Sujatha Buddhe

All Publications

• Objective Comparison of Clinical and Cardiac Magnetic Resonance Biomarkers in Adolescents Presenting With Acute Chest Pain and Elevated Troponins Pre-COVID and Post-COVID Vaccination. Journal of magnetic resonance imaging : JMRI Gulhane, A., Soriano, B., Stanescu, L., Schauer, J., Ferguson, M., Romberg, E., Bhutta, S., Otto, R., Caris, E., Mallenahalli, S., Portman, M., Litt, H., Buddhe, S. 2023

  View details for DOI 10.1002/jmri.28971

  View details for PubMedID 37622988

• Appropriateness of cardiovascular computed tomography and magnetic resonance imaging in patients with conotruncal defects. Journal of cardiovascular computed tomography Pickard, S. S., Armstrong, A. K., Balasubramanian, S., Buddhe, S., Crum, K., Kong, G., Lang, S. M., Lee, M. V., Lopez, L., Natarajan, S. S., Norris, M. D., Parra, D. A., Parthiban, A., Powell, A. J., Priromprintr, B., Rogers, L. S., Sachdeva, S., Shah, S. S., Smith, C. A., Stern, K. W., Xiang, Y., Young, L. T., Sachdeva, R. 2023

  Abstract

  To promote the rational use of cardiovascular imaging in patients with congenital heart disease, the American College of Cardiology developed Appropriate Use Criteria (AUC), but its clinical application and pre-release benchmarks have not been evaluated. We aimed to evaluate the appropriateness of indications for cardiovascular magnetic resonance (CMR) and cardiovascular computed tomography (CCT) in patients with conotruncal defects and to identify factors associated with maybe or rarely appropriate (M/R) indications.Twelve centers each contributed a median of 147 studies performed prior to AUC publication (01/2020) on patients with conotruncal defects. To incorporate patient characteristics and center-level effects, a hierarchical generalized linear mixed model was used.Of the 1753 studies (80% CMR, and 20% CCT), 16% were rated M/R. Center M/R ranged from 4 to 39%. Infants accounted for 8.4% of studies. In multivariable analyses, patient- and study-level factors associated with M/R rating included: age <1 year (OR 1.90 [1.15-3.13]), truncus arteriosus (vs. tetralogy of Fallot, OR 2.55 [1.5-4.35]), and CCT (vs. CMR, OR 2.67 [1.87-3.83]). None of the provider- or center-level factors reached statistical significance in the multivariable model.Most CMRs and CCTs ordered for the follow-up care of patients with conotruncal defects were rated appropriate. However, there was significant center-level variation in appropriateness ratings. Younger age, CCT, and truncus arteriosus were independently associated with higher odds of M/R rating. These findings could inform future quality improvement initiatives and further exploration of factors resulting in center-level variation.

  View details for DOI 10.1016/j.jcct.2023.01.044

  View details for PubMedID 36868899

• Comparative effectiveness of virtual reality (VR) vs 3D printed models of congenital heart disease in resident and nurse practitioner educational experience 3D PRINTING IN MEDICINE Awori, J., Friedman, S. D., Howard, C., Kronmal, R., Buddhe, S. 2023; 9 (1): 2

  Abstract

  Medical trainees frequently note that cardiac anatomy is difficult to conceive within a two dimensional framework. The specific anatomic defects and the subsequent pathophysiology in flow dynamics may become more apparent when framed in three dimensional models. Given the evidence of improved comprehension using such modeling, this study aimed to contribute further to that understanding by comparing Virtual Reality (VR) and 3D printed models (3DP) in medical education.We sought to systematically compare the perceived subjective effectiveness of Virtual Reality (VR) and 3D printed models (3DP) in the educational experience of residents and nurse practitioners.Trainees and practitioners underwent individual 15-minute teaching sessions in which features of a developmentally typical heart as well as a congenitally diseased heart were demonstrated using both Virtual Reality (VR) and 3D printed models (3DP). Participants then briefly explored each modality before filling out a short survey in which they identified which model (3DP or VR) they felt was more effective in enhancing their understanding of cardiac anatomy and associated pathophysiology. The survey included a binary summative assessment and a series of Likert scale questions addressing usefulness of each model type and degree of comfort with each modality.Twenty-seven pediatric residents and 3 nurse practitioners explored models of a developmentally typical heart and tetralogy of Fallot pathology. Most participants had minimal prior exposure to VR (1.1 ± 0.4) or 3D printed models (2.1 ± 1.5). Participants endorsed a greater degree of understanding with VR models (8.5 ± 1) compared with 3D Printed models (6.3 ± 1.8) or traditional models of instruction (5.5 ± 1.5) p < 0.001. Most participants felt comfortable with modern technology (7.6 ± 2.1). 87% of participants preferred VR over 3DP.Our study shows that, overall, VR was preferred over 3DP models by pediatric residents and nurse practitioners for understanding cardiac anatomy and pathophysiology.

  View details for DOI 10.1186/s41205-022-00164-6

  View details for Web of Science ID 000929996000001

  View details for PubMedID 36773171

  View details for PubMedCentralID PMC9918815

• HEARTBEAT4D: An Open-source Toolbox for Turning 4D Cardiac CT into VR/AR. Journal of digital imaging Bindschadler, M., Buddhe, S., Ferguson, M. R., Jones, T., Friedman, S. D., Otto, R. K. 2022; 35 (6): 1759-1767

  Abstract

  Four-dimensional data sets are increasingly common in MRI and CT. While clinical visualization often focuses on individual temporal phases capturing the tissue(s) of interest, it may be possible to gain additional insight through exploring animated 3D reconstructions of physiological motion made possible by augmented or virtual reality representations of 4D patient imaging. Cardiac CT acquisitions can provide sufficient spatial resolution and temporal data to support advanced visualization, however, there are no open-source tools readily available to facilitate the transformation from raw medical images to dynamic and interactive augmented or virtual reality representations. To address this gap, we developed a workflow using free and open-source tools to process 4D cardiac CT imaging starting from raw DICOM data and ending with dynamic AR representations viewable on a phone, tablet, or computer. In addition to assembling the workflow using existing platforms (3D Slicer and Unity), we also contribute two new features: 1. custom software which can propagate a segmentation created for one cardiac phase to all others and export to surface files in a fully automated fashion, and 2. a user interface and linked code for the animation and interactive review of the surfaces in augmented reality. Validation of the surface-based areas demonstrated excellent correlation with radiologists' image-based areas (R > 0.99). While our tools were developed specifically for 4D cardiac CT, the open framework will allow it to serve as a blueprint for similar applications applied to 4D imaging of other tissues and using other modalities. We anticipate this and related workflows will be useful both clinically and for educational purposes.

