Brent J Barber

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Scholarly Contributions

Books

• Abdy, N. A., Barber, B. J., Abdy, N. A., & Barber, B. J. (2011). Pulmonary Valve Stenosis. The 5-Minute Clinical Consult 2011: Lippincott Williams & Wilkins.

Chapters

• Shwaish, N. S., & Barber, B. J. (2015). Ventricular septal defect. In The 5-minute Clinical Consult. Wolters Kluwer Health Adis (ESP).

Journals/Publications

• Hendrickson, A. M., Caryl, N. E., Meziab, O., Andrews, J. G., Barber, B. J., & Seckeler, M. (2025).

  Association Between G6PD Deficiency and Congenital Heart Disease Incidence and Hospital Outcomes

  . Pediatric Cardiology.
• Hendrickson, A. M., Caryl, N. E., Meziab, O., Andrews, J. G., Barber, B. J., & Seckeler, M. D. (2025). Association Between G6PD Deficiency and Congenital Heart Disease Incidence and Hospital Outcomes. Pediatric Cardiology. doi:10.1007/s00246-025-04071-5
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  G6PD deficiency classically presents with hemolytic anemia, but associations have been described with atherogenesis and coronary artery disease secondary to increased oxidative stress. There are limited data that also suggest a link between G6PD deficiency and the development of congenital heart disease (CHD), possibly through the same mechanism. We hypothesize a higher incidence of CHD among individuals with G6PD deficiency as well as worse hospital outcomes for those with CHD and G6PD deficiency. We performed a retrospective review of a national administrative database from 10/2019 to 3/2024 for admissions of individuals aged 0-25 years with ICD-10 codes for moderate-to-severe CHD with and without G6PD deficiency (D55.0, D75.A). Additional data included demographics, hospital outcomes, and costs. Hospital outcomes were compared. There were 213,708 admissions with CHD and
• Meziab, O., Dereszkiewicz, E., Guerrero, C., Hoyer, A., Barber, B. J., Klewer, S. E., & Seckeler, M. (2025).

  Adverse Effect of Bundle Branch Block on Exercise Performance in Patients with Fontan Physiology: From the Pediatric Heart Network Fontan Public Data Set

  . Pediatric Cardiology, 46(4), 813-818. doi:10.1007/s00246-024-03500-1
• Webber, Z., Grisham, L. M., Meziab, O. Z., Hoyer, A. W., Barber, B. J., Price, A. M., Klewer, S. E., Largent-Milnes, T. M., & Seckeler, M. D. (2025). Higher Incidence of Moderate and Severe Congenital Heart Disease in Patients With Neonatal Abstinence Syndrome. Neonatal Network, 44(Issue 4). doi:10.1891/nn-2024-0036
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  Studies suggest that in utero opioid exposure may be associated with congenital heart disease (CHD). We sought to assess the incidence of CHD in infants with neonatal abstinence syndrome (NAS). A review of a national, administrative database from January 2019 to December 2022 was conducted for neonates with an ICD-10 code for NAS and moderate or severe CHD. The incidence of CHD in NAS (the main outcome variable) was compared to those without NAS using χ2 analysis and odds ratios for CHD with significant differences. There were 4,994,919 neonatal admissions during the study period: 26,284 with NAS (2.3% had CHD) and 4,968,715 without NAS (1.1% had CHD) (p
• Webber, Z., Grisham, L., Meziab, O., Hoyer, A., Price, A., Barber, B. J., Klewer, S. E., Largent-Milnes, T., & Seckeler, M. (2025).

  Higher Incidence of Moderate and Severe Congenital Heart Disease in Patients With Neonatal Abstinence Syndrome

  . Neonatal Network, 44(4), 256-260. doi:10.1891/NN-2024-0036
• Meziab, O., Dereszkiewicz, E., Guerrero, C., Hoyer, A., Barber, B., Klewer, S., & Seckeler, M. (2024).

  Adverse Effect of Bundle Branch Block on Exercise Performance in Patients with Fontan Physiology: From the Pediatric Heart Network Fontan Public Data Set

