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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    • If OnlineOpen is available, AHRC and ESRC authors, may self-archive after 24 months
    • Publisher last contacted on 07/08/2014
    • This policy is an exception to the default policies of 'Wiley'
  • Classification
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Publications in this journal

  • [Show abstract] [Hide abstract]
    ABSTRACT: Adult congenital heart disease (ACHD) patients have high rates of untreated depression and anxiety disorders. We evaluated associations among self-reported depression symptoms and alcohol/smoking tobacco use. From 2009 to 2013, 202 ACHD patients (45% male) completed questionnaires on depressive symptoms, anxiety symptoms, and substance use as part of routine clinical care. Data were collected by retrospective chart review. Mean age was 31 ± 10 years, 21% reported often feeling depressed and 33% reported feeling nervous or anxious. Sixty-one percent of patients reported some alcohol intake; 25% reported current or previous smoking tobacco use. Patients with depressive symptoms were 3× as likely to report drinking alcohol (OR 2.89; 95% CI 1.29-6.5) and 5× more likely to report smoking tobacco use (OR 5.17; 95% CI 1.49-17.87). Fourteen percent of patients were prescribed antidepressant/antianxiety medications; 43% of patients on medication reported depressive symptoms. In patients reporting symptoms, those who consumed alcohol were less likely to be on antidepressant/antianxiety medications (21%) than those who did not consume alcohol (56%). Self-reported depressive symptoms are associated with increased alcohol and smoking tobacco use by ACHD patients. Alcohol use may be a means of self-medicating for untreated depression, but further investigation is needed. Risk factors, including depressive symptoms and substance use, should be routinely assessed and addressed in ACHD patients. © 2015 Wiley Periodicals, Inc.
    Congenital Heart Disease 06/2015; DOI:10.1111/chd.12282
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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: 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: 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: 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
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    ABSTRACT: In adults with cardiomyopathy, tissue Doppler imaging (TDI) has been shown to correlate with left ventricular filling pressures (LVFPs) and has been advocated as a surrogate to catheterization. However, this has not been validated in children. This is a retrospective review of children ≤18 years old with dilated, hypertrophic, restrictive, and left ventricular noncompaction cardiomyopathy who underwent cardiac catheterization within 3 months of an echocardiogram. Spearman's correlation coefficients were calculated to assess a correlation between LVFP and mitral inflow E/A ratio, lateral mitral E/E', and septal E/E'. Thirty-eight patients were included in the study; median age was 8.6 years old. The median LVFP was 19 mm Hg, median mean pulmonary artery pressure was 25 mm Hg, and median pulmonary vascular resistance index (PVRi) was 2.4 Wu. There was no significant correlation between LVFP or PVRi with lateral mitral E/E' or septal E/E'. There was a positive correlation between LVFP and mitral inflow E/A ratio (rs = 0.59, P < .01). In a subgroup analysis of patients with hypertrophic or restrictive cardiomyopathy, there was a negative correlation (rs = 0.56, P = .02) between the mean pulmonary artery pressure and septal A'. TDI measures of diastolic function are not reliable surrogates for LVFP, mean pulmonary artery pressures, and PVRi at catheterization in children. © 2015 Wiley Periodicals, Inc.