  View details for DOI 10.1007/s10278-022-00659-y

  View details for PubMedID 35614275

  View details for PubMedCentralID PMC9712868

• The Diagnostic Role of Echocardiographic Strain Analysis in Patients Presenting with Chest Pain and Elevated Troponin: A Multicenter Study JOURNAL OF THE AMERICAN SOCIETY OF ECHOCARDIOGRAPHY Schauer, J., Caris, E., Soriano, B., Ait-Ali, L., Ashwath, R., Balasubramanian, S., Choueiter, N., Christensen, J., Cornicelli, M., Muniz, J., Parra, D., Tham, E., Albers, E., Chikkabyrappa, S., Young, L., Ferguson, M., Buddhe, S. 2022; 35 (8): 857-867

  Abstract

  Myocarditis presenting as acute chest pain with elevated troponins without significant cardiac compromise is rare in previously healthy children, often referred to as myopericarditis. Diagnosis is challenging, as conventional echocardiographic measures of systolic function can be normal. The aim of this study was to demonstrate the diagnostic utility of strain imaging in this scenario.This was a multicenter, retrospective study including patients presenting with chest pain and elevated troponin from 10 institutions who underwent cardiac magnetic resonance imaging and transthoracic echocardiography within 30 days of each other (group 1). Findings were compared with those among 19 control subjects (group 2). Clinical data and conventional echocardiographic and cardiac magnetic resonance imaging data were collected. Echocardiography-derived strain was measured at the core laboratory. Group 1 was divided into subgroups as myocarditis positive (group 1a) or negative (group 1b) on cardiac magnetic resonance imaging on the basis of established criteria.Group 1 included 108 subjects (88 in group 1a, 20 in group 1b). Although all groups had normal mean fractional shortening and mean left ventricular ejection fraction, group 1 had significantly lower ejection fraction (56.8 ± 7.0%) compared with group 2 (62.3 ± 4.9%; P < .005) and fractional shortening (31.2 ± 4.9%) compared with group 2 (34.1 ± 3.5%; P < .05). Additionally, peak global longitudinal strain (GLS) was markedly abnormal in group 1 (-13.9 ± 3.4%) compared with group 2 (-19.8 ± 2.1%; P < .001). In subgroup analysis, GLS was markedly abnormal in group 1a (-13.2 ± 3.0%) compared with group 1b (-17.3 ± 2.6%; P < .001). Fifty-four subjects underwent follow-up echocardiography (46 in group 1a, eight in group 1b), with mean a follow-up time of 10 ± 11 months. At follow-up, whereas ejection fraction and fractional shortening returned to normal in all patients, abnormalities in strain persisted in group 1, with 22% still having abnormal GLS. Moreover, mean GLS was more abnormal in group 1a (-16.1 ± 2.6%) compared with group 1b (-17.4 ± 1.2%; P < .05).The present study demonstrates that echocardiographic GLS is significantly worse in subjects with myopericarditis presenting with chest pain and elevated troponins compared with control subjects even when conventional measures of systolic function are largely normal and that these abnormalities persisted over time.

  View details for DOI 10.1016/j.echo.2022.03.009

  View details for Web of Science ID 000863486000010

  View details for PubMedID 35301094

• Accuracy of Cardiac Magnetic Resonance Imaging in Diagnosing Pediatric Cardiac Masses A Multicenter Study JACC-CARDIOVASCULAR IMAGING Beroukhim, R. S., Ghelani, S., Ashwath, R., Balasubramanian, S., Biko, D. M., Buddhe, S., Campbell, M., Cross, R., Festa, P., Griffin, L., Grotenhuis, H., Hasbani, K., Hashemi, S., Hegde, S., Hussain, T., Jain, S., Kiaffas, M., Kutty, S., Lam, C. Z., Liberato, G., Merlocco, A., Misra, N., Mowers, K. L., Muniz, J., Nutting, A., Parra, D. A., Patel, J. K., Perez-Atayde, A. R., Prasad, D., Rosental, C. F., Shah, A., Samyn, M. M., Sleeper, L. A., Slesnick, T., Valsangiacomo, E., Geva, T. 2022; 15 (8): 1391-1405

  Abstract

  After diagnosis of a cardiac mass, clinicians must weigh the benefits and risks of ascertaining a tissue diagnosis. Limited data are available on the accuracy of previously developed noninvasive pediatric cardiac magnetic resonance (CMR)-based diagnostic criteria.The goals of this study were to: 1) evaluate the CMR characteristics of pediatric cardiac masses from a large international cohort; 2) test the accuracy of previously developed CMR-based diagnostic criteria; and 3) expand diagnostic criteria using new information.CMR studies (children 0-18 years of age) with confirmatory histological and/or genetic diagnosis were analyzed by 2 reviewers, without knowledge of prior diagnosis. Diagnostic accuracy was graded as: 1) single correct diagnosis; 2) correct diagnosis among a differential; or 3) incorrect diagnosis.Of 213 cases, 174 (82%) had diagnoses that were represented in the previously published diagnostic criteria. In 70% of 174 cases, both reviewers achieved a single correct diagnosis (94% of fibromas, 71% of rhabdomyomas, and 50% of myxomas). When ≤2 differential diagnoses were included, both reviewers reached a correct diagnosis in 86% of cases. Of 29 malignant tumors, both reviewers indicated malignancy as a single diagnosis in 52% of cases. Including ≤2 differential diagnoses, both reviewers indicated malignancy in 83% of cases. Of 6 CMR sequences examined, acquisition of first-pass perfusion and late gadolinium enhancement were independently associated with a higher likelihood of a single correct diagnosis.CMR of cardiac masses in children leads to an accurate diagnosis in most cases. A comprehensive imaging protocol is associated with higher diagnostic accuracy.