  . Pediatric Cardiology. doi:10.1007/s00246-024-03500-1
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  Patients with Fontan physiology have reduced exercise performance compared to their peers as well as a higher incidence of bundle branch block (BBB). This study aims to investigate the association between BBB and exercise performance in the Fontan population through a retrospective review of the Pediatric Heart Network Fontan study public use dataset. “Low Performers” were defined as ≤ 25th percentile (for Fontan patients) for each exercise parameter at anaerobic threshold (AT) for gender and age and “Normal Performers” were all other patients. A total of 303 patients with Fontan physiology who underwent exercise testing reached AT and had complete data for BBB. BBB occurred more frequently in Low Performers for VO2 [OR (95% CI): 2.6 (1.4, 4.8)] and Work [OR (95% CI): 2.7 (1.4, 5.1)], suggesting that BBB in the Fontan population is associated with reduced exercise performance. This data adds to the existing clinical evidence of the adverse effects of conduction abnormalities on single ventricle cardiac output and adds support for consideration of cardiac resynchronization and multi-site ventricular pacing in this patient population.
• Meziab, O., Dereszkiewicz, E., Guerrero, C., Hoyer, A., Barber, B., Klewer, S., & Seckeler, M. (2024). Adverse Effect of Bundle Branch Block on Exercise Performance in Patients with Fontan Physiology: From the Pediatric Heart Network Fontan Public Data Set. Pediatric Cardiology. doi:10.1007/s00246-024-03500-1
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  Patients with Fontan physiology have reduced exercise performance compared to their peers as well as a higher incidence of bundle branch block (BBB). This study aims to investigate the association between BBB and exercise performance in the Fontan population through a retrospective review of the Pediatric Heart Network Fontan study public use dataset. “Low Performers” were defined as ≤ 25th percentile (for Fontan patients) for each exercise parameter at anaerobic threshold (AT) for gender and age and “Normal Performers” were all other patients. A total of 303 patients with Fontan physiology who underwent exercise testing reached AT and had complete data for BBB. BBB occurred more frequently in Low Performers for VO2 [OR (95% CI): 2.6 (1.4, 4.8)] and Work [OR (95% CI): 2.7 (1.4, 5.1)], suggesting that BBB in the Fontan population is associated with reduced exercise performance. This data adds to the existing clinical evidence of the adverse effects of conduction abnormalities on single ventricle cardiac output and adds support for consideration of cardiac resynchronization and multi-site ventricular pacing in this patient population.
• Meziab, O., Seckeler, M., Scherer, K., & Barber, B. (2024). Prevalence of cardiovascular implantable electronic devices in children with type 1 myotonic dystrophy. Muscle and Nerve, 70(5). doi:10.1002/mus.28188
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  Introduction/Aims: Type 1 myotonic dystrophy (DM1) is a neuromuscular disorder of multiple organ systems with important electrophysiologic (EP) manifestations, leading to a cumulative incidence of sudden death of 6.6%. Due to genetic anticipation, there is a pediatric subset of this patient population. However, most EP research on DM1 patients has been in adults, making cardiac care for pediatric patients difficult and directed by adult guidelines which often leads to cardiovascular implantable electronic device (CIED) implants. We sought to investigate the prevalence of CIEDs in the pediatric DM1 population. Methods: The Vizient® Clinical Data Base was queried from October 2019 to October 2023 for admissions with and without ICD-10 code for myotonic dystrophy (G71.11), with and without codes for presence of a pacemaker or ICD (Z95.0, Z95.810). Patients who were identified were stratified by age: Pediatric (0–21 years) and Adult (22–50 years). Results: Prevalence of CIED in pediatric DM1 was 2.1% and in adult DM1 was 15.8%. When comparing to pediatric and adult patients with CIED and without DM1, the odds ratio for CIED in pediatric DM1 was 48.8, compared to 23.3 for CIED in adult DM1. Discussion: There are pediatric DM1 patients who have received CIED despite a lack of data to inform this decision-making. Further research will be important to ensure appropriate use of CIED in this population and to develop appropriate guidelines to direct management.
• Chin, C., Barber, B. J., Samson, R. A., & Glasser, J. (2022). A Prolonged QTc Interval Leads to the Diagnosis of Hyperthyroidism in an Adolescent Boy. The Journal of emergency medicine. doi:10.1016/j.jemermed.2021.12.007
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  Syncope is a common cause of pediatric emergency department visits and carries a broad differential diagnosis, which includes a few rare but critical cardiac conditions.We review the case of an adolescent boy who presented to the emergency department after a syncopal event. He was found to have a prolonged QTc interval on electrocardiogram (ECG), without personal or family history or known risk factors. He was screened for thyroid dysfunction on a second ED visit for presyncope and was subsequently diagnosed with hyperthyroidism. The patient was treated with methimazole for 2 weeks and a repeat ECG showed normalization of the QTc interval with a QTc reduction of more than 100 ms; routine thyroid studies showed correction of thyroid stimulating hormone and free thyroxine levels shortly thereafter. WHY SHOULD AN EMERGENCY PHYSICIAN BE AWARE OF THIS?: This case and review of the medical literature should raise awareness for the emergency physician to consider evaluation of thyroid function in pediatric patients with QT interval prolongation and vice versa, potentially averting dangerous dysrhythmias.
• Bernardi, A. M., Moses, S., Barber, B. J., Witte, M. H., & Seckeler, M. D. (2021). Higher Incidence of Protein-Losing Enteropathy in Patients with Single Systemic Right Ventricle. Pediatric Cardiology, 42(Issue 1). doi:10.1007/s00246-020-02468-y
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  Patients with single ventricle congenital heart disease are at risk of unpredictable protein-losing enteropathy (PLE) after surgical palliation. Based on prior reports of physiologic differences for patients with single morphologic right versus left ventricles, we hypothesized that those with right ventricular morphology would have a higher incidence of PLE. We performed a retrospective review of > 15 million pediatric hospitalizations from the Healthcare Cost and Utilization Project KID 2000–2012 databases for admissions 5–21 years old with ICD-9 codes for hypoplastic left heart syndrome (HLHS) and tricuspid atresia (TA) with and without PLE. Incidence of PLE was compared between those with HLHS and TA. In addition, outcomes and costs were compared between admissions with and without PLE and between HLHS and TA. Of 1623 HLHS admissions, 289 (17.8%) had PLE, and of 926 TA admissions, 58 (5.9%) had PLE (p < 0.001). Admissions with PLE were older compared to those without PLE (12 vs 10 years, p < 0.001) and PLE onset occurred at a younger age for HLHS than TA (11 vs 14 years, p < 0.001). There were no differences in hospital outcomes or costs. Review of this large administrative database suggests a higher incidence of PLE in patients with HLHS and a younger age of onset compared to those with TA. These data suggest that a single systemic right ventricle may be an independent risk factor for developing PLE.
• Seckeler, M. D., Kylat, R. I., Kops, S. A., Bhatia, S., Barber, B. J., & Bader, M. Y. (2021). Genetic Characterization of a Model Ciliopathy: Bardet-Biedl Syndrome.. Journal of pediatric genetics, 10(2), 126-130. doi:10.1055/s-0040-1708844
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  Bardet-Biedl syndrome (BBS) is a rare ciliopathy affecting multiple organ systems. Patients with BBS are usually diagnosed later in childhood when clinical features of the disease become apparent. In this article, we presented a case of BBS discovered by whole genome sequencing in a newborn with heterotaxy, duodenal atresia, and complex congenital heart disease. Early diagnosis is important not only for prognostication but also to explore ways to mitigate the cone-rod dysfunction and for exploring newer therapies. Our case highlights the importance of a high index of suspicion and the utility of advanced genetic testing to provide an early diagnosis for a rare disease.
• Seckeler, M. D., Seckeler, M. D., Boe, B. A., Berman, D. P., Barber, B. J., & Armstrong, A. K. (2021). Use of rotational angiography in congenital cardiac catheterisations to generate three-dimensional-printed models.. Cardiology in the young, 1-5. doi:10.1017/s1047951121000275
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  Three-dimensional printing is increasingly utilised for congenital heart defect procedural planning. CT or MR datasets are typically used for printing, but similar datasets can be obtained from three-dimensional rotational angiography. We sought to assess the feasibility and accuracy of printing three-dimensional models of CHD from rotational angiography datasets..Retrospective review of CHD catheterisations using rotational angiography was performed, and patient and procedural details were collected. Imaging data from rotational angiography were segmented, cleaned, and printed with polylactic acid on a Dremel® 3D Idea Builder (Dremel, Mount Prospect, IL, USA). Printing time and materials' costs were captured. CT scans of printed models were compared objectively to the original virtual models. Two independent, non-interventional paediatric cardiologists provided subjective ratings of the quality and accuracy of the printed models..Rotational angiography data from 15 catheterisations on vascular structures were printed. Median print time was 3.83 hours, and material costs were $2.84. The CT scans of the printed models highly matched with the original digital models (root mean square for Hausdorff distance 0.013 ± 0.003 mesh units). Independent reviewers correctly described 80 and 87% of the models (p = 0.334) and reported high quality and accuracy (5 versus 5, p = NS; κ = 0.615)..Imaging data from rotational angiography can be converted into accurate three-dimensional-printed models of CHD. The cost of printing the models was negligible, but the print time was prohibitive for real-time use. As the speed of three-dimensional printing technology increases, novel future applications may allow for printing patient-specific devices based on rotational angiography datasets.
• Seckeler, M. D., Seckeler, M. D., Klewer, S. E., Hoyer, A. W., Colombo, J. N., Bernardi, A. M., Barber, B. J., & Andrews, J. G. (2021). Exercise Performance in Adolescents With Fontan Physiology (from the Pediatric Heart Network Fontan Public Data Set).. The American journal of cardiology, 149, 119-125. doi:10.1016/j.amjcard.2021.03.018
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  In the pediatric population, exercise capacity differs between females and males and the gap widens through adolescence. However, specific age- and sex-based changes in adolescents with congenital heart disease and Fontan palliation have not been reported. The purpose of the current study is to identify age- and sex-specific changes in exercise performance at peak and ventilatory anaerobic threshold (AT) for adolescents with Fontan physiology. Retrospective review of the Pediatric Heart Network Fontan cross sectional study (Fontan 1) public use dataset. Comparisons were made for peak and AT exercise parameters for females and males at 2-year age intervals. In addition, normative values were generated by sex and age at 2-year intervals. χ2 test was used for comparison for categorical variables. Changes in exercise parameters between age groups by sex were compared by ANOVA with post-hoc analysis. Exercise testing was performed in 411 patients. AT was reached in 317 subjects (40% female), of whom, 166 (43% female) reached peak exercise. Peak oxygen consumption decreased 32% through adolescence in females and did not have the typical increase through adolescence for males. Oxygen consumption at AT also decreased with age in both sexes. In conclusion, age- and sex-based exercise performance for adolescents with Fontan physiology are predictably low, but there are additional significant decreases through adolescence for this population, especially in females. We have established normative exercise values for several parameters for this population which will better identify at risk patients and allow for earlier intervention.
• Bader, M., Barber, B. J., Seckeler, M., Bhatia, S., Kylathu, R., & Kops, S. A. (2020). Genetic Characterization of a Model Ciliopathy: Bardet–Biedl's Syndrome. Journal of Pediatric Genetics. doi:DOI: 10.1055/s-0040-1708844
• Barber, B. J., Bedrick, A. D., Witte, M. H., Mustacich, D., Moedano, L., Bernas, M., Lau-Braunhut, S., Behan, S., Klewer, S. E., Kylat, R. I., & Kuo, P. H. (2020). WHOLE-BODY LYMPHANGIOSCINTIGRAPHY AND SPECT/CT IN CHILDREN WITH LYMPHATIC COMPLICATIONS AFTER SURGERY FOR COMPLEX CONGENITAL HEART DISEASE. Lymphology. doi:10.2458/lymph.4638
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  The number of patients surviving repair of complex congenital heart disease (CCHD) has increased due to improved surgical techniques, post operative management and outpatient care. Likewise, this growing patient population has demonstrated an increasing number and complexity of complications involving the lymphatic system. To evaluate the peripheral and central lymphatic system, whole-body lymphangioscintigraphy (LAS) is considered as the initial imaging evaluation of choice. To date, very few publications exist on the value of lymphatic imaging techniques in infants and small children with lymphatic complications following surgery for congenital heart disease. A retrospective review of medical records from 2008 to 2018 was performed for pediatric patients referred for lymphatic complications after CCHD surgery at an academic medical center. LAS and SPECT/CT was performed using intradermal bipedal injections of Tc 99m labeled filtered sulfur colloid, and in some patients also bilateral hand injections, followed by dynamic imaging and whole- body planar imaging typically up to 180 minutes post injection. Clinical decision making and outcomes were recorded. LAS and SPECT/CT were performed without complication in pediatric patients with prior surgery for CCHD. LAS successfully localized various lymphatic abnormalities such as lymphatic obstruction, reflux, and leaks, which were further delineated by SPECT/CT. LAS findings directed further evaluation with more definitive studies, management and prognosis. Five of the ten patients had follow up outcome data – 2 years and up to 10 years. LAS and SPECT/CT are safe and effective techniques for the initial evaluation of lymphatic abnormalities in pediatric patients with CCHD. LAS, particularly with further 3D localization by SPECT/CT, provides functional imaging of peripheral and central lymphatic flow and thus provides guidance for medical therapy, non operative interventional management, and surgical therapy for these diverse, debilitating, and often life threatening disorders.
• Bernardi, A. M., Moses, S., Barber, B. J., Witte, M. H., & Seckeler, M. D. (2020). Higher Incidence of Protein-Losing Enteropathy in Patients with Single Systemic Right Ventricle. Pediatric cardiology.
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  Patients with single ventricle congenital heart disease are at risk of unpredictable protein-losing enteropathy (PLE) after surgical palliation. Based on prior reports of physiologic differences for patients with single morphologic right versus left ventricles, we hypothesized that those with right ventricular morphology would have a higher incidence of PLE. We performed a retrospective review of > 15 million pediatric hospitalizations from the Healthcare Cost and Utilization Project KID 2000-2012 databases for admissions 5-21 years old with ICD-9 codes for hypoplastic left heart syndrome (HLHS) and tricuspid atresia (TA) with and without PLE. Incidence of PLE was compared between those with HLHS and TA. In addition, outcomes and costs were compared between admissions with and without PLE and between HLHS and TA. Of 1623 HLHS admissions, 289 (17.8%) had PLE, and of 926 TA admissions, 58 (5.9%) had PLE (p 
• Combs, D., Edgin, J. O., Klewer, S., Barber, B. J., Morgan, W. J., Hsu, C. H., Abraham, I., & Parthasarathy, S. (2020). OSA and Neurocognitive Impairment in Children With Congenital Heart Disease. Chest, 158(3), 1208-1217.
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  Children with congenital heart disease (CHD) have an increased risk of neurocognitive impairment. No prior studies have evaluated the role of OSA, which is associated with neurocognitive impairment in children without CHD.
• Kops, S. A., Pangburn, S., Barber, B. J., & Seckeler, M. D. (2020). Transcatheter treatment of acquired coronary sinus ostium atresia in a child with complex congenital heart disease. Catheterization and Cardiovascular Interventions, 95(Issue 2). doi:10.1002/ccd.28489
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  Congenital coronary sinus ostium atresia is a rare condition that is typically considered benign, as long as there is adequate drainage via another route. However, in children with single ventricle congenital heart disease, adequate drainage may not be assured after complex surgical interventions, putting them at risk for myocardial injury. We present a patient with complex single ventricle congenital heart disease who developed acquired coronary sinus ostium atresia after surgical intervention that was treated by a transcatheter approach to prevent the sequelae of coronary sinus hypertension.
• Kops, S. A., Pangburn, S., Barber, B. J., & Seckeler, M. D. (2020). Transcatheter treatment of acquired coronary sinus ostium atresia in a child with complex congenital heart disease. Catheterization and cardiovascular interventions : official journal of the Society for Cardiac Angiography & Interventions, 95(2), E62-E65.
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  Congenital coronary sinus ostium atresia is a rare condition that is typically considered benign, as long as there is adequate drainage via another route. However, in children with single ventricle congenital heart disease, adequate drainage may not be assured after complex surgical interventions, putting them at risk for myocardial injury. We present a patient with complex single ventricle congenital heart disease who developed acquired coronary sinus ostium atresia after surgical intervention that was treated by a transcatheter approach to prevent the sequelae of coronary sinus hypertension.
• Kurland, Y., Kylat, R. I., Johnson, D. C., Barber, B. J., Bedrick, A. D., & Bader, M. Y. (2020). Intravenous Immunoglobulin for Congenital Parvovirus Myocarditis. Journal of Pediatric Infectious Diseases, 15(Issue 1). doi:10.1055/s-0037-1620249
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  Congenital parvovirus infection has a diverse presentation ranging from asymptomatic infants to intrauterine demise secondary to red cell aplasia or myocarditis. Treatment is aimed at correcting anemia with intrauterine and postnatal transfusions. We report a case of fetal hydrops with severe atrioventricular regurgitation and myocardial dysfunction secondary to parvovirus infection in a preterm infant. Myocarditis and myocardial dysfunction responded to immunoglobulin administration.
• Seckeler, M. D., Parthasarathy, S., Morgan, W. J., Klewer, S. E., Hsu, C. H., Fernandez, V., Combs, D., Barber, B. J., & Andrews, J. (2020). Abstract 14771: Obstructive Sleep Apnea is Associated With Cardiac Dysfunction in Children With Congenital Heart Disease. Circulation, 142. doi:10.1161/circ.142.suppl_3.14771
• Seckeler, M., Witte, M. H., Barber, B. J., Moses, S., & Bernardi, A. (2020). Higher Incidence of Protein-Losing Enteropathy in Patients with Single Systemic Right Ventricle. American Journal of Cardiology. doi:10.1007/s00246-020-02468-y
• Ghishan, F. K., Dori, Y., Barber, B. J., Witte, M. H., & Kylat, R. (2019). Resolution of protein-losing enteropathy after congenital heart disease repair by selective lymphatic embolization. Pediatr Gastroenterol Hepatol Nutr, 22(6), 1-7.
• Kuo, P. H., Barber, B. J., Kylat, R. I., Klewer, S. E., Behan, S., Lau-Braunhut, S., Bernas, M. J., Moedano, L., Bedrick, A. D., Mustacich, D. J., & Witte, M. H. (2019). Whole-body lymphangioscintigraphy and SPECT/CT in children with lymphatic complications after surgery for complex congenital heart disease. Lymphology, 52(4), 157-165.
  More info

