantly different between earlier tube placements vs. later (92% vs. 100%, respectively; P = .14). Multivariable analysis found inclusion of fundoplication to predict weight gain (P = .006) at time of first discharge.Conclusion Earlier placement of G-tube may increase the rate of recovery from stage 1 of multistage palliative cardiac surgery for single-ventricle physiology. Fundoplication may improve perioperative weight gain when indicated.
    Congenital Heart Disease 06/2015; DOI:10.1111/chd.12272
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    ABSTRACT: Objective Nurse practitioners (NPs) have an established role for delivering competent care to patients in the primary care setting. The aim of this study was to compare satisfaction of patients managed by NPs vs. physicians in the outpatient adult congenital heart disease (CHD) clinic.DesignA prospective study conducted in two outpatient adult CHD clinics to assess patient satisfaction through standardized surveys. Demographic data included CHD diagnosis, age, and New York Heart Association functional class. All patients completed a Short-Form-12 to assess health status.ResultsOf the 371 patients recruited (52% men; median age 29 years, severe CHD complexity 31.5%) and seen by NP (n = 187) or physician (n = 184), physician-managed practices had higher perceived: overall experience, courtesy of provider (P < 0.05) and confidence (trust) in provider (P < 0.1). Overall, patients reported satisfaction with an NP providing care (98%), the NP was able to effectively deal with illness/CHD (95%), and an increased chance (94%) or willingness to see an NP at a future visit. Only 73% reported an understanding of NP training and how an NP differed from a registered nurse. There was a stronger perception of how an NP differed from a physician (83%).Conclusion Patient satisfaction was high regardless of whether care was provided by physicians or NPs. However, patients appear to make distinctions in what they believe the type of care each practitioner is best at providing. Patient education regarding competence of the different health care providers may continue to improve patient satisfaction.
    Congenital Heart Disease 06/2015; 63(12). DOI:10.1111/chd.12273
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    ABSTRACT: Background We aimed to determine the correlation between noninvasive testing (exercise stress testing [EST] and adenosine responsiveness of accessory pathway [AP] ) and invasive electrophysiology study (EPS) for assessment antegrade conduction of the AP in Wolff–Parkinson–White syndrome.Patients and Method This prospective, observational study enrolled 40 children (58% male children, median age of 13 years, and median weight of 47.5 kg) with Wolff–Parkinson–White syndrome. Conduction through the AP to a cycle length of ≤250 ms was considered rapid or high-risk; otherwise, patients were nonrapid or low-risk.ResultsThe sudden disappearance of the delta-wave was seen in 10 cases (25%) during EST. Accessory pathway was found to be high-risk in 13 cases (13/40, 32.5%) while the accessory path was identified as low-risk in 27 cases; however, six patients (15%) had blocked AP conduction with adenosine during EPS. Low-risk classification by EST alone to identify patients with nonrapid conduction in baseline EPS had a specificity of 93% and a positive predictive value of 90% (accuracy 54%). Blocked AP conduction with adenosine as a marker of nonrapid baseline AP conduction had a specificity of 93% and a positive predictive value of 84%. Finally, AP was adenosine nonresponsive in the majority of patients (28/30, 93%) with persistent delta-waves, 40% of those who had a sudden disappearance of delta-waves had an adenosine-responsive AP (P value: .028).Conclusion Abrupt loss of preexcitation during EST and blocked AP conduction with adenosine had high specificity and positive predictive value for nonrapid and low-risk antegrade conduction during baseline invasive EPS. Successful risk stratification of pediatric patients with Wolff–Parkinson–White is possible through the use of EST and the adenosine responsiveness of AP.
    Congenital Heart Disease 06/2015; DOI:10.1111/chd.12270