  View details for DOI 10.1016/j.jcmg.2021.07.010

  View details for Web of Science ID 000889068700005

  View details for PubMedID 34419404

• Persistent Cardiac Magnetic Resonance Imaging Findings in a Cohort of Adolescents with Post-Coronavirus Disease 2019 mRNA Vaccine Myopericarditis JOURNAL OF PEDIATRICS Schauer, J., Buddhe, S., Gulhane, A., Sagiv, E., Studer, M., Colyer, J., Chikkabyrappa, S., Law, Y., Portman, M. A. 2022; 245: 233-237

  Abstract

  We describe the evolution of cardiac magnetic resonance imaging findings in 16 patients, aged 12-17 years, with myopericarditis after the second dose of the Pfizer mRNA coronavirus disease 2019 vaccine. Although all patients showed rapid clinical improvement, many had persistent cardiac magnetic resonance imaging findings at 3- to 8-month follow-up.

  View details for DOI 10.1016/j.jpeds.2022.03.032

  View details for Web of Science ID 000814834300046

  View details for PubMedID 35351530

  View details for PubMedCentralID PMC8957353

• Worldwide Disparities in Recovery of Cardiac Testing 1 Year Into COVID-19. Journal of the American College of Cardiology Einstein, A. J., Hirschfeld, C., Williams, M. C., Vitola, J. V., Better, N., Villines, T. C., Cerci, R., Shaw, L. J., Choi, A. D., Dorbala, S., Karthikeyan, G., Lu, B., Sinitsyn, V., Ansheles, A. A., Kudo, T., Bucciarelli-Ducci, C., Nørgaard, B. L., Maurovich-Horvat, P., Campisi, R., Milan, E., Louw, L., Allam, A. H., Bhatia, M., Sewanan, L., Malkovskiy, E., Cohen, Y., Randazzo, M., Narula, J., Morozova, O., Pascual, T. N., Pynda, Y., Dondi, M., Paez, D. 2022; 79 (20): 2001-2017

  Abstract

  The extent to which health care systems have adapted to the COVID-19 pandemic to provide necessary cardiac diagnostic services is unknown.The aim of this study was to determine the impact of the pandemic on cardiac testing practices, volumes and types of diagnostic services, and perceived psychological stress to health care providers worldwide.The International Atomic Energy Agency conducted a worldwide survey assessing alterations from baseline in cardiovascular diagnostic care at the pandemic's onset and 1 year later. Multivariable regression was used to determine factors associated with procedure volume recovery.Surveys were submitted from 669 centers in 107 countries. Worldwide reduction in cardiac procedure volumes of 64% from March 2019 to April 2020 recovered by April 2021 in high- and upper middle-income countries (recovery rates of 108% and 99%) but remained depressed in lower middle- and low-income countries (46% and 30% recovery). Although stress testing was used 12% less frequently in 2021 than in 2019, coronary computed tomographic angiography was used 14% more, a trend also seen for other advanced cardiac imaging modalities (positron emission tomography and magnetic resonance; 22%-25% increases). Pandemic-related psychological stress was estimated to have affected nearly 40% of staff, impacting patient care at 78% of sites. In multivariable regression, only lower-income status and physicians' psychological stress were significant in predicting recovery of cardiac testing.Cardiac diagnostic testing has yet to recover to prepandemic levels in lower-income countries. Worldwide, the decrease in standard stress testing is offset by greater use of advanced cardiac imaging modalities. Pandemic-related psychological stress among providers is widespread and associated with poor recovery of cardiac testing.

  View details for DOI 10.1016/j.jacc.2022.03.348

  View details for PubMedID 35589162

  View details for PubMedCentralID PMC9109706

• Survey of centers performing cardiovascular magnetic resonance in pediatric and congenital heart disease: a report of the Society for Cardiovascular Magnetic Resonance JOURNAL OF CARDIOVASCULAR MAGNETIC RESONANCE Buddhe, S., Soriano, B. D., Powell, A. J. 2022; 24 (1): 10

  Abstract

  There are few data on practice patterns and trends for cardiovascular magnetic resonance (CMR) in pediatric and congenital heart disease. The Society for Cardiovascular Magnetic Resonance (SCMR) sought to address this deficiency by performing an international survey of CMR centers.Surveys consisting of 31 (2014) and 33 (2018) items were designed to collect data on the use of CMR for the evaluation of pediatric and congenital heart disease patients. They were sent to all SCMR members in 2014 and 2018. One response per center was collected.There were 93 centers that responded in 2014 and 83 in 2018. The results that follow show data from 2014 and 2018 separated by a dash. The median annual number of pediatric/congenital CMR cases per center was 183-209. The median number of scanners for CMR was 2-2 (range, 1-8) with 58-63% using only 1.5T scanners and 4-4% using only 3T scanners. The mean number of attending/staff reading CMRs was 3.7-2.6; among them, 52-61% were pediatric or adult cardiologists and 47-38% were pediatric or adult radiologists. The median annual case volume per attending was 54-86. The median number of technologists per center doing CMRs was 4-5. The median scanner time allocated for a non-sedated examination was 75-75 min (range, 45-120). Among the 21 centers responding to both surveys, the mean annual case volume increased from 320 in 2014 to 445 in 2018; 17 (81%) of the centers had an increase in annual case volume. For this subgroup, the median attending/staff per center was 4 in both 2014 and 2018. The median scanner time allotted per study was unchanged at 90 min. The mean time for an attending/staff physician to perform a typical CMR examination including reporting was 143-141 min.These survey data provide a novel comprehensive view of CMR practice in pediatric and congenital heart disease. This information is useful for internal benchmarking, resource allocation, addressing practice variation, quality improvement initiatives, and identifying unmet needs.