  The number of patients surviving repair of complex congenital heart disease (CCHD) has increased due to improved surgical techniques, post operative management and outpatient care. Likewise, this growing patient population has demonstrated an increasing number and complexity of complications involving the lymphatic system. To evaluate the peripheral and central lymphatic system, whole-body lymphangioscintigraphy (LAS) is considered as the initial imaging evaluation of choice. To date, very few publications exist on the value of lymphatic imaging techniques in infants and small children with lymphatic complications following surgery for congenital heart disease. A retrospective review of medical records from 2008 to 2018 was performed for pediatric patients referred for lymphatic complications after CCHD surgery at an academic medical center. LAS and SPECT/CT was performed using intradermal bipedal injections of Tc 99m labeled filtered sulfur colloid, and in some patients also bilateral hand injections, followed by dynamic imaging and whole- body planar imaging typically up to 180 minutes post injection. Clinical decision making and outcomes were recorded. LAS and SPECT/CT were performed without complication in pediatric patients with prior surgery for CCHD. LAS successfully localized various lymphatic abnormalities such as lymphatic obstruction, reflux, and leaks, which were further delineated by SPECT/CT. LAS findings directed further evaluation with more definitive studies, management and prognosis. Five of the ten patients had follow up outcome data - 2 years and up to 10 years. LAS and SPECT/CT are safe and effective techniques for the initial evaluation of lymphatic abnormalities in pediatric patients with CCHD. LAS, particularly with further 3D localization by SPECT/CT, provides functional imaging of peripheral and central lymphatic flow and thus provides guidance for medical therapy, non operative interventional management, and surgical therapy for these diverse, debilitating, and often life threatening disorders.
• Kuo, P. H., Barber, B. J., Kylat, R. I., Klewer, S. E., Behan, S., Lau-Braunhut, S., Bernas, M. J., Moedano, L., Bedrick, A. D., Mustacich, D. J., & Witte, M. H. (2019). Whole-body lymphangioscintigraphy and SPECT/CT in children with lymphatic complications after surgery for complex congenital heart disease. Lymphology, 52(Issue 4).
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  The number of patients surviving repair of complex congenital heart disease (CCHD) has increased due to improved surgical techniques, post operative management and outpatient care. Likewise, this growing patient population has demonstrated an increasing number and complexity of complications involving the lymphatic system. To evaluate the peripheral and central lymphatic system, whole-body lymphangioscintigraphy (LAS) is considered as the initial imaging evaluation of choice. To date, very few publications exist on the value of lymphatic imaging techniques in infants and small children with lymphatic complications following surgery for congenital heart disease. A retrospective review of medical records from 2008 to 2018 was performed for pediatric patients referred for lymphatic complications after CCHD surgery at an academic medical center. LAS and SPECT/CT was performed using intradermal bipedal injections of Tc 99m labeled filtered sulfur colloid, and in some patients also bilateral hand injections, followed by dynamic imaging and whole- body planar imaging typically up to 180 minutes post injection. Clinical decision making and outcomes were recorded. LAS and SPECT/CT were performed without complication in pediatric patients with prior surgery for CCHD. LAS successfully localized various lymphatic abnormalities such as lymphatic obstruction, reflux, and leaks, which were further delineated by SPECT/CT. LAS findings directed further evaluation with more definitive studies, management and prognosis. Five of the ten patients had follow up outcome data - 2 years and up to 10 years. LAS and SPECT/CT are safe and effective techniques for the initial evaluation of lymphatic abnormalities in pediatric patients with CCHD. LAS, particularly with further 3D localization by SPECT/CT, provides functional imaging of peripheral and central lymphatic flow and thus provides guidance for medical therapy, non operative interventional management, and surgical therapy for these diverse, debilitating, and often life threatening disorders.
• Kylat, R. I., Witte, M. H., Barber, B. J., Dori, Y., & Ghishan, F. K. (2019). Resolution of Protein-Losing Enteropathy after Congenital Heart Disease Repair by Selective Lymphatic Embolization. Pediatric gastroenterology, hepatology & nutrition, 22(6), 594-600.
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  With improving survival of children with complex congenital heart disease (CCHD), postoperative complications, like protein-losing enteropathy (PLE) are increasingly encountered. A 3-year-old girl with surgically corrected CCHD (ventricular inversion/L-transposition of the great arteries, ventricular septal defect, pulmonary atresia, post-double switch procedure [Rastelli and Glenn]) developed chylothoraces. She was treated with pleurodesis, thoracic duct ligation and subsequently developed chylous ascites and PLE (serum albumin ≤0.9 g/dL) and was malnourished, despite nutritional rehabilitation. Lymphangioscintigraphy/single-photon emission computed tomography showed lymphatic obstruction at the cisterna chyli level. A segmental chyle leak and chylous lymphangiectasia were confirmed by gastrointestinal endoscopy, magnetic resonance (MR) enterography, and MR lymphangiography. Selective glue embolization of leaking intestinal lymphatic trunks led to prompt reversal of PLE. Serum albumin level and weight gain markedly improved and have been maintained for over 3 years. Selective interventional embolization reversed this devastating lymphatic complication of surgically corrected CCHD.
• Kylat, R. I., Witte, M. H., Barber, B. J., Dori, Y., & Ghishan, F. K. (2019). Resolution of protein-losing enteropathy after congenital heart disease repair by selective lymphatic embolization. Pediatric Gastroenterology, Hepatology and Nutrition, 22(Issue 6). doi:10.5223/pghn.2019.22.6.594
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  With improving survival of children with complex congenital heart disease (CCHD), postoperative complications, like protein-losing enteropathy (PLE) are increasingly encountered. A 3-year-old girl with surgically corrected CCHD (ventricular inversion/L-transposition of the great arteries, ventricular septal defect, pulmonary atresia, post-double switch procedure [Rastelli and Glenn]) developed chylothoraces. She was treated with pleurodesis, thoracic duct ligation and subsequently developed chylous ascites and PLE (serum albumin ≤0.9 g/dL) and was malnourished, despite nutritional rehabilitation. Lymphangioscintigraphy/single-photon emission computed tomography showed lymphatic obstruction at the cisterna chyli level. A segmental chyle leak and chylous lymphangiectasia were confirmed by gastrointestinal endoscopy, magnetic resonance (MR) enterography, and MR lymphangiography. Selective glue embolization of leaking intestinal lymphatic trunks led to prompt reversal of PLE. Serum albumin level and weight gain markedly improved and have been maintained for over 3 years. Selective interventional embolization reversed this devastating lymphatic complication of surgically corrected CCHD.
• Tefera, E., Gedlu, E., Nega, B., Tadesse, B. T., Chanie, Y., Dawoud, A., Moges, F. H., Bezabih, A., Moges, T., Centella, T., Marianeschi, S., Coca, A., Collado, R., Kassa, M. W., Johansson, S., van Doorn, C., Barber, B. J., & Teodori, M. (2019). Factors associated with perioperative mortality in children and adolescents operated for tetralogy of Fallot: A sub-Saharan experience. Journal of Cardiac Surgery, 34(Issue 12). doi:10.1111/jocs.14270
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  Background: Patients with tetralogy of Fallot are now surviving to adulthood with timely surgical intervention. However, many patients in low-income countries have no access to surgical intervention. This paper reports the surgical access and perioperative mortality in a sub-Saharan center that was mainly dependent on visiting teams. Methods: We reviewed records of patients operated from January 2009 to December 2014. We examined perioperative outcomes, primarily focusing on factors associated with perioperative mortality. Results: During this period, 62 patients underwent surgery. Fifty-seven (91.9%) underwent primary repair, while 5 (6.5%) underwent palliative shunt surgery. Of the five patients with shunt surgery, four ultimately underwent total repair. Eight (12.9%) patients died during the perioperative period. Factors associated with perioperative mortality include repeated preoperative phlebotomy procedures (P
• Tefera, E., Gedlu, E., Nega, B., Tadesse, B. T., Chanie, Y., Dawoud, A., Moges, F. H., Bezabih, A., Moges, T., Centella, T., Marianeschi, S., Coca, A., Collado, R., Kassa, M. W., Johansson, S., van Doorn, C., Barber, B. J., & Teodori, M. (2019). Factors associated with perioperative mortality in children and adolescents operated for tetralogy of Fallot: A sub-Saharan experience. Journal of cardiac surgery, 34(12), 1478-1485.
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  Patients with tetralogy of Fallot are now surviving to adulthood with timely surgical intervention. However, many patients in low-income countries have no access to surgical intervention. This paper reports the surgical access and perioperative mortality in a sub-Saharan center that was mainly dependent on visiting teams.
• Witte, M. H., Mustacich, D., Moedano, L., Behan, S., Bernas, M. J., Klewer, S. E., Barber, B. J., Kuo, P. H., & Kylathu, R. (2019). Whole Body Lymphangioscintigraphy and SPECT-CT in Infants and Children with Lymphatic Congestion after Surgical Repair of Complex Congenital Heart Disease. 27th World Congress of Lymphology Abstract Booklet.
• Bader, M., Barber, B., Dudani, R., Johnson, D., & Kurland, Y. (2018). Congenital Parvovirus Myocarditis treated with IVIG. Pediatrics, 142(1_MeetingAbstract), 185-185. doi:10.1542/peds.142.1ma2.185
• Combs, D., Morgan, W. J., Abraham, I., Parthasarathy, S., Parthasarathy, S., Morgan, W. J., Hsu, C. H., Edgin, J. O., Combs, D., Barber, B. J., & Abraham, I. (2018). 0745 Sleep-disordered Breathing Is Associated With Neurocognitive Impairment In Children With Congenital Heart Disease. Sleep, 41(suppl_1), A277-A277. doi:10.1093/sleep/zsy061.744
• Combs, D., Skrepnek, G., Seckeler, M. D., Barber, B. J., Morgan, W. J., & Parthasarathy, S. (2018). Sleep-Disordered Breathing is Associated With Increased Mortality in Hospitalized Infants With Congenital Heart Disease. Journal of clinical sleep medicine : JCSM : official publication of the American Academy of Sleep Medicine, 14(9), 1551-1558.
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  Sleep-disordered breathing (SDB) has adverse cardiovascular effects in children and adults. In adults with cardiac disease, SDB is highly prevalent and confers increased mortality risk. It is unknown if SDB confers a similar risk in infants with congenital heart disease (CHD). We evaluated clinical and economic outcomes associated with SDB among inpatient infants with CHD in the United States from 1997-2012.
• Combs, D., Skrepnek, G., Seckeler, M. D., Barber, B. J., Morgan, W. J., & Parthasarathy, S. (2018). Sleep-disordered breathing is associated with increased mortality in hospitalized infants with congenital heart disease. Journal of Clinical Sleep Medicine, 14(Issue 9). doi:10.5664/jcsm.7334
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  Study Objectives: Sleep-disordered breathing (SDB) has adverse cardiovascular effects in children and adults. In adults with cardiac disease, SDB is highly prevalent and confers increased mortality risk. It is unknown if SDB confers a similar risk in infants with congenital heart disease (CHD). We evaluated clinical and economic outcomes associated with SDB among inpatient infants with CHD in the United States from 1997–2012. Methods: This retrospective, cross-sectional study used discharge data from the Kids’ Inpatient Database. Inclusion criteria included diagnosed CHD and age younger than 1 year. Exclusion criteria included apnea of prematurity, cardiac surgery during admission, and invasive mechanical ventilation. Generalized linear models were used to assess outcomes of mortality, length of stay, and total charges after controlling for SDB, clinical characteristics, hospital characteristics, and economic factors. Results: Across 461,778 inpatient infant cases of CHD from 1997–2012, 4,839 involved SDB (14% obstructive, 4% central, 82% not specified). Multivariable analyses show that central sleep apnea was independently associated with increased risk of inpatient mortality (odds ratio 4.3), 92% longer inpatient stay, and 112% higher total charges when compared to infants with CHD without comorbid SDB (P < .05). Obstructive and unspecified SDB were associated with longer adjusted lengths of stay (56% and 18%, respectively) and higher charges (48% and 21%, respectively) relative to infants with CHD without comorbid SDB (P < .001). Conclusions: SDB, particularly central sleep apnea, was independently associated with worse outcomes in hospitalized infants with CHD. Further research on whether treatment of SDB in infants with CHD can abrogate adverse patient outcomes is needed.
• Abdy, N. A., Valdes, S. O., Sorrell, V. L., Klewer, S. E., & Barber, B. J. (2017). Apical hypertrophic cardiomyopathy in an adolescent. Congenital heart disease, 5(2), 182-7.
