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: 1.08

Impact Factor Rankings

2015 Impact Factor Available summer 2016
2014 Impact Factor 1.076
2013 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 1.24
Cited half-life 3.80
Immediacy index 0.23
Eigenfactor 0.00
Article influence 0.47
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

Publications in this journal

  • [Show abstract] [Hide abstract]
    ABSTRACT: Objectives: To evaluate the use of smartphone and tablet technology ("smart technology" or ST) in remote consultation for pediatric and congenital cardiac catheterization. Design: Two online surveys, to early-career (Mentees) and senior (Mentors) pediatric interventional cardiologists, designed to assess the current use and attitudes toward ST for remote consultation. In addition, the current literature is reviewed for use of remote consultations and potential legal and patient privacy concerns. Results: Forty-six (40%) Mentees and 19 (48%) Mentors responded. 74% of Mentees report using ST for remote consultation, 26% for nearly every case, with 45% of communication occurring during a procedure. Over one-third of Mentees report they have performed an intervention for the first time based on ST consultation. Of Mentors that reported receiving ST consultation, 58% were comfortable providing advice via this method, but 42% felt that the imaging sent was inadequate to provide appropriate advice for at least one case. Both groups felt the speed of consultation was much faster using ST. Privacy measures were reportedly used in up to 85% of consultations, while only 12% of respondents obtained informed consent for the consultation. The most common barrier identified by Mentees for use of ST for consultations was patient privacy concerns. Conclusions: ST use is prevalent among early-career pediatric and congenital interventional cardiologists for consultation regarding catheterization procedures. There are many technologic, legal, and patient privacy concerns that will need to be addressed as the use of ST for remote consultation grows.
    Congenital Heart Disease 11/2015; DOI:10.1111/chd.12308
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    ABSTRACT: Aim: To determine if a transthoracic echocardiography (TTE) can be used as the sole diagnostic imaging modality to evaluate children with congenital heart disease (CHD) undergoing cardiac surgery. Methods: A retrospective study was carried out at the King Abdulaziz Cardiac Center. We reviewed all pediatric patients who underwent cardiac surgery during the period January 2011 to December 2011. Results: Three hundred ninety-one pediatric patients with CHD fulfilled the inclusion criteria. Of these patients, 287 (73%) underwent surgical interventions based on a TTE alone, while 105 (27%) required additional diagnostic imaging modalities, including a cardiac catheterization (68/105; 65%), cardiac computed tomography angiography (36/105; 35%), or cardiac magnetic resonance imaging (1/105; 1%). A TTE was not enough for all the patients who underwent a cardiac catheterization to find out additional anatomical information (22%), either to directly measure pulmonary artery pressures (62%) or to study vascular reactivity in patients with pulmonary hypertension (16%). Of 36 patients who underwent a cardiac computed tomography angiography, five (14%) had additional information to be added to TTE findings. Of all the patients, 81% had enough information using only the TTE compared to 19% in whom the TTE was not enough to provide all needed information. Only seven of 392 (0.02%) patients had additional minor intraoperative findings that did not affect the surgical decision. Conclusion: Despite the emergence of other imaging modalities, a TTE can be used as the sole diagnostic imaging modality for a preoperative assessment in the majority of children with CHD. Other imaging modalities can be employed with limited indications.
    Congenital Heart Disease 11/2015; DOI:10.1111/chd.12312
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    ABSTRACT: Background: Percutaneous closure of atrial septal defect (ASD) has become the preferred method in treatment of the majority of cases. The aim of this study was to evaluate the echocardiographic effects of percutaneous closure of secundum ASD in adults and assess which parameters predict good response to closure. Design: We prospectively included 42 patients with secundum ASD treated successfully with percutaneous device closure. All patients underwent transthoracic echocardiography examination with tissue Doppler imaging before, 24 hours after and within 3 months of intervention. Measurements of arterial stiffness were carried out using a Mobil-O-Graph arteriography system. Results: Remodeling of heart chambers occurred immediately and persisted at 3 months after ASD closure. Significant decreases were observed in right ventricle (RV) end-diastolic diameter, right atrium volume index, and tricuspid annular plane systolic excursion both after the procedure and at 3 months (P < .01 for all). The RV and left ventricle (LV) tissue Doppler-myocardial performance index demonstrated to decline during follow up (P = .0001). Significant correlations were found between pulse-wave velocity, augmentation index, pulmonary artery stiffness, and LV-RV tissue Doppler-myocardial performance index at third month. Linear regression analyses showed that pulse-wave velocity is the most effective parameter of LV and pulmonary artery stiffness is the most effective parameter of RV functional recovery, respectively, assessed by tissue Doppler-myocardial performance index. Conclusions: Percutaneous closure of secundum ASD in adults has various sustained benefits on multiple echocardiographic parameters within 3 months. The changes in RV and LV function after device closure were significantly correlated with the degree of pulmonary artery stiffness and pulse-wave velocity, respectively.