  View details for DOI 10.1186/s12968-021-00830-4

  View details for Web of Science ID 000750363800001

  View details for PubMedID 35109865

  View details for PubMedCentralID PMC8812017

• Is biventricular vascular coupling a better indicator of ventriculo-ventricular interaction in congenital heart disease? CARDIOLOGY IN THE YOUNG Yang, E. L., Kutty, S., Soriano, B. D., Mallenahalli, S., Ferguson, M. R., Lewin, M. B., Buddhe, S. 2021; 31 (12): 2009-2014

  Abstract

  Ventriculo-ventricular interactions are known to exist, though not well quantified. We hypothesised that the ventricular-vascular coupling ratio assessed by cardiovascular MRI would provide insight into this relationship. We also sought to compare MRI-derived ventricular-vascular coupling ratio to echocardiography and patient outcomes.Children with cardiac disease and biventricular physiology were included. Sanz's and Bullet methods were used to calculate ventricular-vascular coupling ratio by MRI and echocardiography, respectively. Subgroup analysis was performed for right and left heart diseases. Univariate and multivariate regressions were performed to determine associations with outcomes.A total of 55 patients (age 14.3 ± 2.5 years) were included. Biventricular ventricular-vascular coupling ratio by MRI correlated with each other (r = 0.41; p = 0.003), with respect to ventricle's ejection fraction (r = -0.76 to -0.88; p < 0.001) and other ventricle's ejection fraction (r = -0.42 to -0.47; p < 0.01). However, biventricular ejection fraction had only weak correlation with each other (r = 0.31; p = 0.02). Echo underestimated ventricular-vascular coupling ratio for the left ventricle (p < 0.001) with modest correlation to MRI-derived ventricular-vascular coupling ratio (r = 0.43; p = 0.002). There seems to be a weak correlation between uncoupled right ventricular-vascular coupling ratio with the need for intervention and performance on exercise testing (r = 0.33; p = 0.02).MRI-derived biventricular ventricular-vascular coupling ratio provides a better estimate of ventriculo-ventricular interaction in children and adolescents with CHD. These associations are stronger than traditional parameters and applicable to right and left heart conditions.

  View details for DOI 10.1017/S1047951121001426

  View details for Web of Science ID 000840613900020

  View details for PubMedID 33875035

• Echocardiographic assessment of right ventricular volume in repaired tetralogy of Fallot: a novel approach to an older technique JOURNAL OF ECHOCARDIOGRAPHY Lee, J. K., Chikkabyrappa, S., Bhat, A., Buddhe, S. 2022; 20 (2): 106-114

  Abstract

  In repaired tetralogy of Fallot (rTOF), right ventricular (RV) enlargement leads to poor outcomes. However, evaluating the RV has limitations; cardiac magnetic resonance (CMR) and 3D echocardiography have barriers including cost and accessibility. Traditional echocardiography is limited given the complex geometry and anterior location of the RV. We propose a novel echocardiographic evaluation of RV volume using 2 separate views.This is a retrospective study of rTOF patients with echocardiogram, CMR, and exercise tests. By echocardiogram, we collected RV length in parasternal long axis (PLAX), area in 4-chamber (4C) view, and measurements per standard guidelines. RV end-diastolic and end-systolic volume (RVEDV and RVESV) were calculated as 5/9 (4C area * PLAX length).Forty-five patients with 66 sets of CMR, echocardiogram, and exercise tests were included (mean age 13.3 ± 3.2 years). The echocardiographic RVEDV and RVESV showed strong correlation with CMR parameters (r = 0.81 and 0.72; p≤ 0.0001), and moderate correlation with peak oxygen pulse (0.63 and 0.49; p≤0.0001). Guideline measurements had no significant correlation. Echocardiographic RVEDV and RVESV were higher in those requiring subsequent pulmonary valve replacement. Indexed echocardiographic RVEDV of 93 ml/m2 had 92% sensitivity and 50% specificity (area under curve 0.75 (p = 0.001)) in predicting CMR RV/LV EDV ratio > 2, which is an early indicator for pulmonary valve replacement.This novel technique correlates strongly with CMR, better than traditional parameters. While echocardiogram will not replace CMR, this method would be useful in predicting the RV volume, progression of dilation, and timing of CMR.

  View details for DOI 10.1007/s12574-021-00558-1

  View details for Web of Science ID 000723962600001

  View details for PubMedID 34850366

• Myopericarditis After the Pfizer Messenger Ribonucleic Acid Coronavirus Disease Vaccine in Adolescents JOURNAL OF PEDIATRICS Schauer, J., Buddhe, S., Colyer, J., Sagiv, E., Law, Y., Chikkabyrappa, S., Portman, M. A. 2021; 238: 317-320

  Abstract

  Reports have emerged of myocarditis and pericarditis predominantly after the second dose of the coronavirus disease messenger ribonucleic acid vaccine. We describe 13 patients aged 12-17 years who presented with chest pain within 1 week after their second dose of the Pfizer vaccine and were found to have elevated serum troponin levels and evidence of myopericarditis.

  View details for DOI 10.1016/j.jpeds.2021.06.083

  View details for Web of Science ID 000719233600055

  View details for PubMedID 34228985

  View details for PubMedCentralID PMC8253718

• 3D models improve understanding of congenital heart disease 3D PRINTING IN MEDICINE Awori, J., Friedman, S. D., Chan, T., Howard, C., Seslar, S., Soriano, B. D., Buddhe, S. 2021; 7 (1): 26

  Abstract

  Understanding congenital heart disease (CHD) is vital for medical personnel and parents of affected children. While traditional 2D schematics serve as the typical approach used, several studies have shown these models to be limiting in understanding complex structures. Recent world-emphasis has shifted to 3D printed models as a complement to 2D imaging to bridge knowledge and create new opportunities for experiential learning. We sought to systematically compare 3D digital and physical models for medical personnel and parent education compared to traditional methods.3D printed and digital models were made out of MRI and CT data for 20 common CHD. Fellows and nurse practitioners used these models to explore intra-cardiac pathologies following traditional teaching. The models were also used for parent education in outpatient settings after traditional education. The participants were then asked to fill out a Likert scale questionnaire to assess their understanding and satisfaction with different teaching techniques. These ratings were compared using paired t-tests and Pearson's correlation.Twenty-five medical personnel (18 fellows; 2 nurses; 4 nurse practitioners and one attending) and twenty parents participated in the study. The diagnosis varied from simple mitral valve pathology to complex single ventricle palliation. Parent and medical personnel perceived understanding with digital models was significantly higher than traditional (p = 0.01). Subjects also felt that physical models were overall more useful than digital ones (p = 0.001). Physicians using models for parent education also perceived the models to be useful, not significantly impacting their clinical workflow.3D models, both digital and printed, enhance medical personnel and parental perceived understanding of CHD.