    Congenital Heart Disease 05/2015; 63(12). DOI:10.1111/chd.12267
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    ABSTRACT: Objective Traditionally, karyotype and fluorescence in situ hybridization (FISH) were used for cytogenetic testing of infants with congenital heart disease (CHD) who underwent cardiac surgery at our institution. Recently, chromosome microarray analysis (CMA) has been performed in lieu of the traditional tests. A standardized approach to cytogenetic testing does not exist in this population. The purpose of this study was to assess the utility of CMA based on our current ordering practice.DesignWe reviewed the records of all infants (<1 year old) who underwent cardiac surgery at our institution from January 2010 to June 2013. Data included results of all cytogenetic testing performed. Diagnostic yield was calculated as the percentage of significant abnormal results obtained by each test modality. Patients were grouped by classification of CHD.ResultsTwo hundred seventy-five (51%) of 535 infants who underwent cardiac surgery had cytogenetic testing. Of those tested, 154 (56%) had multiple tests performed and at least 18% were redundant or overlapping. The utilization of CMA has increased each year since its implementation. The diagnostic yield for karyotype, FISH and CMA was 10%, 12%, and 14%, respectively. CMA yield was significantly higher in patients with septal defects (33%, P = .01) compared with all other CHD classes. CMA detected abnormalities of unknown clinical significance in 13% of infants tested.Conclusions In our center, redundant cytogenetic testing is frequently performed in infants undergoing cardiac surgery. The utilization of CMA has increased over time and abnormalities of unknown clinical significance are detected in an important subset of patients. A screening algorithm that risk-stratifies based on classification of CHD and clinical suspicion may provide a practical, data-driven approach to genetic testing in this population and limit unnecessary resource utilization.
    Congenital Heart Disease 05/2015; 10(3). DOI:10.1111/chd.12241
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    ABSTRACT: Hypertrophic cardiomyopathy is underscored by profound phenotypic and genotypic heterogeneity. Echocardiographically, hypertrophic cardiomyopathy can be categorized into four morphological subtypes: reverse curve, sigmoidal, neutral contour, and apical variant. Previous studies indicate that reverse curve hypertrophic cardiomyopathy is the strongest predictor of a positive genetic test. Little is known about the spectrum and prevalence of mutations and genotype–phenotype correlations in apical hypertrophic cardiomyopathy.
    Congenital Heart Disease 05/2015; 10(3). DOI:10.1111/chd.12242
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    ABSTRACT: Objective The impact of obesity on surgical morbidity in adults with congenital heart disease is currently unknown. The aim of our study was to investigate the impact of obesity on postoperative outcomes in adults with congenital heart disease undergoing reoperation for pulmonary valve replacement.MethodsA retrospective analysis was performed assessing the influence of obesity on surgical outcomes. Obesity was defined as a body mass index ≥30 kg/m2.ResultsThe mean body mass index of the cohort was 25.9 ± 6.9 kg/m2. The cohort included 71 patients with 17 patients (24%) being obese. There was no postoperative mortality. Obese patients had a longer hospital length of stay (6.6 vs. 4.7 days; P < .001) and increased incidence of postoperative arrhythmias (29% vs. 5.6%; P = .003) compared with nonobese patients. Multivariable analysis performed using logistic regression with backwards elimination demonstrated obesity was independently associated with hospital length of stay >5 days (odds ratio [OR] = 5.2; 95% confidence interval [CI]: 1.5–18.2, P = .01) and with increased postoperative arrhythmias (OR = 4.2; 95% CI: 1.7–40, P < .01).Conclusions Obesity is associated with increased morbidity in adults with congenital heart disease undergoing pulmonary valve replacement, including longer hospitalization and higher risk for postoperative arrhythmias.