    Congenital Heart Disease 11/2015; DOI:10.1111/chd.12302
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    ABSTRACT: Objective: The aim of this study was to compare the functional capacity of children with TGA long-term after ASO with that of healthy children. Design: Retrospective study. Patients: All patients that performed cardiopulmonary exercise test (CPET) were included in the study. As a control group, healthy children in evaluation for physical activity that performed CPET during the same period were also enrolled. Results: Thirty-one TGA patients (19 male) were compared with 29 age-matched controls (21 male). Maximum oxygen consumption was higher in the control group (45.47 ± 8.05 vs. 40.52 ± 7.19, P = .017), although within normal limits in both groups (above 90% of predicted value). The heart rate behavior during exercise was different in both groups, with a mean chronotropic index significantly lower in the TGA group (63% ± 14 vs. 81% ± 12, P < .001). Conclusions: Our results showed that exercise capacity long-term after ASO in TGA is well preserved although lower than in healthy children what might be explained by the presence of chronotropic incompetence in the TGA group.
    Congenital Heart Disease 11/2015; DOI:10.1111/chd.12303
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    ABSTRACT: Transparency-sharing data or information about outcomes, processes, protocols, and practices-may be the most powerful driver of health care improvement. In this special article, the development and growth of transparency within the National Pediatric Cardiology Quality Improvement Collaborative is described. The National Pediatric Cardiology Quality Improvement Collaborative transparency journey is guided by equal numbers of clinicians and parents of children with congenital heart disease working together in a Transparency Work Group. Activities are organized around four interrelated levels of transparency (individual, organizational, collaborative, and system), each with a specified purpose and aim. A number of Transparency Work Group recommendations have been operationalized. Aggregate collaborative performance is now reported on the public-facing web site. Specific information that the Transparency Work Group recommends centers provide to parents has been developed and published. Almost half of National Pediatric Cardiology Quality Improvement Collaborative centers participated in a pilot of transparently sharing their outcomes achieved with one another. Individual centers have also begun successfully implementing recommended transparency activities. Despite progress, barriers to full transparency persist, including health care organization concerns about potential negative effects of disclosure on reputation and finances, and lack of reliable definitions, data, and reporting standards for fair comparisons of centers. The National Pediatric Cardiology Quality Improvement Collaborative's transparency efforts have been a journey that continues, not a single goal or destination. Balanced participation of clinicians and parents has been a critical element of the collaborative's success on this issue. Plans are in place to guide implementation of additional transparency recommendations across all four levels, including extension of the activities beyond the collaborative to support transparency efforts in national cardiology and cardiac surgery societies.