  View details for DOI 10.1186/s41205-021-00115-7

  View details for Web of Science ID 000692403300001

  View details for PubMedID 34471999

  View details for PubMedCentralID PMC8411549

• COVID-19 Vaccination-Associated Myocarditis in Adolescents. Pediatrics Jain, S. S., Steele, J. M., Fonseca, B., Huang, S., Shah, S., Maskatia, S. A., Buddhe, S., Misra, N., Ramachandran, P., Gaur, L., Eshtehardi, P., Anwar, S., Kaushik, N., Han, F., Chaudhuri, N. R., Grosse-Wortmann, L. 2021

  View details for DOI 10.1542/peds.2021-053427

  View details for PubMedID 34389692

• Differences in right ventricular-pulmonary vascular coupling and clinical indices between repaired standard tetralogy of Fallot and repaired tetralogy of Fallot with pulmonary atresia. Diagnostic and interventional imaging Buddhe, S., Jani, V., Sarikouch, S., Gaur, L., Schuster, A., Beerbaum, P., Lewin, M., Kutty, S. 2021; 102 (2): 85-91

  Abstract

  The purpose of this study was to compare ventricular vascular coupling ratio (VVCR) between patients with repaired standard tetralogy of Fallot (TOF) and those with repaired TOF-pulmonary atresia (TOF-PA) using cardiovascular magnetic resonance (CMR).Patients with repaired TOF aged>6 years were prospectively enrolled for same day CMR, echocardiography, and exercise stress test following a standardized protocol. Sanz's method was used to calculate VVCR as right ventricle (RV) end-systolic volume/pulmonary artery stroke volume. Regression analysis was used to examine associations with exercise test parameters, New York Heart Association (NYHA) class, RV size and biventricular systolic function.A total of 248 subjects were included; of these 222 had repaired TOF (group I, 129 males; mean age, 15.9±4.7 [SD] years [range: 8-29 years]) and 26 had repaired TOF-PA (group II, 14 males; mean age, 17.0±6.3 [SD] years [range: 8-29 years]). Mean VVCR for all subjects was 1.54±0.64 [SD] (range: 0.43-3.80). Mean VVCR was significantly greater in the TOF-PA group (1.81±0.75 [SD]; range: 0.78-3.20) than in the standard TOF group (1.51±0.72 [SD]; range: 0.43-3.80) (P=0.03). VVCR was greater in the 68 NYHA class II subjects (1.79±0.66 [SD]; range: 0.75-3.26) compared to the 179 NYHA class I subjects (1.46±0.61 [SD]; range: 0.43-3.80) (P<0.001).Non-invasive determination of VVCR using CMR is feasible in children and adolescents. VVCR showed association with NYHA class, and was worse in subjects with repaired TOF-PA compared to those with repaired standard TOF. VVCR shows promise as an indicator of pulmonary artery compliance and cardiovascular performance in this cohort.

  View details for DOI 10.1016/j.diii.2020.05.008

  View details for PubMedID 32513548

• Uncommon Association of Aortopulmonary Window in a Patient with Complex Univentricular Heart and CHARGE Syndrome. Kansas journal of medicine Chikkabyrappa, S., Mahadevaiah, G., Doshi, A. R., Lee, J., Sagiv, E., Buddhe, S. 2021; 14: 48-50

  View details for DOI 10.17161/kjm.vol1414591

  View details for PubMedID 33654544

• Tripartite Assessment of Right Ventricular Systolic Function in Persistent Pulmonary Hypertension of the Newborn PEDIATRIC CARDIOLOGY Chikkabyrappa, S. M., Critser, P., Roane, J., Buddhe, S., Tretter, J. T. 2020; 41 (6): 1206-1211

  Abstract

  Non-invasive evaluation of right ventricular (RV) systolic function in neonates with pulmonary hypertension (PH) with traditional metrics including RV fractional area change (FAC) and tricuspid annular systolic plane excursion (TAPSE) has improved outcomes. Apical three-chamber (3C) RV-FAC, a novel tripartite assessment of the RV, has recently been described in healthy infants. We assess the utility of 3C RV-FAC and biplane RV-FAC in delayed transitioning and neonatal PH. Echocardiograms for 22 normal infants and 22 infants with PH were retrospectively analyzed for RV systolic function indices including four chamber (4C), 3C, and biplane RV-FAC, TAPSE, Tei index, and RV systolic excursion velocity (S'). 4C, 3C, and biplane RV-FAC correlated with PH severity and was decreased in neonates with PH compared to normal neonates (biplane RV-FAC 31.7 ± 13.4% vs. 41 .9 ± 4.7%, p = 0.002). TAPSE was significantly decreased in neonates with PH, but did not correlate with PH severity. Other RV systolic function metrics were not significantly different between normal neonates and neonates with PH. 3C RV-FAC and biplane RV-FAC are lower in neonates with PH. 3C and biplane RV-FAC may allow for improved assessment of global RV systolic dysfunction in newborns with delayed transitioning or PH compared to the commonly used regional methods.