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  To our knowledge, this is one of the few reported cases of apical hypertrophic cardiomyopathy in an adolescent patient in the United States. We describe a clinical presentation of an adolescent male who presented for cardiac evaluation and was found to have an apical variant of hypertrophic cardiomyopathy.
• Barber, B. J., Burch, G. H., Tripple, D., & Balaji, S. (2017). Resolution of plastic bronchitis with atrial pacing in a patient with fontan physiology. Pediatric cardiology, 25(1), 73-6.
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  We describe a 5-year-old boy with Fontan physiology and a ventricular pacemaker who developed severe plastic bronchitis. Evaluation by cardiac catheterization revealed profoundly altered hemodynamics, which improved with atrial pacing. Following implantation of an atrial pacemaker, which restored atrioventricular (A-V) synchrony, the patient's hemodynamics greatly improved and his plastic bronchitis resolved.
• Barber, B. J., Donnerstein, R. L., Secomb, T. W., Pogreba-Brown, K., Steelman, R., Ellenby, M. S., Shen, I., & Ungerleider, R. M. (2017). The dicrotic pulse: a common, non-ominous finding after the Ross operation. Pediatric cardiology, 28(4), 247-9.
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  We noted a dicrotic pulse in several patients following a Ross operation. Although the etiology of this unique arterial waveform is not completely understood, it has been reported as a sign of low cardiac output and a poor prognosis. We reviewed preoperative echocardiograms and postoperative radial arterial pressure tracings in 33 patients who underwent a Ross procedure between 2000 and 2004. We found a dicrotic pulse to occur commonly (20/33; 61%) following a Ross operation. Moderate to severe preoperative aortic insufficiency was present in 19/20 patients (95%) in whom a dicrotic pulse was noted and in only 3/13 (23%) who did not exhibit a postoperative dicrotic pulse (p
• Hainstock, M. R., Gruchala, N. E., Fike, N., Samson, R. A., Klewer, S. E., & Barber, B. J. (2017). Postural orthostatic tachycardia in a teenager with Klinefelter syndrome. Congenital heart disease, 3(6), 440-2.
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  To our knowledge this is the first reported case of postural orthostatic tachycardia syndrome (POTS) in a patient with Klinefelter syndrome. We describe a classic clinical presentation of POTS in an adolescent male with Klinefelter syndrome. Although the etiology of POTS appears to be multifactorial, there is a strong female predominance that suggests a genetic basis. Our patient with Klinefelter syndrome may further support a link with POTS to the X chromosome.
• Ihnat, C. L., Zimmerman, H., Copeland, J. G., Meaney, F. J., Sobonya, R. E., Larsen, B. T., Blair, B., Lax, D., & Barber, B. J. (2017). Left ventricular assist device support as a bridge to recovery in young children. Congenital heart disease, 6(3), 234-40.
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  Left ventricular assist device (LVAD) experience and follow-up data in children are limited. We report the deployment and successful weaning from LVAD in young children with severe heart failure (HF).
• Kim, S., Zhu, Y., Romitti, P. A., Fox, D. J., Sheehan, D. W., Valdez, R., Matthews, D., & Barber, B. J. (2017). Associations between timing of corticosteroid treatment initiation and clinical outcomes in Duchenne muscular dystrophy. Neuromuscular Disorders, 27(Issue 8). doi:10.1016/j.nmd.2017.05.019
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  The long-term efficacy of corticosteroid treatment and timing of treatment initiation among Duchenne muscular dystrophy (DMD) patients is not well-understood. We used data from a longitudinal, population-based DMD surveillance program to examine associations between timing of treatment initiation (early childhood [before or at age 5 years], late childhood [after age 5 years], and naïve [not treated]) and five clinical outcomes (age at loss of ambulation; ages at onset of cardiomyopathy, scoliosis, and first fracture; and pulmonary function). Hazard ratios (HRs) and 95% confidence intervals (CIs) were estimated using survival analysis. DMD patients who initiated corticosteroid treatment in early childhood had a higher risk of earlier onset cardiomyopathy compared to cases who initiated treatment in late childhood (HR = 2.0, 95% CI = [1.2, 3.4]) or treatment naïve patients (HR = 1.9, 95% CI = [1.1, 3.2]), and higher risk of suffering a fracture (HR = 2.3, 95% CI = [1.4, 3.7] and HR = 2.6, 95% CI = [1.6, 4.2], respectively). Patients with early childhood treatment had slightly decreased respiratory function compared with those with late childhood treatment. Ages at loss of ambulation or scoliosis diagnosis did not differ statistically among treatment groups. We caution that the results from our study are subject to several limitations, as they were based on data abstracted from medical records. Further investigations using improved reporting of disease onset and outcomes are warranted to obtain a more definitive assessment of the association between the timing of corticosteroid treatment and disease severity.
• Kim, S., Zhu, Y., Romitti, P. A., Fox, D. J., Sheehan, D. W., Valdez, R., Matthews, D., Barber, B. J., & , M. S. (2017). Associations between timing of corticosteroid treatment initiation and clinical outcomes in Duchenne muscular dystrophy. Neuromuscular disorders : NMD, 27(8), 730-737.
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  The long-term efficacy of corticosteroid treatment and timing of treatment initiation among Duchenne muscular dystrophy (DMD) patients is not well-understood. We used data from a longitudinal, population-based DMD surveillance program to examine associations between timing of treatment initiation (early childhood [before or at age 5 years], late childhood [after age 5 years], and naïve [not treated]) and five clinical outcomes (age at loss of ambulation; ages at onset of cardiomyopathy, scoliosis, and first fracture; and pulmonary function). Hazard ratios (HRs) and 95% confidence intervals (CIs) were estimated using survival analysis. DMD patients who initiated corticosteroid treatment in early childhood had a higher risk of earlier onset cardiomyopathy compared to cases who initiated treatment in late childhood (HR = 2.0, 95% CI = [1.2, 3.4]) or treatment naïve patients (HR = 1.9, 95% CI = [1.1, 3.2]), and higher risk of suffering a fracture (HR = 2.3, 95% CI = [1.4, 3.7] and HR = 2.6, 95% CI = [1.6, 4.2], respectively). Patients with early childhood treatment had slightly decreased respiratory function compared with those with late childhood treatment. Ages at loss of ambulation or scoliosis diagnosis did not differ statistically among treatment groups. We caution that the results from our study are subject to several limitations, as they were based on data abstracted from medical records. Further investigations using improved reporting of disease onset and outcomes are warranted to obtain a more definitive assessment of the association between the timing of corticosteroid treatment and disease severity.
• Kylat, R. I., Johnson, D., Bedrick, A. D., Barber, B. J., Kurland, Y., & Bader, M. (2017). Intravenous Immunoglobulin for congenital Parvovirus myocarditis. Journal of Pediatric Infectious diseases. doi:10.1055/s-0037-1620249
• Kylathu, R., Kylathu, R., Johnson, D., Johnson, D., Bedrick, A. D., Bedrick, A. D., Barber, B. J., Barber, B. J., Kurland, Y., Kurland, Y., Bader, M., & Bader, M. (2017). Intravenous Immunoglobulin for congenital Parvovirus myocarditis. Journal of Pediatric Infectious diseases.
• Seckeler, M. D., Lawson, E., Barber, B. J., & Klewer, S. E. (2017). Percutaneous management of complex acquired aortic coarctation in an adult with tetralogy of Fallot and pulmonary atresia. Annals of Pediatric Cardiology, 10(Issue 3). doi:10.4103/apc.apc_19_17
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  We present the case of a female adult with complex cyanotic congenital heart disease who had long-standing thoracic aortic obstruction due to scarring from earlier surgical procedures. She was symptomatic but felt to be too high risk for surgical intervention. With careful planning, she was able to undergo successful stenting of her aorta with subsequent clinical improvement. This case highlights some of the complexities of caring for adults with congenital heart disease and the importance of a thorough understanding of their anatomy and physiology and prior interventions before undertaking interventions.
• Colombo, J. N., Seckeler, M. D., Barber, B. J., Krupinski, E. A., Weinstein, R. S., Sisk, D., & Lax, D. (2016). Application and Utility of iPads in Pediatric Tele-echocardiography. Telemedicine and e-Health, 22(Issue 5). doi:10.1089/tmj.2015.0114
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  Background: Telemedicine is used with increasing frequency to improve patient care in remote areas. The interpretation of medical imaging on iPad® (Apple, Cupertino, CA) tablets has been reported to be accurate. There are no studies on the use of iPads for interpretation of pediatric echocardiograms. We compared the quality of echo images, diagnostic accuracy, and review time using three different modalities: remote access on an iPad Air (iPad), remote access via a computer (Remote), and direct access on a computer linked through Ethernet to the server, the "gold standard" (Direct). Materials and Methods: Fifty consecutive archived pediatric echocardiograms were interpreted using the three modalities. Studies were analyzed blindly by three pediatric cardiologists; review time, diagnostic accuracy, and image quality were documented. Diagnostic accuracy was assessed by comparing the study diagnoses with the official diagnosis in the patient's chart. Discrepancies between diagnoses were graded as major (more than one grade difference) or minor (one grade difference in severity of lesion). Results: There were no significant differences in accuracy among the three modalities. There was one major discrepancy (size of patent ductus arteriosus); all others were minor, hemodynamically insignificant. Image quality ratings were better for iPad than Remote; Direct had the highest ratings. Review times (mean [standard deviation] minutes) were longest for iPad (5.89 [3.87]) and then Remote (4.72 [2.69]), with Direct having the shortest times (3.52 [1.42]) (p < 0.0001). Conclusions: Pediatric echocardiograms can be interpreted using convenient, portable devices while preserving accuracy and quality with slightly longer review times (1-2 min). These findings are important in the current era of increasing need for mobile health.
• Colombo, J. N., Seckeler, M. D., Barber, B. J., Krupinski, E. A., Weinstein, R. S., Sisk, D., & Lax, D. (2016). Application and Utility of iPads in Pediatric Tele-echocardiography. Telemedicine journal and e-health : the official journal of the American Telemedicine Association, 22(5), 429-33.
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  Telemedicine is used with increasing frequency to improve patient care in remote areas. The interpretation of medical imaging on iPad(®) (Apple, Cupertino, CA) tablets has been reported to be accurate. There are no studies on the use of iPads for interpretation of pediatric echocardiograms. We compared the quality of echo images, diagnostic accuracy, and review time using three different modalities: remote access on an iPad Air (iPad), remote access via a computer (Remote), and direct access on a computer linked through Ethernet to the server, the "gold standard" (Direct).
• Seckeler, M. D., Zahedieh, S., Seckeler, M. D., Scherer, K., Klewer, S. E., Daines, C. L., Barber, B. J., & Andrews, J. (2016). Regional and Racial Variation in Hospitalization Costs in Patients with Duchenne Muscular Dystrophy. Pediatrics, 140, 28-28. doi:10.1542/peds.140.1_meetingabstract.28
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  Advances in management for Duchenne muscular dystrophy (DMD) have improved survival. The purpose of this study was to describe hospital outcomes and costs for DMD patients and to identify regional and racial variation. Retrospective review of University Health System Consortium Clinical Data Base/Resource Manager, a …
• Hill, M. G., Sekhon, M. K., Reed, K. L., Anderson, C. F., Borjon, N. D., Tardiff, J. C., & Barber, B. J. (2015). Intrauterine Treatment of a Fetus with Familial Hypertrophic Cardiomyopathy Secondary to MYH7 Mutation. Pediatric Cardiology, 36(Issue 8). doi:10.1007/s00246-015-1250-1
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  There is no clear consensus on optimal management of fetuses affected by familial hypertrophic cardiomyopathy (HCM). Intrauterine treatment of the condition has not been attempted in any standardized fashion. We report the case of a fetus treated by maternal propranolol during the third trimester after septal hypertrophy and diastolic dysfunction was diagnosed on fetal echocardiogram. The pregnancy went successfully to term, and fetal septal hypertrophy was noted to improve prior to delivery.
• Hill, M. G., Sekhon, M. K., Reed, K. L., Anderson, C. F., Borjon, N. D., Tardiff, J. C., & Barber, B. J. (2015). Intrauterine Treatment of a Fetus with Familial Hypertrophic Cardiomyopathy Secondary to MYH7 Mutation. Pediatric cardiology, 36(8), 1774-7.
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  There is no clear consensus on optimal management of fetuses affected by familial hypertrophic cardiomyopathy (HCM). Intrauterine treatment of the condition has not been attempted in any standardized fashion. We report the case of a fetus treated by maternal propranolol during the third trimester after septal hypertrophy and diastolic dysfunction was diagnosed on fetal echocardiogram. The pregnancy went successfully to term, and fetal septal hypertrophy was noted to improve prior to delivery.