    Congenital Heart Disease 11/2015; DOI:10.1111/chd.12314
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    ABSTRACT: Background: Lymphopenia is common in patients with Fontan circulation and no history of protein-losing enteropathy, but this phenomenon has not been significantly described in the literature. Methods: We retrospectively identified patients with Fontan circulation who underwent catheterization between January 2003 and January 2013 at our center. Patients who had complete blood count with differential drawn within 12 months of the catheterization were included. Patients were excluded if complete blood count with differential was drawn in setting of possible infection or if there was history of protein-losing enteropathy (PLE). Possible associations between patient characteristics and absolute lymphocyte count (ALC) were examined. Results: Fifteen patients were included. The median age at catheterization was 10.2 years (3.8-26.9) and median time from Fontan operation was 6.5 years (0.7-22.1). Twelve (80%) patients had undergone extracardiac Fontan and 9 (60%) had fenestration placed. The median time between complete blood count with differential and catheterization was 2 days (0-346). The median inferior vena cava (IVC) pressure was 13 mm Hg (7-20). The median ALC was 1.5 × 10(3) /μL (0.8-4.5). Four patients (26.7%) met criteria for lymphopenia with ALC < lower limit of normal and 7 (46.7%) patients had an ALC ≤ lower limit of normal. ALC was not associated with any hemodynamic variables but was associated with platelet count (rho = 0.5, P = .04), total white blood cell count (rho = 0.8, P ≤ .001), and absolute monocyte count (0.7, P = .002). Conclusions: In a cohort of patients with Fontan circulation and no history of protein-losing enteropathy who underwent catheterization, lymphopenia was common and positively associated with low platelet count. Thrombocytopenia has been shown to correlate with the degree of hepatic fibrosis in those with Fontan and, thus, hepatic fibrosis may underlie lymphopenia in these patients.
    Congenital Heart Disease 11/2015; DOI:10.1111/chd.12313
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    ABSTRACT: Background: Accurate quantification of aortic dilatation is critical in children with syndromes associated with thoracic aortic aneurysm, yet classification of normality is difficult. Current methods of normalization use body surface area to account for growth, despite a nonlinear relationship of body surface area to aortic root dimensions. In contrast, height has a linear relationship with aortic root dimensions in normal children, is simple to measure and requires no secondary calculation. We evaluated the diagnostic accuracy of an height-based aortic root-indexing method, aortic root cross-sectional area/height ratio (AHr), in children with Marfan and Loeys-Dietz syndromes. Methods: A cohort of 54 children with Marfan or Loeys-Dietz syndromes, aged 3 months to 17 years, were evaluated with a transthoracic echocardiogram. AHr was measured in diastole at sinuses of Valsalva (SoV) and proximal ascending aorta (pAA) in a group of normal subjects matched for age and body surface area and normal values were provided. AHr values were recorded for patients and compared with z-scores results obtained with Gautier's and Campens's nomograms. Results: AHr values in the group of normal subjects were 2.6 ± 0.6 at SoV and 2 ± 0.5 at pAA. Categorization of z-scores and AHr showed good correspondence between AHr and Gautier's method (P = .341 at SoV and .185 at pAA) and AHr and Campens method (P =.465 at SoV and 0.110 at pAA). Conclusions: There was a good correspondence of AHr results with two different z-scores. AHr is a simple to use and valid option to quantify aortic root dilatation in pediatric patients.
    Congenital Heart Disease 11/2015; DOI:10.1111/chd.12315

  • Congenital Heart Disease 11/2015; DOI:10.1111/chd.12301
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    ABSTRACT: Objective: As the adult congenital heart disease population increases, poor transition from pediatric to adult care can lead to suboptimal quality of care and an increase in individual and institutional costs. In 2008, the American College of Cardiology and American Heart Association updated the adult congenital heart disease practice guidelines and in 2011, the American Heart Association recommended transition guidelines to standardize and encourage appropriate timing of transition to adult cardiac services. The objective of this study was to evaluate if patient age or complexity of congenital heart disease influences pediatric cardiologists' decision to transfer care to adult providers and to evaluate the compliance of different types of cardiology providers with current adult congenital heart disease treatment guidelines. Design: A single-center retrospective review of 991 adult congenital heart disease patients identified by ICD-9 code from 2010 to 2012. Setting: Academic and community outpatient cardiology clinics. Patients: Nine hundred ninety-one patients who are 18 years and older with congenital heart disease. Intervention: None. Outcomes measures: The compliance with health maintenance and transfer of care recommendations in the outpatient setting. Results: For patients seen by pediatric cardiologists, only 20% had transfer of care discussions documented, most often in younger simple patients. Significant differences in compliance with preventative health guidelines were found between cardiology provider types. Conclusion: Even though a significant number of adults with congenital heart disease are lost to appropriate follow-up in their third and fourth decades of life, pediatric cardiologists discussed transfer of care with moderate and complex congenital heart disease patients less frequently. Appropriate transfer of adults with congenital heart disease to an adult congenital cardiologist provides an opportunity to reinforce the importance of regular follow-up in adulthood and may improve outcomes as adult congenital cardiologists followed the adult congenital heart disease guidelines more consistently than pediatric or adult cardiologists.