  View details for DOI 10.1007/s00246-020-02376-1

  View details for Web of Science ID 000537358000002

  View details for PubMedID 32488510

• Tetralogy of Fallot With Pulmonary Atresia: Anatomy, Physiology, Imaging, and Perioperative Management SEMINARS IN CARDIOTHORACIC AND VASCULAR ANESTHESIA Ganigara, M., Sagiv, E., Buddhe, S., Bhat, A., Chikkabyrappa, S. M. 2021; 25 (3): 208-217

  Abstract

  Tetralogy of Fallot (ToF) with pulmonary atresia (ToF-PA) is a complex congenital heart defect at the extreme end of the spectrum of ToF, with no antegrade flow into the pulmonary arteries. Patients differ with regard to the sources of pulmonary blood flow. In the milder spectrum of disease, there are confluent branch pulmonary arteries fed by ductus arteriosus. In more severe cases, however, the ductus arteriosus is absent, and the sole source of pulmonary blood flow is via major aortopulmonary collateral arteries (MAPCAs). The variability in the origin, size, number, and clinical course of these MAPCAs adds to the complexity of these patients. Currently, the goal of management is to establish pulmonary blood flow from the right ventricle (RV) with RV pressures that are ideally less than half of the systemic pressure to allow for closure of the ventricular septal defect. In the long term, patients with ToF-PA are at higher risk for reinterventions to address pulmonary arterial or RV-pulmonary artery conduit stenosis, progressive aortic root dilation and aortic insufficiency, and late mortality than those with less severe forms of ToF.

  View details for DOI 10.1177/1089253220920480

  View details for Web of Science ID 000535730600001

  View details for PubMedID 32450763

• Rare Association of Absent Pulmonary Valve Syndrome with Double Outlet Right Ventricle and Hypoplastic Left Heart Complex. Kansas journal of medicine Frandsen, E. L., Doshi, A. R., Buddhe, S., Arya, B., Chikkabyrappa, S. M. 2020; 13: 21-22

  View details for PubMedID 32047585

• The Early "Unnatural" History Following Surgical Repair of Ventricular Septal Defects. Kansas journal of medicine Chikkabyrappa, S. M., Tretter, J. T., Doshi, A. R., Buddhe, S., Bhatla, P., Ludomirsky, A. 2019; 12 (4): 121-124

  Abstract

  INTRODUCTION: Surgical outcomes for simple ventricular septal defects (VSD) have been excellent in the past three decades. For this project, the timing of resolution of left-sided dilation and mitral regurgitation (MR) following VSD repair was assessed.METHODS: Echocardiographic data surrounding surgery of 42 consecutive children who underwent surgical patch repair of a VSD were reviewed. The echocardiograms were reviewed up to a mean of 12 months post-operatively (range 9 - 14 months). Quantitative data indexed to body surface area including left atrial (LA) volume, mitral valve annulus diameter, and left ventricular end-diastolic dimension (LVEDD) was analyzed.RESULTS: The majority of our pre-surgical cohort had only trace (44%) or no MR (31%), with a small proportion having mild (16%) or moderate MR (9%). No patients had moderate or greater MR following repair at follow-up. The median mitral valve annular Z-score was 1.8 (SD 1.6; range: -1.2 to 4.1) pre-operatively, improving to a 0.6 (range: -1.7 to 2.4; p < 0.001) at follow-up. LA dilation was present in 70% of patients, with a median LA volume Z-score of 1.1 (range: -2.6 to 15.5), decreasing to 13% median Z-score -1.2 (range: -3.5 to 2.9; p < 0.001) at follow-up. LV dilation was present in 81% of pre-operative patients with a median LVEDD Z-score of 3.0 (range: -2.0 to 7.9). There was significant improvement in qualitative assessment of LV enlargement (25%) with a median LVEDD Z-score of 0.5 (range: -2.1 to 2.9; p < 0.001) at follow-up. Discharge echocardiogram was performed at a mean of 5.7 days (range: 3 - 12 days) following surgery.CONCLUSIONS: Normalization of LA, mitral valve annulus, and LV size occurred within the first three months in the majority of patients, with significant changes occurring within the first post-operative week following surgical repair for VSD.

  View details for PubMedID 31803353

• Preoperative Physiology, Imaging, and Management of Coarctation of Aorta in Children SEMINARS IN CARDIOTHORACIC AND VASCULAR ANESTHESIA Ganigara, M., Doshi, A., Naimi, I., Mahadevaiah, G., Buddhe, S., Chikkabyrappa, S. M. 2019; 23 (4): 379-386

  Abstract

  Coarctation of the aorta (CoA) is a narrowing of the proximal thoracic aorta typically located at the junction of the aorta with the ductus arteriosus. While it is a simple lesion to understand, considerable variation exists in the anatomy and pathophysiology, leading to varied clinical presentation, management options, and prognosis. On the one hand critical CoA manifests in the neonatal period as a duct-dependent lesion, while less severe forms of obstruction present later in childhood or adulthood as hypertension or incidentally noted precordial murmurs. While transthoracic echocardiography is usually adequate, older children and adults may need more advanced imaging modalities like computed tomography and magnetic resonance imaging prior to intervention. Depending on the type of lesion, management options currently available include surgery and percutaneous balloon angioplasty and stenting. Even after successful interventions, these patients need life-long surveillance for residual aortic obstruction and systemic hypertension with variable long-term clinical outcomes.

  View details for DOI 10.1177/1089253219873004

  View details for Web of Science ID 000488726600001

  View details for PubMedID 31535945

• An unusual case of concordant ventriculoarterial connections, subpulmonary infundibulum, and parallel arterial trunks: a diagnostic challenge CARDIOLOGY IN THE YOUNG Nelson, J. A., Soriano, B. D., Buddhe, S. 2019; 29 (7): 980-982

  Abstract

  We present an unusual case of concordant ventriculoarterial connections, subpulmonary infundibulum, and parallel arterial trunks. This case was complicated by extreme pulmonary artery tortuosity and low arching aorta causing severe tracheal compression. We discuss the difficulty in prenatal diagnosis, necessity for advanced imaging postnatally, and associated airway complications.