• Seckeler, M. D., Lawson, E., Barber, B. J., & Klewer, S. E. (2017). Percutaneous management of complex acquired aortic coarctation in an adult with tetralogy of Fallot and pulmonary atresia. Annals of pediatric cardiology, 10(3), 295-297.
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  We present the case of a female adult with complex cyanotic congenital heart disease who had long-standing thoracic aortic obstruction due to scarring from earlier surgical procedures. She was symptomatic but felt to be too high risk for surgical intervention. With careful planning, she was able to undergo successful stenting of her aorta with subsequent clinical improvement. This case highlights some of the complexities of caring for adults with congenital heart disease and the importance of a thorough understanding of their anatomy and physiology and prior interventions before undertaking interventions.
• Colombo, J. N., Seckeler, M. D., Krupinski, E. A., Weinstein, R. S., Lax, D., Weinstein, R. S., Sisk, D., Seckeler, M. D., Lax, D., Krupinski, E. A., Colombo, J. N., & Barber, B. J. (2014). Abstract 16748: Application and Utility of iPads in Pediatric Tele-Echocardiography. Circulation, 130.
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  The remote acquisition and interpretation of medical information (telemedicine) is an integral part of health care. Radiologic images (eg CT scans) can be accurately interpreted on tablet devices with high display resolution. To date, there have been no published studies on the use of iPads for interpretation of pediatric echocardiograms (echo). OBJECTIVES: We evaluated and compared the quality of echo images, diagnostic accuracy, and review time for pediatric echos using 3 different modalities: remote access on an iPad Air (iPad), remote access via computer (Remote), and direct access on computer Ethernet linked to the server (Direct). METHODS: Fifty archived pediatric echos were re-interpreted using the 3 modalities. Studies were analyzed blindly and independently by two pediatric cardiologists. Outcome measures included: time to review, diagnoses and discrepancies, image quality, sharpness, and jitter. Diagnostic accuracy was assessed by comparing the study diagnoses with the original interpretation in...
• Barber, B. J., Andrews, J. G., Lu, Z., West, N. A., Meaney, F. J., Price, E. T., Gray, A., Sheehan, D. W., Pandya, S., Yang, M., & Cunniff, C. (2013). Oral corticosteroids and onset of cardiomyopathy in Duchenne muscular dystrophy. Journal of Pediatrics, 163(Issue 4). doi:10.1016/j.jpeds.2013.05.060
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  Objective To estimate the age when cardiomyopathy develops in boys with Duchenne muscular dystrophy (DMD) and to analyze the effect of corticosteroid treatment on the age of cardiomyopathy onset. Study design We identified a population-based sample of 462 boys with DMD, born between 1982 and 2005, in 5 surveillance sites in the US. Echocardiographic and corticosteroid treatment data were collected. Cardiomyopathy was defined by a reduced fractional shortening (
• Barber, B. J., Andrews, J. G., Lu, Z., West, N. A., Meaney, F. J., Price, E. T., Gray, A., Sheehan, D. W., Pandya, S., Yang, M., & Cunniff, C. (2013). Oral corticosteroids and onset of cardiomyopathy in Duchenne muscular dystrophy. The Journal of pediatrics, 163(4), 1080-4.e1.
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  To estimate the age when cardiomyopathy develops in boys with Duchenne muscular dystrophy (DMD) and to analyze the effect of corticosteroid treatment on the age of cardiomyopathy onset.
• Han, L. M., Klewer, S. E., Blank, K. M., Seckeler, M. D., & Barber, B. J. (2013). Feasibility of pulse oximetry screening for critical congenital heart disease at 2643-foot elevation. Pediatric Cardiology, 34(Issue 8). doi:10.1007/s00246-013-0716-2
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  To evaluate the feasibility of implementing a pulse oximetry screening protocol at a city of mild elevation with a specific focus on the false-positive screening rate. Pulse oximetry screening was performed according to the proposed guidelines endorsed by the American Academy of Pediatrics at a center in Tucson, AZ, at an elevation of 2,643 ft (806 m). During a 10-month period in 2012, 1069 full-term asymptomatic newborns were screened ≥24 h after birth. The mean preductal oxygen saturation was 98.5 ± 1.3 % (range 92-100 %), and the mean postductal oxygen saturation was 98.6 ± 1.3 % (range 94-100 %). Of 1,069 patients screened, 7 were excluded secondary to protocol violations, and 1 screened positive. An echocardiogram was performed on the newborn with the positive screen, and it was normal with the exception of right-to-left shunting across a patent foramen ovale. The false-positive rate was 1/1,062 or 0.094 %. The pulse oximetry screening guidelines recommended by the American Academy of Pediatrics are feasible at an elevation of 2,643 ft (806 m) with a low false-positive rate. Adjustments to the protocol are not required for centers at elevations ≤2,643 ft. Future studies at greater elevations are warranted. © 2013 Springer Science+Business Media New York.
• Han, L. M., Klewer, S. E., Blank, K. M., Seckeler, M. D., & Barber, B. J. (2013). Feasibility of pulse oximetry screening for critical congenital heart disease at 2643-foot elevation. Pediatric cardiology, 34(8), 1803-7.
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  To evaluate the feasibility of implementing a pulse oximetry screening protocol at a city of mild elevation with a specific focus on the false-positive screening rate. Pulse oximetry screening was performed according to the proposed guidelines endorsed by the American Academy of Pediatrics at a center in Tucson, AZ, at an elevation of 2,643 ft (806 m). During a 10-month period in 2012, 1069 full-term asymptomatic newborns were screened ≥ 24 h after birth. The mean preductal oxygen saturation was 98.5 ± 1.3 % (range 92-100 %), and the mean postductal oxygen saturation was 98.6 ± 1.3 % (range 94-100 %). Of 1,069 patients screened, 7 were excluded secondary to protocol violations, and 1 screened positive. An echocardiogram was performed on the newborn with the positive screen, and it was normal with the exception of right-to-left shunting across a patent foramen ovale. The false-positive rate was 1/1,062 or 0.094 %. The pulse oximetry screening guidelines recommended by the American Academy of Pediatrics are feasible at an elevation of 2,643 ft (806 m) with a low false-positive rate. Adjustments to the protocol are not required for centers at elevations ≤ 2,643 ft. Future studies at greater elevations are warranted.
• Haley, J. E., Klewer, S. E., Barber, B. J., Meaney, F. J., Donnerstein, R. L., Weinstein, R. S., Krupinski, E., Warda, G., Lopez, A. M., & Lax, D. (2012). Remote diagnosis of congenital heart disease in Southern Arizona: Comparison between tele-echocardiography and videotapes. Telemedicine and e-Health, 18(Issue 10). doi:10.1089/tmj.2012.0037
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  Objective: We report our experience with tele-echocardiography and echocardiograms recorded digitally or on videotape (recorded-echos) at The University of Arizona from August 2006 to December 2010 and compare their quality and diagnostic accuracy. Materials and Methods: Tele-echocardiograms (tele-echos) were transmitted from the Yuma Regional Medical Center to The University of Arizona via a T-1 and aT-3 line at a bandwidth of 768 kilobits per second. Recorded-echos were shipped for interpretation to The University of Arizona by overnight mail. Diagnostic accuracy was assessed by comparing tele- and recorded-echos with electrocardiograms performed by a pediatric cardiologist (PedsCard-echos). Results: Three hundred forty-six tele-echos in 260 patients and 455 recorded-echos in 406 patients were performed (median age, 6 and 8 days, respectively). Indications included possible congenital heart disease (CHD), patent ductus arteriosus (PDA), and persistent pulmonary hypertension of the newborn. Diagnostic categories included complex CHD, non-critical disease, PDA, and other. PedsCard-echos were available for 27% of the tele-echo and 30% of the recorded-echo patients. Comparisons between tele- and PedsCard-echo yielded no discrepancies in 12 (23%), expected resolution of condition in 26 (49%), and minor in 14 (26%). One (2%) major discrepancy was detected. Comparisons between recorded- and PedsCard-echo showed no discrepancies in 28 (40%), expected resolution of condition in 14 (20%), and minor discrepancies in 28 (40%) patients. No significant difference with respect to discrepancies was detected between tele- and recorded-echos. There was significant (p
• Haley, J. E., Klewer, S. E., Barber, B. J., Meaney, F. J., Donnerstein, R. L., Weinstein, R. S., Krupinski, E., Warda, G., Lopez, A. M., & Lax, D. (2012). Remote diagnosis of congenital heart disease in southern Arizona: comparison between tele-echocardiography and videotapes. Telemedicine journal and e-health : the official journal of the American Telemedicine Association, 18(10), 736-42.
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  We report our experience with tele-echocardiography and echocardiograms recorded digitally or on videotape (recorded-echos) at The University of Arizona from August 2006 to December 2010 and compare their quality and diagnostic accuracy.
• Parikh, K., Witte, M. H., Samson, R., Teodori, M., Carpenter, J. B., Lowe, M. C., Morgan, W., Hardin, C., Brown, M., Naughton, Y., Sinha, S., & Barber, B. J. (2012). Successful treatment of plastic bronchitis with low fat diet and subsequent thoracic duct ligation in child with fontan physiology. Lymphology, 45(2), 47-52.
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  Plastic bronchitis is a rare condition characterized by the formation and expectoration of long, branching bronchial casts that develop in the tracheobronchial tree and cause airway obstruction. Plastic bronchitis has become increasingly recognized as a feared complication of the Fontan operation with a mortality of up to 50%. We report an 11 year old boy who developed severe plastic bronchitis following Fontan repair and the successful long-term control of cast formation utilizing a low-fat diet and subsequent thoracic duct ligation.
• Parikh, K., Witte, M. H., Samson, R., Teodori, M., Carpenter, J. B., Lowe, M. C., Morgan, W., Hardin, C., Brown, M., Naughton, Y., Sinha, S., & Barber, B. J. (2012). Successful treatment of plastic bronchitis with low fat diet and subsequent thoracic duct ligation in child with fontan physiology. Lymphology, 45(Issue 2).
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  Plastic bronchitis is a rare condition characterized by the formation and expectoration of long, branching bronchial casts that develop in the tracheobronchial tree and cause airway obstruction. Plastic bronchitis has become increasingly recognized as a feared complication of the Fontan operation with a mortality of up to 50%. We report an 11 year old boy who developed severe plastic bronchitis following Fontan repair and the successful long-term control of cast formation utilizing a low-fat diet and subsequent thoracic duct ligation.
• Ihnat, C. L., Zimmerman, H., Copeland, J. G., Meaney, F. J., Sobonya, R. E., Larsen, B. T., Blair, B., Lax, D., & Barber, B. J. (2011). Left ventricular assist device support as a bridge to recovery in young children. Congenital Heart Disease, 6(Issue 3). doi:10.1111/j.1747-0803.2011.00494.x
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  Objective. Left ventricular assist device (LVAD) experience and follow-up data in children are limited. We report the deployment and successful weaning from LVAD in young children with severe heart failure (HF). Design. From 2004-2009, 13 children suffering from HF were placed on LVAD. All presented with a dilated left ventricle (LV) with severely reduced contractility, secondary to myocarditis, atrial arrhythmia or idiopathic HF. This study reports their outcomes and longitudinal follow-up. Results. Of 13 young children with HF (ages 1 month-6 years; mean 19.2 months) placed on LVAD: eight weaned to recovery and successful hospital discharge, one was transplanted and four died. Echo follow-up in the weaned patients (mean age 22.1 months) revealed significant improvements from pre-LVAD measurements: LV end-diastolic dimension (LVED) mean z-score decreased from +4.8 to +0.95 (P
• Abdy, N. A., Valdes, S. O., Sorrell, V. L., Klewer, S. E., & Barber, B. J. (2010). Apical Hypertrophic Cardiomyopathy in an Adolescent. Congenital Heart Disease, 5(Issue 2). doi:10.1111/j.1747-0803.2009.00346.x
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  To our knowledge, this is one of the few reported cases of apical hypertrophic cardiomyopathy in an adolescent patient in the United States. We describe a clinical presentation of an adolescent male who presented for cardiac evaluation and was found to have an apical variant of hypertrophic cardiomyopathy. © 2010 Copyright the Authors. Journal Compilation © 2010 Wiley Periodicals, Inc.
• Zimmerman, H., Covington, D., Smith, R., Inaht, C., Barber, B., & Copeland, J. (2010). Erratum: Recovery of dilated cardiomy-opathies in infants and children using left ventricular assist devices (ASAIO Journal (2010) 56 (364-368)). ASAIO Journal, 56(Issue 5). doi:10.1097/mat.0b013e3181f1928e