    Congenital Heart Disease 11/2015; DOI:10.1111/chd.12309
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    ABSTRACT: Background and aims: Patients who underwent a successful repair of the aortic coarctation show chronic hyperdynamic state and normal left ventricular (LV) geometry; however, there are few data regarding the LV systolic function in the long term. Accordingly, we assessed LV systolic mechanics and factors associated with LV systolic dysfunction (LVSD) in patients with repaired CoA. Methods: Clinical and echocardiographic data from 19 repaired CoA were analyzed 28 ± 13 years after surgery. Stress-corrected midwall shortening (sc-MS) and mitral annular peak systolic velocity (S') were analyzed as indexes of LV circumferential and longitudinal systolic function, respectively. Echocardiographic data of CoA patients were compared with 19 patients matched for age and hypertension and 38 healthy controls. Sc-MS was considered impaired if <89%, S' if <8.5 cm/s (10th percentiles of healthy controls, respectively). Results: There were no statistical differences between study groups in LV volumes, mass and geometry. LV ejection fraction and Sc-MS were similar in all groups, however, CoA group had a significantly lower peak S' in comparison with matched and healthy controls (7.1 ± 1.3, 10.3 ± 1.9, and 11.1 ± 1.5, respectively; all P < 0.001). Prevalence of longitudinal LVSD defined as low S' was 84% in CoA, 13% in matched, and 5% in healthy control group (all P<0.05). Multivariate logistic regression analysis revealed that low peak S' was independently related to higher E/E' ratio and the presence of CoA. Conclusions: Patients who underwent a successful repair of CoA commonly show asymptomatic longitudinal LVSD associated with worse LV diastolic function in the long-term follow-up.
    Congenital Heart Disease 11/2015; DOI:10.1111/chd.12310
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    ABSTRACT: Objective: There is a paucity of data on clinical correlates and outcomes of pulmonary hypertension (PH) in patients with D-transposition of the great arteries (D-TGA) in the era of inhaled nitric oxide (iNO) and extracorporeal membrane oxygenation (ECMO). Our objective was to compare clinical characteristics and outcomes of infants with D-TGA with and without PH, defined as hypoxemia that required iNO and/or ECMO. Methods: We undertook a single-center retrospective chart review involving infants with gestational age ≥32 weeks with D-TGA who, underwent arterial switch operation over a 12-year period. Demographic and clinical data, details of the repair and postoperative complications were abstracted. Results: Our cohort (n = 93), 61 (66%) of whom were males, had a mean (SD) gestational age and birth weight of 38.7 (1.8) weeks and 3.2 (0.6) kg, respectively. PH requiring iNO and/or ECMO was noted in 20 (21.5%) infants. Infants with PH had significantly lower birth weight [2.8 (0.56) vs. 3.33 (0.61)] and gestational age [37.7 (2.1) vs. 38.9 (1.7)] than those without PH. Rates of postoperative complications (duration of pressors, sedative medicaiton and duration of hospital stay, and mechanical ventilation were higher in the group with PH. Of the five (5.4%) infants who died, four received iNO and ECMO. Death or postoperative complications tended to be associated with lower gestational age [OR 0.689; 95% CI: 0.469-1.012, P = 0.058] but not with D-TGA category or bypass duration. Conclusions: Despite aggressive treatment with iNO and ECMO, the coexistence of PH in this population is associated with higher rates of mortality and postoperative complications. Our results also suggest that an early term birth may be associated with PH in infants with D-TGA.