    Congenital Heart Disease 05/2015; DOI:10.1111/chd.12266
  • Congenital Heart Disease 05/2015; 10(3):191-2. DOI:10.1111/chd.12274
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    ABSTRACT: IntroductionHeart transplant recipients are at risk for developing left ventricular (LV) dysfunction. While traditional echocardiographic parameters have limitations, speckle tracking echocardiography (STE) is a novel technique shown to be more sensitive and accurate in adult studies for evaluating ventricular systolic and diastolic function.DesignPediatric heart transplant recipients undergoing routine cardiac catheterization were included. Ratio of mitral early diastolic velocity-to-strain rate during early LV filling (E/SRE) and global longitudinal peak systolic strain (GLPSS) was measured by STE imaging. These were compared with wedge pressures by catheterization and traditional echocardiographic parameters.ResultsA total of 50 subjects (46% males) were included. Mean age of the subjects was 13.0 ± 6.3 years and time since transplant was 4.1 years (range 0.2–17.1 years). While peak mitral inflow to late diastolic velocity (E/A ratio) was the only traditional diastolic function parameter having significant correlation with pulmonary capillary wedge pressure (PCWP) (r = 0.3; P = .3), STE-derived E/SRE had modest correlation with PCWP (r = 0.55; P < .01). Also, while most traditional systolic function parameters were normal, 12 subjects (24%) had GLPSS > −18%. Interestingly, subjects with coronary artery disease (n = 6) had significantly higher E/SRE (71.9 ± 28.4) compared to subjects without (45.2 ± 10.8; P < .001).Conclusion Diastolic function parameters by STE imaging correlate better with gold standard PCWP measurement than traditional echocardiographic parameters. Also, utilizing STE, abnormalities of longitudinal LV systolic function may be more common than previously thought in pediatric heart transplant recipients without acute graft rejection, despite “normal” systolic function by traditional echocardiogram.
    Congenital Heart Disease 05/2015; DOI:10.1111/chd.12263
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    ABSTRACT: Objective After complete surgical repair the majority of tetralogy of Fallot (ToF), patients stay ≤2 days in the Cardiac Intensive Care Unit (CICU) while some may stay longer. We undertook this study to investigate the factors associated with shorter vs. longer length of stay in the CICU to help manage resources effectively.DesignPatients who underwent ToF repair at Aga Khan University, Pakistan, between July 2006 and December 2011 were studied in a case-control design. Clinical parameters were compared between short stay group (SSG) (≤2 days) and long stay group (LSG) (>2 days). Odds ratios were calculated, and regression was performed.ResultsNinety-eight patients (LSG 65, SSG 33) were included. Patients with lower preoperative saturation were 2.67 times more likely to be in the LSG group (P = .02). At 4 hours postoperatively, patients with a higher inotropic score (odds ratio [OR] = 3.03, confidence interval [CI] = 1.19–7.7, P = .02), higher central venous pressure (OR = 3.04, CI = 1.27–7.32, P = .013), and significant tachycardia at 4 hours (OR = 3.5, CI = 1.19–10.3. P = .02) were at risk for having a prolonged CICU stay. On multivariate analysis, significant postoperative tachycardia at 4 hours (z-score ≥3) was highly specific (sensitivity = 38.5%, specificity = 84.9%) for predicting the chances of being in the LSG. Other predictors included preop O2 saturation ≤82.5% (sensitivity = 61.1%, specificity = 63.0%) and CVP ≥10 mm Hg at 4 hours (sensitivity = 55.4%, specificity = 71.9%).Conclusion Patients who end up staying longer in the CICU have features that are distinctive in the immediate postoperative period, and this can help clinicians in identifying patients who may need more support.
    Congenital Heart Disease 05/2015; DOI:10.1111/chd.12259
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    ABSTRACT: Background Long QT syndrome (LQTS) is a genetic heart rhythm disorder that may present with syncope, seizures, or sudden cardiac death. Breath holding spells (BHS) occur in 5% of all children and have been noted in children with LQTS anecdotally. The purpose of this study was to determine the frequency of BHS in children diagnosed with LQTS at ≤5 years of age.DesignA retrospective review was performed to identify children diagnosed with LQTS who were ≤5 years old at initial presentation to our LQTS clinic from August 1999 to November 2013. The mean length of follow-up was 6.4 ± 2.8 years. The electronic medical records were reviewed for clinical presentation of BHS, as well as LQTS-associated symptoms, diagnostic tests, and treatment.ResultsThe study cohort consisted of 115 children with LQTS (58% male; median age at diagnosis, 11 months [range, birth to 5 years]; mean corrected QT interval (QTc), 478 ± 60 milliseconds). At presentation, 80% of patients were asymptomatic. Genetic testing revealed type 1 LQTS (LQT1) in 48%. Overall, 5 of 115 patients (4.3%) had BHS (2 of 5 [40%] male, mean QTc: 492 ± 14 milliseconds, 4 [80%] with family history of LQTS). BHS were the presenting symptom in 1 of 23 symptomatic patients (4.3%). All BHS occurred in patients with LQT1 (P = .02).Conclusions Although BHS among children with LQTS are relatively rare and occur at similar frequency as the general population, they can be the presenting symptom for a heart rhythm disorder. Careful attention to BHS is important to distinguish an innocent BHS from a potential LQTS-triggered cardiac event so that proper treatment is initiated.