• Zimmerman, H., Smith, R. D., Inaht, C., Ihnat, C., Covington, D., Copeland, J. G., & Barber, B. J. (2010). Recovery of dilated cardiomyopathies in infants and children using left ventricular assist devices.. ASAIO journal (American Society for Artificial Internal Organs : 1992), 56(4), 364-8. doi:10.1097/mat.0b013e3181e1d228
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  Most infants and children implanted with left ventricular assist devices (LVADs) are bridged to cardiac transplantation. Prioritizing recovery may decrease this trend. Patients were treated with LVAD ventricular decompression, medical heart failure therapy, and bolus therapy with a beta-agonist before weaning trials. Devices were removed if adequate function was observed. Eleven patients with a mean age of 1.7 years presented for LVAD implantation. The mean Z score for left ventricular end diastolic diameter (LVEDD) was +5.5 (+1.6 to +7.3), and the mean fractional shortening was 9% (5%-14%). They were on maximal medical therapy and inotropic support. Duration of device support ranged from 6 to 22 days (mean: 13 days). There were three deaths, one from preimplant anoxic brain damage and two from thromboembolism. Eight patients (73%) recovered, were explanted, and are alive 0.6-6 years with hearts that have a mean Z score for LVEDD of 1.0 (0.09-3.7) and fractional shortening of 23%-36%. Left ventricular assist device decompression of dilated left ventricles in infants and children led to long-term survival in 73%. Ventricular size was significantly reduced and contractility significantly increased. None of these patients required transplantation.
• Hainstock, M. R., Gruchala, N. E., Fike, N., Samson, R. A., Klewer, S. E., & Barber, B. J. (2008). Postural orthostatic tachycardia in a teenager with klinefelter syndrome. Congenital Heart Disease, 3(Issue 6). doi:10.1111/j.1747-0803.2008.00218.x
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  To our knowledge this is the first reported case of postural orthostatic tachycardia syndrome (POTS) in a patient with Klinefelter syndrome. We describe a classic clinical presentation of POTS in an adolescent male with Klinefelter syndrome. Although the etiology of POTS appears to be multifactorial, there is a strong female predominance that suggests a genetic basis. Our patient with Klinefelter syndrome may further support a link with POTS to the X chromosome. © 2008 Copyright the Authors Journal compilation © 2008 Blackwell Publishing.
• Redford, D. T., Quigley, T. W., Gandy, K. L., & Barber, B. J. (2008). Transesophageal echocardiography for the intraoperative evaluation and management of a left ventricular vegetation. Anesthesia and Analgesia, 106(Issue 6). doi:10.1213/ane.0b013e31817320d2
• Redford, D. T., Quigley, T. W., Gandy, K. L., & Barber, B. J. (2008). Transesophageal echocardiography for the intraoperative evaluation and management of a left ventricular vegetation. Anesthesia and analgesia, 106(6), 1636-8.
• Bagatell, R., Hainstock, M., Lowe, M. C., Barber, B. J., & Samson, R. A. (2007). The perfect storm: Torsades de Pointes in a child with leukemia. Pediatric blood & cancer, 49(7), 996-9.
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  Torsades de Pointes (TdP) is a life-threatening ventricular arrhythmia that can be associated with metabolic abnormalities, exposure to arrhythmogenic medications, and congenital long-QT syndrome. This report describes a patient with ALL and multiple complications of therapy who developed TdP. The patient had no evidence of congenital long-QT syndrome, but a constellation of factors appears to have led to QT prolongation, ventricular ectopy, and TdP. Although the patient suffered cardiac arrest, rapid recognition of TdP and prompt defibrillation resulted in an excellent outcome.
• Barber, B. J., Donnerstein, R. L., Secomb, T. W., Pogreba-Brown, K., Steelman, R., Ellenby, M. S., Shen, I., & Ungerleider, R. M. (2007). The dicrotic pulse: A common, non-ominous finding after the ross operation. Pediatric Cardiology, 28(Issue 4). doi:10.1007/s00246-006-0005-4
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  We noted a dicrotic pulse in several patients following a Ross operation. Although the etiology of this unique arterial waveform is not completely understood, it has been reported as a sign of low cardiac output and a poor prognosis. We reviewed preoperative echocardiograms and postoperative radial arterial pressure tracings in 33 patients who underwent a Ross procedure between 2000 and 2004. We found a dicrotic pulse to occur commonly (20/33; 61%) following a Ross operation. Moderate to severe preoperative aortic insufficiency was present in 19/20 patients (95%) in whom a dicrotic pulse was noted and in only 3/13 (23%) who did not exhibit a postoperative dicrotic pulse (p < 0.001). A dicrotic pulse was not associated with an increased use of vasoactive infusions or longer hospitalization following the Ross operation. The dicrotic pulse should be recognized as a common postoperative finding in Ross patients that does not herald a delayed postoperative convalescence. The mechanism for a dicrotic pulse in these patients is speculative but may result from changes in vascular compliance secondary to chronic aortic insufficiency. © 2007 Springer Science+Business Media, LLC.
• Copeland, J. G., Smith, R. G., Samson, R., Nolan, P. E., Jg, C., Copeland, J. G., & Barber, B. J. (2007). 395: Bridge to recovery with pulsatile pump support in children under two years of age. Journal of Heart and Lung Transplantation, 26(2), S201-S202. doi:10.1016/j.healun.2006.11.416
• Soper, M. S., Sahn, D. J., Lin, G. C., Li, X. K., Kwee, Y. H., Gundle, K. R., Coffman, J. E., & Barber, B. J. (2007). Abstract 2572: Relationship Between Circumferential and Radial Strain Rate in the Endocardium and the Epicardium: Validation Study Using an In-Vitro Pumped Pig Heart Model. Circulation, 116.
• Cripe, L. H., Barber, B. J., Spicer, R. L., Wong, B. L., Weidner, N., Benson, D. W., & Markham, L. W. (2006). Outpatient continuous inotrope infusion as an adjunct to heart failure therapy in Duchenne muscular dystrophy. Neuromuscular Disorders, 16(Issue 11). doi:10.1016/j.nmd.2006.07.021
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  We report the use of continuous intravenous inotrope infusion as a palliative management strategy for the treatment of symptomatic, refractory, end stage cardiac dysfunction in patients with Duchenne muscular dystrophy. Milrinone and/or dobutamine administered by continuous intravenous infusion provided symptomatic and objective cardiovascular improvement up to 30 months in 3 individuals with Duchenne muscular dystrophy and severe dilated cardiomyopathy. Continuous inotrope infusion should be considered a practical treatment strategy for end stage cardiac dysfunction in Duchenne muscular dystrophy patients when cardiac transplantation is not a viable option. © 2006 Elsevier B.V. All rights reserved.
• Cripe, L. H., Barber, B. J., Spicer, R. L., Wong, B. L., Weidner, N., Benson, D. W., & Markham, L. W. (2006). Outpatient continuous inotrope infusion as an adjunct to heart failure therapy in Duchenne muscular dystrophy. Neuromuscular disorders : NMD, 16(11), 745-8.
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  We report the use of continuous intravenous inotrope infusion as a palliative management strategy for the treatment of symptomatic, refractory, end stage cardiac dysfunction in patients with Duchenne muscular dystrophy. Milrinone and/or dobutamine administered by continuous intravenous infusion provided symptomatic and objective cardiovascular improvement up to 30 months in 3 individuals with Duchenne muscular dystrophy and severe dilated cardiomyopathy. Continuous inotrope infusion should be considered a practical treatment strategy for end stage cardiac dysfunction in Duchenne muscular dystrophy patients when cardiac transplantation is not a viable option.
• Redford, D. T., Kim, A. S., Barber, B. J., & Copeland, J. G. (2006). Transesophageal echocardiography for the intraoperative evaluation of a large anterior mediastinal mass. Anesthesia and Analgesia, 103(Issue 3). doi:10.1213/01.ane.0000226095.59926.94
• Redford, D. T., Kim, A. S., Barber, B. J., & Copeland, J. G. (2006). Transesophageal echocardiography for the intraoperative evaluation of a large anterior mediastinal mass. Anesthesia and analgesia, 103(3), 578-9.
• Barber, B. J., Bagatell, R., Hainstock, M., Lowe, M. C., & Samson, R. A. (2005). The perfect storm: Torsades de Pointes in a child with leukemia. Pediatric Blood & Cancer, 49(7), 996-999. doi:10.1002/pbc.20712
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  Torsades de Pointes (TdP) is a life-threatening ventricular arrhythmia that can be associated with metabolic abnormalities, exposure to arrhythmogenic medications, and congenital long-QT syndrome. This report describes a patient with ALL and multiple complications of therapy who developed TdP. The patient had no evidence of congenital long-QT syndrome, but a constellation of factors appears to have led to QT prolongation, ventricular ectopy, and TdP. Although the patient suffered cardiac arrest, rapid recognition of TdP and prompt defibrillation resulted in an excellent outcome.
• Barber, B. J., Batra, A. S., Burch, G. H., Shen, I., Ungerleider, R. M., Brown, J. W., Turrentine, M. W., Mori, M., Hsieh, Y. C., & Balaji, S. (2005). Acute hemodynamic effects of pacing in patients with Fontan physiology: a prospective study. Journal of the American College of Cardiology, 46(10), 1937-42.
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  The purpose of this research was to assess the hemodynamic response to atrial, ventricular, and dual-chamber pacing in patients with Fontan physiology.
• Barber, B. J., Batra, A. S., Burch, G. H., Shen, I., Ungerleider, R. M., Brown, J. W., Turrentine, M. W., Mori, M., Hsieh, Y. C., & Balaji, S. (2005). Acute hemodynamic effects of pacing in patients with fontan physiology: A prospective study. Journal of the American College of Cardiology, 46(Issue 10). doi:10.1016/j.jacc.2005.07.045
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  OBJECTIVES: The purpose of this research was to assess the hemodynamic response to atrial, ventricular, and dual-chamber pacing in patients with Fontan physiology. BACKGROUND: Bradycardia, due to sinus node dysfunction or atrioventricular (AV) block, with need for pacing, is common after the Fontan operation. The optimal pacing mode for Fontan patients is unknown, but is critical, as hemodynamic aberrancies may cause severe clinical deterioration. We hypothesized that AV synchrony is vital for maximizing Fontan hemodynamics. METHODS: A cross-over trial was conducted with 21 patients (age 2 to 18 years, median 4 years; male patients = 13) in the intensive care unit after a Fontan operation. Hemodynamic parameters, including mean left atrial pressure (LAP, in mm Hg), mean pulmonary artery pressure (PAP, in mm Hg), mean arterial blood pressure (MAP, in mm Hg), and indexed cardiac output via Fick (Qs, in l/min/m2) were measured with atrial, ventricular, and dual-chamber pacing. Measurements were made after pacing for 10 min in each mode, and a 10-min rest was given between each pacing maneuver. RESULTS: Asynchronous ventricular (VOO) pacing resulted in significantly worse hemodynamics when compared to dual-chamber (DOO) and atrial (AOO) pacing with a higher LAP (9.4 VOO; 6.8 DOO; 5.4 AOO) and PAP (15.2 VOO; 13.5 DOO; 12.7 AOO) and lower Qs (3.0 VOO; 3.5 DOO; 3.9 AOO) and MAP (60.1 VOO; 66.5 DOO; 67.2 AOO). CONCLUSIONS: Asynchronous ventricular pacing, after the Fontan procedure, has acute, adverse hemodynamic consequences (elevated LAP and PAP and decreased Qs and MAP). © 2005 by the American College of Cardiology Foundation.
• Barber, B. J., Burch, G. H., Tripple, D., & Balaji, S. (2004). Resolution of Plastic Bronchitis with Atrial Pacing in a Patient with Fontan Physiology. Pediatric Cardiology, 25(Issue 1). doi:10.1007/s00246-003-0529-9
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  We describe a 5-year-old boy with Fontan physiology and a ventricular pacemaker who developed severe plastic bronchitis. Evaluation by cardiac catheterization revealed profoundly altered hemodynamics, which improved with atrial pacing. Following implantation of an atrial pacemaker, which restored atrioventricular (A-V) synchrony, the patient's hemodynamics greatly improved and his plastic bronchitis resolved.
• Sahn, D. J., Li, X., Jones, M. E., Hashimoto, I., & Barber, B. J. (2004). 1112-150 Regional spectrum of tissue Doppler-derived myocardial acceleration during isovolumic relaxation and its relationship to peak filling. Journal of the American College of Cardiology, 43(5), A342. doi:10.1016/s0735-1097(04)91449-0
• Soper, M. S., Sahn, D. J., Li, X., Hwee, Y. K., Hashimoto, I., Giraud, G. D., Coffman, J. E., & Barber, B. J. (2004). 1037-147 Validation study of strain rate in comparison to tissue velocity for determining endocardial, epicardial and global left ventricular function: An in vitro study in isolated porcine left ventricles. Journal of the American College of Cardiology, 43(5), A318. doi:10.1016/s0735-1097(04)91345-9
• Soper, M. S., Sahn, D. J., Lin, G. C., Li, X., Hwee, Y. K., Coffman, J. E., & Barber, B. J. (2004). 1054-149 Applicability of radial versus tangential strain rate measurements for separating normal and abnormal muscle segments of the left ventricle: In vitro study using an extracted pig heart. Journal of the American College of Cardiology, 43(5), A321. doi:10.1016/s0735-1097(04)91357-5
• Zimmerman, A. T., Steelman, R., Shoun, P., Pate, M. F., Olson, M., & Barber, B. J. (2004). Total airway obstruction by a mucous plug relieved with extreme positive pressure: case report of a pediatric patient.. Journal of emergency nursing, 30(5), 473-4. doi:10.1016/j.jen.2004.04.005