    Congenital Heart Disease 11/2015; DOI:10.1111/chd.12304
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    ABSTRACT: A program to collect and analyze cardiac catheterization, electrophysiologic studies and cardiac operations in children was initiated in 1982. The purpose was to help centers compare their experience and outcomes with a group of centers to determine areas where their performance might improve. Cardiac centers became members of the Pediatric Cardiac Care Consortium and submitted demographic data and copies of procedure reports regularly to a central office. Data were extracted from the reports, coded by trained coders and entered into a computer database. Annually, the data were analyzed to compare the experience of an individual center with that of the entire group of centers. The annual data were adjusted for severity on the basis of eight factors selected after discussion with participants in the Consortium. Adjustment was by multivariate analysis. Reports were prepared for each center and distributed at an annual meeting. The data were used by centers to review operations where the mortality rate exceeded +2 standard deviations of the group. With discussion, the center staff often initiated changes to improve outcome. The outcome could then be monitored by the annual reports. Our data were also utilized in the creation of the Risk Adjustment for Surgery for Congenital Heart Disease (RACHS)-1 categories of disease severity. The mortality rates of our centers were comparable with the combined hospital discharge data from New York, Massachusetts, and California. From 1982 through 2007, the mortality rates of our centers dropped for each RACHS-1 category, falling to less than 1% for categories 1 and 2 for the last 5-year period. During the 25 years, we received data from 52 centers about 137 654 patients who underwent 117 756 cardiac operations.
    Congenital Heart Disease 10/2015; DOI:10.1111/chd.12297
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    ABSTRACT: Surgery for congenital heart disease has advanced significantly in the past 50 years, such that repair of "simple" lesions, such as atrial septal defect, ventricular septal defect, and coarctation of the aorta carries minimal risk, with mortality risk much less than 1%. It was once thought successful repair of these lesions was definitively corrective. There is mounting evidence, however, that there are long-term complications after these repairs, prompting the need for continued follow-up. This review describes the current understanding of diagnosis, treatment, and long-term outcomes for these patients, with the goal of advocating for lifelong surveillance. As the perioperative care of these repairs has evolved significantly over time, so must the way in which we study these patients in the long-term.
    Congenital Heart Disease 09/2015; 10(5). DOI:10.1111/chd.12298
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    ABSTRACT: Objective: Obesity affects adults with congenital heart disease (CHD). The effect of an increased body mass index (BMI) with respect to morbidity and mortality has not been evaluated in adults with complex CHD. Our objective was to evaluate the effects of increased BMI on heart failure and mortality in univentricular patients who had undergone Fontan palliation. Methods: A query of Fontan patients' first appointments at the Washington University Center for Adults with CHD between 2007 and 2014 yielded 79 patients. BMI status as normal (<25 kg/m(2) ), overweight (≥25, <30 kg/m(2) ), and obese (≥30 kg/m(2) ) was established at the patient's first appointment. We analyzed demographics, diuretic requirements, New York Heart Association (NYHA) class, and laboratory values using Student's two-sample t-test and Fisher's exact test. Mortality was assessed via survival curves, and hazard ratios were compiled with proportional hazard modeling. Results: The recent average BMI was significantly greater in patients with NYHA classes II-IV (29.3 ± 9 kg/m(2) ) compared with asymptomatic patients (24.8 ± 5.1 kg/m(2) , P = .006). Additionally, the average BMI of patients with a high diuretic requirement (≥40 mg/day IV furosemide equivalent) was obese, at 32.15 ± 9.1 kg/m(2) , compared with 25.91 ± 7.3 kg/m(2) for those on no or lower doses of diuretics (P = .009). Eighteen of the 79 patients met an endpoint of death, hospice placement, or cardiac transplant by the study conclusion. Kaplan-Meier analysis from time of first appointment until recent follow-up revealed a significant association between time to combined endpoint and BMI class. Cox proportional hazard modeling with age adjustment yielded a hazard ratio of 3.2 (95% CI 1.096-9.379) for obesity upon first presentation to an adult CHD clinic. Conclusions: In patients with univentricular hearts and Fontan palliation, obesity is associated with symptomatic heart failure and mortality.
    Congenital Heart Disease 09/2015; DOI:10.1111/chd.12296
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    ABSTRACT: No abstract is available for this article.
    Congenital Heart Disease 09/2015; 10(5). DOI:10.1111/chd.12305
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    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