    Congenital Heart Disease 05/2015; DOI:10.1111/chd.12262
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    ABSTRACT: The purpose of this study was to compare neurological development of children with cyanotic or hemodynamically impaired congenital heart disease (CHD) and healthy controls by using "Bayley Scales of Infant and Toddler Development Screening Test, Third Edition" (Bayley-III). Children with CHD (n = 37) and healthy controls (n = 24) aged between 1 and 41 months who were admitted to the Department of Pediatric Cardiology at our university hospital were included. The participants were assessed using Bayley-III test. All patients had cyanotic or hemodynamically impaired CHD. Weight, height, body mass index (BMI), mid-arm circumference (MAC), triceps skinfold thickness (TSF), and head circumference (HC) were measured and standard deviation scores (SDSs) were determined. SDS values of weight, height, BMI, MAC, and TSF of the patients as well as HC values were significantly lower than the control group (P < .001). Compared with controls, the patients had significantly lower mean scores in all Bayley-III subscales (P < .001). We observed similar results in Bayley-III scores including the mean values of cognitive, language, and global motor scores for the CHD patients with and without cardiac surgery (P > .05). This study demonstrated that children with cyanotic or hemodynamically impaired CHD have delayed neurodevelopmental outcomes compared with healthy children as assessed using Bayley-III. © 2015 Wiley Periodicals, Inc.
    Congenital Heart Disease 04/2015; DOI:10.1111/chd.12269
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    ABSTRACT: This study investigated the clinician practices on perioperative anticoagulation in children with prosthetic mechanical heart valves who undergo elective surgeries. An online survey was administered to members of PediHeartNet. The survey consisted of multiple choice questions and clinical scenarios. The study described clinical practice patterns and variables that influence the clinicians' bridging anticoagulation decisions. Ninety-one respondents completed the survey; 68% were affiliated with university settings; 91% were pediatric cardiologists, and 49% had ≥10 years of experience in pediatric cardiology. Approximately one-half of the respondents (54%) independently provided perioperative anticoagulation management to their patients, while 46% utilized cardiac or hematology anticoagulation services. Resources that influenced bridging decisions included hematology experts (20%), American College of Chest Physicians guidelines (34%), and the clinicians' personal experience (56%). In planning for major surgeries, 47% of the respondents hospitalized patients for unfractionated heparin (UFH) and 46% prescribed outpatient low molecular weight heparin (LMWH). For minor surgeries, 58% hospitalized patients for UFH, 22% prescribed outpatient LMWH, and 17% opted out of bridging anticoagulation. Immediately after mitral valve replacement, 23% used bridging anticoagulation with UFH. When LMWH was used, there were no reports of thromboembolic complications. Major bleeding complications were rare and reported by 2% of the respondents. This was the first documentation that clinical practice of bridging perioperative anticoagulation in children with mechanical heart valves varies widely among pediatric cardiac specialists. There is poor adoption of published guidelines and a tendency toward more conservative strategies. Further studies comparing the safety and efficacy of LMWH vs. UFH as perioperative anticoagulation agents in children with mechanical heart valves are needed to further clarify our findings. Quality assurance initiatives and education are also needed to improve guidelines adherence and standardize practice management. © 2015 Wiley Periodicals, Inc.
    Congenital Heart Disease 04/2015; DOI:10.1111/chd.12268
  • Congenital Heart Disease 04/2015; DOI:10.1111/chd.12264