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  Patricia Shoun is Staff RN, Pediatric ICU, Robert Steelman is Assistant Professor, Pediatric Critical Care, Brent Barber is Pediatric Cardiology Fellow, Matthew Olson is Pediatric Respiratory Therapist, and Mary Frances D. Pate is Pediatric ICU Clinical Nurse Specialist, Doernbecher Children’s Hospital, Oregon Health & Science University, Portland, Ore. For reprints, write: Mary Frances D. Pate, DSN, RN, Doernbecher Children’s Hospital, 3181 SW Sam Jackson Park Rd, Portland, OR 97239; E-mail: patem@ohsu.edu. J Emerg Nurs 2004;30:473-4. Available online 28 May 2004. 0099-1767/$30.00 Copyright n 2004 by the Emergency Nurses Association. doi:10.1016/j.jen.2004.04.005
• Broberg, C. S., Pantely, G. A., Barber, B. J., Mack, G. K., Lee, K., Thigpen, T., Davis, L. E., Sahn, D., & Hohimer, A. R. (2003). Validation of the Myocardial Performance Index by Echocardiography in Mice: A Noninvasive Measure of Left Ventricular Function. Journal of the American Society of Echocardiography, 16(Issue 8). doi:10.1067/s0894-7317(03)00399-7
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  Background: The myocardial performance index (MPI) is a Doppler-based measure of left ventricular (LV) function. It is noninvasive, independent of LV shape, and does not require dimensional measurements. However, it has never been validated in mice. Methods: A total of 29 anesthetized mice with IV pressure catheters underwent echocardiography (2-dimensional, M-mode, and Doppler) at baseline and during manipulations of β-adrenergic tone, temperature, preload, and afterload. The maximum derivative of LV pressure with respect to time (dP/dtmax) was compared with MPI, fractional shortening (FS), mean velocity of circumferential fiber shortening, and the FS/MPI ratio. Results: MPI (baseline 0.44 ± 0.07) correlated strongly with dP/dtmax (R = -.779, P × .001), as did FS and mean velocity of circumferential fiber shortening. MPI differed significantly with contractility, preload, and afterload manipulation. FS/MPI showed the best correlation with dP/dtmax. Conclusions: MPI strongly correlates with dP/dtmax over a range of hemodynamic conditions in mice. It can be used as a noninvasive index of LV function in this species.
• Broberg, C. S., Pantely, G. A., Barber, B. J., Mack, G. K., Lee, K., Thigpen, T., Davis, L. E., Sahn, D., & Hohimer, A. R. (2003). Validation of the myocardial performance index by echocardiography in mice: a noninvasive measure of left ventricular function. Journal of the American Society of Echocardiography : official publication of the American Society of Echocardiography, 16(8), 814-23.
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  The myocardial performance index (MPI) is a Doppler-based measure of left ventricular (LV) function. It is noninvasive, independent of LV shape, and does not require dimensional measurements. However, it has never been validated in mice.
• Li, X., Jones, M., Wang, H. F., Davies, C. H., Swanson, J. C., Hashimoto, I., Rusk, R. A., Schindera, S. T., Barber, B. J., & Sahn, D. J. (2003). Strain Rate Acceleration Yields a Better Index for Evaluating Left Ventricular Contractile Function as Compared with Tissue Velocity Acceleration during Isovolumic Contraction Time: An in Vivo Study. Journal of the American Society of Echocardiography, 16(Issue 12). doi:10.1067/j.echo.2003.07.006
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  Objective: Our study aimed to investigate whether strain rate acceleration (SRA) during isovolumic contraction time (IVCT) could serve as a sensitive indicator of myocardial function. Methods: A total of 8 sheep underwent occlusion of left anterior descending coronary artery or diagonal branches and 2 sheep underwent left circumflex coronary artery occlusion to create septal, apical, or basal segment myocardial ischemia 19 to 27 weeks before the study. Baseline, volume-loading, dobutamine, and metoprolol infusion were used to produce 4 hemodynamic stages for each sheep. Doppler tissue imaging was acquired using a 5-MHz probe (GE/VingMed Vivid Five, GE Medical Systems, Milwaukee, Wis) on openchest animals using the liver as a standoff at the apex. Using software (EchoPac, GE Medical Systems), SRA during IVCT was calculated and compared with tissue velocity acceleration (TVA) during IVCT from areas located in the normal and ischemic zones. Also, invasively monitored left ventricle dP/dt was measured as reference contractile function. Results: Both TVA and SRA during IVCT showed higher values for normal tissue than for ischemic area (P < .0001). SRA for normal wall segments changed significantly during the 4 stages (P = .01) with corresponding changes on high-fidelity left ventricular pressure catheters (r = 0.92). TVA over normal segments showed no significant change (P = .29) in the 4 hemodynamic stages. Both TVA and SRA of the ischemic segments showed no significant change with pharmacologic maneuvers or loading conditions. Conclusions: SRA and TVA during IVCT are both useful indicators for detecting abnormal heart wall motion. However, SRA tends to be more sensitive than TVA for differentiating the response to stress conditions.
• Li, X., Jones, M., Wang, H. F., Davies, C. H., Swanson, J. C., Hashimoto, I., Rusk, R. A., Schindera, S. T., Barber, B. J., & Sahn, D. J. (2003). Strain rate acceleration yields a better index for evaluating left ventricular contractile function as compared with tissue velocity acceleration during isovolumic contraction time: an in vivo study. Journal of the American Society of Echocardiography : official publication of the American Society of Echocardiography, 16(12), 1211-6.
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  Our study aimed to investigate whether strain rate acceleration (SRA) during isovolumic contraction time (IVCT) could serve as a sensitive indicator of myocardial function.
• Sahn, D. J., & Barber, B. J. (2003). Book Review Paediatric Cardiology Second edition. Edited by Robert H. Anderson, Edward J. Baker, Fergus J. Macartney, Michael L. Rigby, Elliot A. Shinebourne, and Michael Tynan. 2071 pp., in two volumes, illustrated. London, Churchill Livingstone, 2002. $399. 0-443-07990-0. The New England Journal of Medicine, 348(16), 1613-1614. doi:10.1056/nejm200304173481625
• Sahn, D. J., Li, X., Hashimoto, I., Coffman, J. E., & Barber, B. J. (2003). Myocardial strain rate is superior to tissue Doppler imaging for evaluation of left ventricular subendocardial function. Journal of the American College of Cardiology, 41(6), 493. doi:10.1016/s0735-1097(03)82690-6
• Sahn, D. J., Li, X., Jones, M. E., Hejmadi, A., Hashimoto, I., & Barber, B. J. (2003). Quantitative assessment of regional peak myocardial acceleration during isovolumic contraction and relaxation time by tissue doppler imaging. Journal of the American College of Cardiology, 41(6), 455. doi:10.1016/s0735-1097(03)82523-8
• Sakaguchi, R., Sahn, D. J., Li, X., Hejmadi, A., Hashimoto, I., Burch, G. H., & Barber, B. J. (2003). Pulse pressure propagation changes in the aorta with a stent: An in vitro study simulating an adequate result of coarctation treatment with a stent in place. Journal of the American College of Cardiology, 41(6), 489. doi:10.1016/s0735-1097(03)82670-0
• Erdman, S. H., Barber, B. J., & Barton, L. L. (2002). Aspergillus cholangitis: A late complication after Kasai portoenterostomy. Journal of Pediatric Surgery, 37(Issue 6). doi:10.1053/jpsu.2002.32914
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  Ascending bacterial cholangitis can be a recurring complication in older children and adolescents who have undergone successful surgery for biliary atresia. The authors describe a 19-year-old girl with a history of recurrent late-onset, febrile cholangitis in whom persistent jaundice developed. Aspergillus terreus was isolated from her biliary fluid obtained at percutaneous transhepatic cholangiography. Despite prolonged external biliary drainage and systemic amphotericin B therapy, the fungus was not eliminated from her biliary system. Aspergillus cholangitis can occur in the portoenterostomy patient and may be highly resistant to conventional therapy. Copyright 2002, Elsevier Science (USA). All rights reserved.
• Erdman, S. H., Barber, B. J., & Barton, L. L. (2002). Aspergillus cholangitis: A late complication after Kasai portoenterostomy. Journal of pediatric surgery, 37(6), 923-5.
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  Ascending bacterial cholangitis can be a recurring complication in older children and adolescents who have undergone successful surgery for biliary atresia. The authors describe a 19-year-old girl with a history of recurrent late-onset, febrile cholangitis in whom persistent jaundice developed. Aspergillus terreus was isolated from her biliary fluid obtained at percutaneous transhepatic cholangiography. Despite prolonged external biliary drainage and systemic amphotericin B therapy, the fungus was not eliminated from her biliary system. Aspergillus cholangitis can occur in the portoenterostomy patient and may be highly resistant to conventional therapy.
• Oriokot, F. O., Naluyinda, F., Greivenkamp, J. E., Duncan, B., Canfield, L. M., & Barber, B. J. (2000). The Night Vision Threshold Test (NVTT): a simple instrument for testing dark adaptation in young children.. Journal of tropical pediatrics, 46(1), 30-5. doi:10.1093/tropej/46.1.30
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  It is estimated that 41 per cent of the population aged under 5 in the developing world has an inadequate vitamin A dietary intake resulting in increased morbidity and mortality. Half a million children go blind each year as a result of vitamin A deficiency. Thirteen and a half million have night blindness, the first sign of vitamin A deficiency. Unfortunately, there is no simple, sensitive and inexpensive means to identify the child who has marginal levels of vitamin A and thus institute means to prevent their development of severe deficiency. A low cost, simple, easy-to-use instrument designed to detect a young child's ability to adapt to darkness was tested in children admitted to the Mwanamugimu Nutrition Unit at Makerere Medical School in Kampala, Uganda. Despite the severe degree of malnutrition found in these children, Night Vision Threshold Test results and serum retinol levels were related (r = 0.41, p < 0.05). Further efficacy trials for this instrument are planned at community sites in Nepal.
• Zarlenga, B., Simon, J. L., Schlichter, A. J., Quilindro, A. H., Niremberg, M., Kreutzer, G. O., Kreutzer, E. A., Hoffman, C., Batista, N., & Barber, B. J. (1995). 985-67 Study of Pulmonary Perfusion Using Scintigraphy in Total or Partial By-pass of the Right Ventricle at Rest and with Exertion. Journal of the American College of Cardiology, 25(2), 305A. doi:10.1016/0735-1097(95)92726-l
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  The purpose of our study was to determine the distribution of pulmonary flow at rest and with exertion in total or partial by-pass of the right ventricle (TBP and PBP). During 1990–1994, 98 patients (p) were studied within their first post-operative year utilizing pulmonary scintigraphy with 99 m TcMM. The radioactive agent was injected intravenously by superior(arm) and inferior (leg) routes in these patients with congenital cardiopathies consisting of a univentricular atrioventricular (AV) connection or other complex cardiopathies with pulmonary stenosis or previous pulmonary arterial banding unamendable to biventricular correction. Pulmonary perfusion was considered to be homogeneous with a right/left lung ratio of l60/40, right dominant with g80/20, and slightly right dominant with a 60–70/30–40 ratio. 10 p were further studied utilizing 99 m Tc-MAA scintigraphy during maximal exertion (ergometric stress test). Results TBP (33 p): The pulmonary perfusion was homogeneous (52/48) in 20 p with Atriopulmonary anastomosis (APA), right dominant (88/12) in 7 p [5 p with Total Cavo-pulmonary anastomosis (TCP) and 2 p with Kawashima's operation] as determined equally by superior and inferior injection routes (p l 0.001). Perfusion was slightly right dominant (68/32) aw determined via superior injection routes in 6 p with combined surgery: Bidirectional Cavopulmonary anastomosis (CP2-D) and APA (p l 0.001). PBP (65 p): Pulmonary perfusion, as determined by superior injection routes, was right dominant (86/14) in CP2-D (p l 0.001). It was equally right dominant in 59 p with pulsatile flow (87/13), -anteriograde ventricular flow or subclavian-pulmonary anastomosis-, as in 6 p with non-pulsatile flow (88/12) (p:NS). Exercise The pulmonary perfusion was right dominant (92/8) in 9 p (3 CP2-D, 3 TCP. 3 CP2-D and APA) and remained right dominant with exertion (88/12), In 1 p with APA and homogeneous perfusion (52/48) flow persisted to be homogeneous (54/46) with exertion. Conclusions 1] Pulmonary perfusion is homogeneous in APA as determined equally by superior and inferior injection routes; there is a dominantly right disbalance in TCP, Kawashima's operation, and CP2-D pulsatile or non-pulsatile flow; and slightly right dominant in CP2-D and APA combined surgery. Perfusion appears to dominate the lung on whose respective side the Cavo-pulmonary anastomosis (CPA) was placed. 2] After present techniques of CPA to a branch of the pulmonary artery, a disbalance of pulmonary perfusion was observed and found to persist exertion. 3] With respect to pulmonary perfusion, APA appears to be the best option as it provides a mixing chamber to maintain homogeneous perfusion to both lungs.