  View details for DOI 10.1017/S1047951119000684

  View details for Web of Science ID 000484422700023

  View details for PubMedID 31250773

• Common Arterial Trunk: Physiology, Imaging, and Management SEMINARS IN CARDIOTHORACIC AND VASCULAR ANESTHESIA Chikkabyrappa, S., Mahadevaiah, G., Buddhe, S., Alsaied, T., Tretter, J. 2019; 23 (2): 225-236

  Abstract

  Common arterial trunk (CAT), or truncus arteriosus, is a rare form of cyanotic congenital heart disease and is highly associated with DiGeorge syndrome (microdeletion 22q11.2). Prenatal diagnosis is highly feasible, allowing proper delivery planning and postnatal management. The clinical presentation is highly variable depending on the anatomical variation; however, most commonly presenting with mild cyanosis and significant tachypnea, although these patients can often go undetected in the immediate newborn period. Transthoracic echocardiography is adequate for diagnosis and detailed anatomical delineation in the majority. Additional imaging modalities such as cardiac catheterization, computed tomography angiography, or cardiac magnetic resonance imaging can be helpful in those with more complex pulmonary artery (PA) or aortic anatomy, or in the older repaired. The surgical management of CAT is complete repair in the neonatal period with resection of branch PAs from the CAT with placement of a right ventricular (RV)-to-PA conduit and patch closure of the ventricular septal defect. Overall surgical outcomes are excellent in most centers, with the expectation that the child will eventually outgrow the RV-to-PA conduit and require reoperation. Other potential reoperations or postsurgical interventions in addition to the RV-to-PA conduit may involve the truncal valve or branch PAs.

  View details for DOI 10.1177/1089253218821382

  View details for Web of Science ID 000468994200009

  View details for PubMedID 30596352

• Etanercept With IVIg for Acute Kawasaki Disease: A Randomized Controlled Trial. Pediatrics Portman, M. A., Dahdah, N. S., Slee, A., Olson, A. K., Choueiter, N. F., Soriano, B. D., Buddhe, S., Altman, C. A. 2019

  Abstract

  Patients with Kawasaki disease can develop life-altering coronary arterial abnormalities, particularly in those resistant to intravenous immunoglobulin (IVIg) therapy. We tested the tumor necrosis factor α receptor antagonist etanercept for reducing both IVIg resistance and coronary artery (CA) disease progression.In a double-blind multicenter trial, patients with Kawasaki disease received either etanercept (0.8 mg/kg; n = 100) or placebo (n = 101) subcutaneously starting immediately after IVIg infusion. IVIg resistance was the primary outcome with prespecified subgroup analyses according to age, sex, and race. Secondary outcomes included echocardiographic CA measures within subgroups defined by coronary dilation (z score >2.5) at baseline. We used generalized estimating equations to analyze z score change and a prespecified algorithm for change in absolute diameters.IVIg resistance occurred in 22% (placebo) and 13% (etanercept) of patients (P = .10). Etanercept reduced IVIg resistance in patients >1 year of age (P = .03). In the entire population, 46 (23%) had a coronary z score >2.5 at baseline. Etanercept reduced coronary z score change in those with and without baseline dilation (P = .04 and P = .001); no improvement occurred in the analogous placebo groups. Etanercept (n = 22) reduced dilation progression compared with placebo (n = 24) by algorithm in those with baseline dilation (P = .03). No difference in the safety profile occurred between etanercept and placebo.Etanercept showed no significant benefit in IVIg resistance in the entire population. However, preplanned analyses showed benefit in patients >1 year. Importantly, etanercept appeared to ameliorate CA dilation, particularly in patients with baseline abnormalities.

  View details for DOI 10.1542/peds.2018-3675

  View details for PubMedID 31048415

• Echocardiographic surveillance in children after tetralogy of Fallot repair: Adherence to guidelines? International journal of cardiology Annavajjhala, V. n., Valente, A. M., Lopez, L. n., Sachdeva, R. n., Glickstein, J. S., Natarajan, S. S., Buddhe, S. n., Altmann, K. n., Soriano, B. D., Colquitt, J. L., Altman, C. A., Sasaki, N. n., Sakarovitch, C. n., Tacy, T. A., Geva, T. n., Selamet Tierney, E. S. 2019

  Abstract

  Longitudinal clinical surveillance by transthoracic echocardiography (TTE) is an established practice in children with repaired tetralogy of Fallot (TOF). Non-Invasive Imaging Guidelines recommends a list of reporting elements that should be addressed during routine TTE in this population. In this study, we assessed the adherence to these recommendations.This was a multi-center (n = 8) retrospective review of TTE reports in children ≤11 years of age who have had complete TOF repair. We included 10 patients from each participating center (n = 80) and scored 2 outpatient follow-up TTE reports on each patient. The adherence rate was based on completeness of TTE reporting elements derived from the guidelines.We reviewed 160 TTE reports on 80 patients. Median age was 4.4 months (IQR 1.5-6.6) and 3.6 years (IQR 1.3-6.4) at the time of complete surgical repair and first TTE report, respectively. The median adherence rate to recommended reporting elements was 61% (IQR 53-70). Of the 160 reports, 9 (7%) were ≥80% adherent and 40 (25%) were ≥70% adherent. Quantitative measurements of right ventricular outflow tract (RVOT), right ventricular (RV) size and function, and branch pulmonary arteries were least likely to be reported.Overall adherence to the most recent published imaging guidelines for surveillance of children with repaired TOF patients was suboptimal, especially for reporting of RVOT, RV size and function, and branch pulmonary arteries. Further studies are needed to explore the barriers to adherence to guidelines and most importantly, whether adherence is associated with clinical outcomes.