Proceedings Publications

• McWhirter, A., Awomolo, A., Barber, B. J., & Coppola, L. (2021, Spring).

  Prenatal diagnosis of porencephaly in the setting of fetal congenital heart disease

  . In Journal of Ultrasound in Medicine, 40 (SUPPL), S123.
• Combs, D., Edgin, J. O., Klewer, S., Barber, B. J., Morgan, W. J., Hsu, C. H., Abraham, I., & Parthasarathy, S. (2020). OSA and Neurocognitive Impairment in Children With Congenital Heart Disease. In American Heart Association Scientific Sessions., 158.
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  Background: Children with congenital heart disease (CHD) have an increased risk of neurocognitive impairment. No prior studies have evaluated the role of OSA, which is associated with neurocognitive impairment in children without CHD. Research Question: Is OSA is associated with neurocognitive impairment in children with CHD? Study Design and Methods: Children aged 6 to 17 years with corrected moderate to complex CHD without syndromes that may affect neurocognition were recruited from the pediatric cardiology clinic. Participants underwent home sleep testing and neurocognitive testing, including a validated Intellectual Quotient (IQ) test as well as validated tests of memory (Paired Associates Learning test), executive function (Intra-Extra Dimensional set shift test), and attention (Simple Reaction Test) from the CANTAB neurocognitive testing battery. Results: Complete results were available for 30 children. Seventeen children (57%) were found to have OSA. Total IQ was markedly lower in children with CHD and comorbid OSA compared with children with CHD without comorbid OSA (mean, 86 ± 12 vs 98 ± 11; P = .01). Children with CHD and OSA did significantly worse on the Paired Associates Learning test, with a median of eight total errors (interquartile range [IQR], 2.25-15) compared with children with CHD without OSA (median total errors, 2, IQR, 1-8; P = .02). Interpretation: Children with CHD and comorbid OSA have impaired neurocognition compared with children with CHD without comorbid OSA. OSA may be a reversible cause of neurocognitive impairment in children with CHD. Further research is needed to evaluate the effects of OSA treatment on neurocognitive impairment in children with CHD.
• Witte, M. H., Mustacich, D., Moedano, L., Behan, S., Bernas, M. J., Klewer, S. E., Barber, B. J., Kuo, P. H., & Kylathu, R. (2019, September). Whole Body Lymphangioscintigraphy and SPECT-CT in Infants and Children with Lymphatic Congestion after Surgical Repair of Complex Congenital Heart Disease. In 27th World Congress of Lymphology Abstract Booklet.
• Parthasarathy, S., Abraham, I. L., Hsu, C., Morgan, W. J., Barber, B. J., Edgin, J. O., & Combs, D. (2018, Fall). Sleep-disordered breathing is associated with neurocognitive impairment in children with congenital heart disease. In Sleep, 41, A277.
• Parthasarathy, S., Abraham, I. L., Hsu, C., Morgan, W. J., Klewer, S. E., Barber, B. J., Edgin, J. O., & Combs, D. (2018, Fall). Obstructive sleep apnea is a novel risk factor for neurocognitive impairment in children with congenital heart disease. In Circulation, 138(Suppl 1), 14956.

Presentations

• Barnes, T., Erickson, R. P., & Witte, M. H. (2023, Fall). Non-Invasive Peripheral and Central Lymphatic Imaging in Neonates and Young Children with Lymphatic Disorders. 29th World Congress of Lymphology. Genoa, Italy: International Society of Lymphology.
• Seckeler, M., Klewer, S. E., Barber, B. J., Hoyer, A., Guerrero, C. E., Meziab, O., Dereszkiewicz, E., Seckeler, M., Klewer, S. E., Barber, B. J., Hoyer, A., Guerrero, C. E., Meziab, O., & Dereszkiewicz, E. (2023, March). Negative Effect of Bundle Branch Block on Exercise Performance in Patients with Fontan Physiology (from the Pediatric Heart Network Fontan Public Data Set) . American College of Cardiology ACC.23/WCC Scientific Sessions. New Orleans, Louisiana.
• Seckeler, M., Witte, M. H., Barber, B. J., Moses, S., & Bernardi, A. (2019, October). Higher incidence of protein-losing enteropathy in patients with single systemic right ventricle. Finalist, Young Investigator Award, 2019 American Academy of Pediatrics National Conference & Exhibition. New Orleans, LA.
• Witte, M. H., Mustacich, D., Moedano, L., Behan, S., Bernas, M. J., Klewer, S. E., Barber, B. J., Kuo, P. H., & Kylathu, R. (2019, September). Whole Body Lymphangioscintigraphy and SPECT-CT in Infants and Children with Lymphatic Congestion after Surgical Repair of Complex Congenital Heart Disease. 27th World Congress of Lymphology. Buenos Aires and Iguazu, Argentina: International Society of Lymphology.
• Parthasarathy, S., Parthasarathy, S., Abraham, I. L., Abraham, I. L., Hsu, C., Hsu, C., Morgan, W. J., Morgan, W. J., Barber, B. J., Barber, B. J., Edgin, J. O., Edgin, J. O., Combs, D., & Combs, D. (2018, June). Sleep-disordered breathing is associated with neurocognitive impairment in children with congenital heart disease. 2018 meeting of the Associated Professional Sleep Societies. Baltimore, MD.
• Combs, D. A., Skrepnek, G., Seckeler, M., Barber, B. J., & Parthasarathy, S. (2016, June). Sleep-disordered breathing is associated with increased mortality among hospitalized infants with congenital heart disease. 2016 meeting of the Associated Professional Sleep Societies. Denver, CO.

Poster Presentations

• Bose, C., Chin, C., Caryl, N., Meziab, O., Barber, B. J., Andrews, J. G., Seckeler, M., Bose, C., Chin, C., Caryl, N., Meziab, O., Barber, B. J., Andrews, J. G., & Seckeler, M. (2024, November).

  Hospital Outcomes of Patients With Type I Diabetes Mellitus and Moderate and Severe Congenital Heart Disease

  . American Heart Association 2024 Scientific Sessions. Chicago.
• Caryl, N., Macaraeg, M., Bose, C., Meziab, O., Guerrero, C., Andrews, J. G., Barber, B. J., & Seckeler, M. (2024, November).

  Incidence of Autoimmune Disease in Patients with Congenital Heart Disease

  . American Heart Association 2024 Scientific Sessions. Chicago.
• Coppola, L., Lesser, K. B., Bah, O., Barber, B. J., & Sisti, G. (2024, April).

  Intermittent reversed end-diastolic flow in the middle cerebral artery of a fetus with congenital heart disease.

  . UltraCon. Austin, Texas: American Institute of Ultrasound in Medicine.
• Hendrickson, A., Caryl, N., Meziab, O., Andrews, J. G., Barber, B. J., & Seckeler, M. (2024, November).

  Association Between G6PD Deficiency and Congenital Heart Disease Incidence and Hospital Outcomes

  . American Heart Association 2024 Scientific Sessions. Chicago.
• Seckeler, M., Hoyer, A., Klewer, S. E., Barber, B. J., & Chatterjee, A. (2024, September).

  3D modelling to plan transcatheter closure of an inferior sinus venosus defect

  . Pediatric and Adult Interventional Cardiac Symposium 2024. San Diego.
• Seckeler, M., Klewer, S. E., Guerrero, C., Meziab, O., Fox, K., Hoyer, A., Barber, B. J., Andrews, J. G., Mathena, S., & Yatsenko, V. (2024, November). Healthcare utilization trends for adolescents and young adults with moderate and severe congenital heart disease. American Heart Association 2024 Scientific Sessions. Chicago.
• Barber, B. J., Scherer, K., Seckeler, M., Meziab, O., Barber, B. J., Scherer, K., Seckeler, M., & Meziab, O. (2023, August). Prevalence of Cardiovascular Implantable Electronic Devices in Children with Type 1 Myotonic Dystrophy. 8th World Congress of Pediatric Cardiology and Cardiac Surgery. Washington, DC.
• Coppola, L., Barber, B. J., Awomolo, A., & McWhirter, A. (2021, Spring).

  Prenatal diagnosis of porencephaly in the setting of fetal congenital heart disease

  . AIUM Annual Integrative Ultrasound Meeting.
• Combs, D. A., Seckeler, M., Seckeler, M., Seckeler, M., Fernandez, V., Klewer, S. E., Klewer, S. E., Klewer, S. E., Barber, B. J., Parthasarathy, S., Parthasarathy, S., Parthasarathy, S., Morgan, W. J., Andrews, J. G., Andrews, J. G., Andrews, J. G., Hsu, C., Hsu, C., Hsu, C., , Hsu, C., et al. (2020, November). Obstructive Sleep Apnea Is Associated with Cardiac Dysfunction In Children With Congenital Heart Disease. 2020 American Heart Association Scientific SessionsAmerican Heart Association.
• Barber, B. J., John, N., Seckeler, M., & Nayak, S. (2018, May). Multimodality imaging and 3-D model of anatomically corrected malposition of the great arteries (S,L,D).. Arizona Chapter of the American College of Cardiology. Scottsdale, AZ: Arizona Chapter of the American College of Cardiology.
• Barber, B. J., Nigro, J. J., Seckeler, M., & Nayak, S. (2018, May). Multimodality Imaging and 3-D Model of Anatomically Corrected Malposition of the Great Arteries (S, L, D). Arizona Chapter of the American College of Cardiology Annual Meeting. Scottsdale, AZ.
• Combs, D., Edgin, J. O., Barber, B. J., Klewer, S. E., Morgan, W. J., Hsu, C., Abraham, I. L., & Parthasarathy, S. (2018, November). Obstructive Sleep Apnea is a Novel Risk Factor for Neurocognitive Impairment in Children with Congenital Heart Disease. 2018 American Heart Association Scientific Sessions. Chicago, IL.
• Coombs, D., Edgin, J. O., Barber, B. J., Klewer, S. E., Morgan, W. J., Hsu, C., & Parthasarathy, S. (2018, Nov). Obstructive sleep apnea is a novel risk factor for neurocognitive impairment in children with congenital heart disease. American Heart Association. Chicago.
• Parthasarathy, S., Abraham, I. L., Hsu, C., Morgan, W. J., Barber, B. J., Edgin, J. O., & Combs, D. (2018, Fall). Sleep-disordered breathing is associated with neurocognitive impairment in children with congenital heart disease. Sleep Research SocietySleep Research Society.
• Parthasarathy, S., Abraham, I. L., Hsu, C., Morgan, W. J., Klewer, S. E., Barber, B. J., Edgin, J. O., & Combs, D. (2018, Fall). Obstructive sleep apnea is a novel risk factor for neurocognitive impairment in children with congenital heart disease. American Heart AssociationAmerican Heart Association.
• Summerford, K., Bernardi, A., Shehab, Z. M., Barber, B. J., & Janet, L. (2018, June). Meningitis-like presentation of Haemophilus parainfluenzae endocarditis in an otherwise healthy adolescent.. Pediatrics in the Red Rocks. Sedona, AZ: Arizona Chapter of the American Academy of Pediatrics.
• Barber, B. J. (2017, Fall). Intravenous immunoglobulin for congenital parvovirus myocarditis.. American Academy of Pediatrics. Chicago, IL: AAP.
• Combs, D., Edgin, J. O., Barber, B. J., Morgan, W. J., Hsu, C., Abraham, I. L., & Parthasarathy, S. (2017, July). Sleep-disordered breathing is associated with memory impairment in children with congenital heart disease. 15th International Symposium on Sleep and Breathing. Madison, WI.
• Sylvester, M., & Barber, B. J. (2017, Jan). Comparison of outcomes and costs for protein losing enteropathy following single ventricle palliation for right and left ventricle morphology.. Oral presentation Western Regional Meeting of Pediatric Research. Carmel, California: Oral Presentation at nal Meeting of Pediatric Research.
• Young, K., & Barber, B. J. (2017, July). Improvement of hyperechogenic myocardium after prenatal treatment with dexamethasone in fetus exposed to maternal anti-SSA/Ro and anti-SSB/La antibodies.. 7th World Congress of Pediatric Cardiology and Cardiac Surgery. Barcelona, Spain. Barcelona, Spain: World Congress.
• Parthasarathy, S., Combs, D. A., Morgan, W. J., Edgin, J. O., Archbold, K., Rice, S. A., Barber, B. J., Barber, B. J., Archbold, K., Rice, S. A., Edgin, J. O., Morgan, W. J., Combs, D. A., & Parthasarathy, S. (2015, May). Prevalence of Sleep-Disordered Breathing in Children with Congenital Heart Disease. 2015 International meeting of the American Thoracic Society. Denver, CO.
• Zahedieh, S., Seckeler, M., Andrews, J., Klewer, S. E., Scherer, K., Daines, C. L., & Barber, B. J. (2015, Fall). Regional and Racial Variation in Hospitalization Costs in Patients with Duchenne Muscular Dystrophy. American Academy of Pediatrics National Conference and Exhibition. Washington, DC: AAP.

Case Studies

• Bah, O., Sisti, G., Barber, B. J., Lesser, K. B., & Coppola, L. (2024.

  Intermittent reversed end-diastolic flow in the middle cerebral artery of a fetus with congenital heart disease.

  (pp S1-S259).
  More info

  Bah O, Sisti G, Barber B, Lesser K, Coppola L.  Intermittent reversed end-diastolic flow in the middle cerebral artery of a fetus with congenital heart disease.  J Ultrasound Med 43 (suppl 1):S1-S259, 2024.