  View details for DOI 10.1016/j.ijcard.2019.09.075

  View details for PubMedID 31668657

• Cardiac Management of the Patient With Duchenne Muscular Dystrophy PEDIATRICS Buddhe, S., Cripe, L., Friedland-Little, J., Kertesz, N., Eghtesady, P., Finder, J., Hor, K., Judge, D. P., Kinnett, K., McNally, E. M., Raman, S., Thompson, W., Wagner, K. R., Olson, A. K. 2018; 142: S72-S81

  Abstract

  Duchenne muscular dystrophy (DMD) results in a progressive cardiomyopathy that produces significant morbidity and mortality. To improve the quality of life in patients with DMD, cardiac care is focused on surveillance and management, with the goal of slowing the onset and progression of heart failure complications. The current article is intended to be an expanded review on the cardiac management data used to inform the 2018 DMD Care Considerations recommendations as well as be a discussion on clinical controversies and future management directions. The new cardiac guidance includes changes regarding noninvasive imaging surveillance of cardiac function and pharmacologic therapy. Many emerging therapies lack sufficient evidence-based data to be recommended in the 2018 DMD Care Considerations. These are discussed in the present article as clinical controversies and future directions. Important emerging therapies include new heart failure medications, mechanical circulatory support with ventricular assist devices, heart transplantation, and internal cardiac defibrillators. Future research studies should be focused on the risks and benefits of these advanced therapies in patients with DMD. We conclude this review with a brief discussion on the relationship between the heart and the recently developed medications that are used to directly target the absence of dystrophin in DMD.

  View details for DOI 10.1542/peds.2018-0333I

  View details for Web of Science ID 000511642900008

  View details for PubMedID 30275251

  View details for PubMedCentralID PMC6566852

• Determinants of Physician, Sonographer, and Laboratory Productivity: Analysis of the Third Survey from the American Society of Echocardiography Committee on Pediatric Echocardiography Laboratory Productivity JOURNAL OF THE AMERICAN SOCIETY OF ECHOCARDIOGRAPHY Soriano, B. D., Fleishman, C. E., Van Hoever, A. M., Wright, B., Printz, B., Tacy, T. A., Allada, V., Lai, W. W., Buddhe, S., Srivastava, S. 2018; 31 (9): 976-982

  Abstract

  The American Society of Echocardiography Committee on Pediatric Echocardiography Laboratory Productivity was formed in 2011 to study institutional factors that could influence the clinical productivity of physicians and sonographers in academic pediatric echocardiography laboratories. In the previous two surveys, staff clinical productivity remained stable while total echocardiography volumes increased. This third survey was designed to assess how clinical productivity is associated with laboratory infrastructure elements such as training, administrative tasks, quality improvement, research, and use of focused cardiac ultrasound (FCU).Survey questions were sent by e-mail to North American laboratories. The aims were to assess (1) educational and training obligations, (2) academic productivity and research, (3) laboratory medical director satisfaction, (4) quality improvement, (5) laboratory leadership roles, and (6) impact and use of FCU. Survey responses were compared with clinical productivity metrics defined in the first two surveys.There were 38 responses. Academic productivity was higher at institutions with more dedicated imaging personnel, personnel with dedicated protected academic time, and advanced imaging fellows. Academic productivity did not correlate with clinical productivity and was not significantly affected by the presence of dedicated research sonographers. The satisfaction level of laboratory medical directors was related to dedicated administrative time and an administrative stipend. The majority of administrative roles were tasked to the laboratory medical director with support of the technical director. FCU was listed as a hospital privilege at four institutions (13%). Twenty-two (58%) were training FCU providers in one or more subspecialties. FCU was not associated with clinical or academic productivity.This third survey gathered supplemental data to complement the clinical productivity data collected from the first two surveys. Together, the results of these surveys further describe the range of factors that can affect North American academic pediatric echocardiography laboratories.

  View details for DOI 10.1016/j.echo.2018.03.001

  View details for Web of Science ID 000443286600004

  View details for PubMedID 29778293

• Diagnostic role of strain imaging in atypical myocarditis by echocardiography and cardiac MRI PEDIATRIC RADIOLOGY Wisotzkey, B. L., Soriano, B. D., Albers, E. L., Ferguson, M., Buddhe, S. 2018; 48 (6): 835-842

  Abstract

  The diagnosis of myocarditis presenting as isolated acute chest pain with elevated troponins but normal systolic function is challenging with significant drawbacks even for the gold-standard endomyocardial biopsy.This study aimed to evaluate the diagnostic role of strain imaging by echocardiography and cardiac MRI in these patients.This was a retrospective review of children with cardiac MRI for acute chest pain with elevated troponins compared to normal controls. Echocardiographic fractional shortening, ejection fraction, speckle-tracking-derived peak longitudinal, radial, and circumferential strain were compared to cardiac MRI ejection fraction, T2 imaging, late gadolinium enhancement, speckle-tracking-derived peak longitudinal strain, radial strain, and circumferential strain.Group 1 included 10 subjects diagnosed with myocarditis, 9 (90%) males with a median age of 15.5 years (range: 14-17 years) compared with 10 age-matched controls in group 2. All subjects in group 1 had late gadolinium enhancement consistent with myocarditis and troponin ranged from 2.5 to >30 ng/ml. Electrocardiogram changes included ST segment elevation in 6 and abnormal Q waves in 1. Qualitative echocardiographic function was normal in both groups and mean fractional shortening was similar (35±6% in group 1 vs. 34±4% in group 2, P=0.70). Left ventricle ejection fraction by cardiac MRI, however, was lower in group 1 (52±9%) compared to group 2 at (59±4%) (P=0.03). Cardiac MRI derived strain was lower in group 1 vs. group 2 for speckle-tracking-derived peak longitudinal strain (-12.8±2.8% vs. -17.1±1.5%, P=0.001), circumferential strain (-12.3±3.8% vs. -15.8±1.2%, P=0.020) and radial strain (13.6±3.7% vs. 17.2±3.2%, P=0.040). Echocardiography derived strain was also lower in group 1 vs. group 2 for speckle-tracking-derived peak longitudinal strain (-15.6±3.9% vs. -20.8±2.2%, P<0.002), circumferential strain (-16±3% vs. -19.8±1.9%, P<0.003) and radial strain (17.3±6.1% vs. 24.8±6.3%, P=0.010).In previously asymptomatic children, myocarditis can present with symptoms of acute chest pain suspicious for coronary ischemia. Cardiac MRI and echocardiographic strain imaging are noninvasive, radiation-free tests of immense diagnostic utility in these situations. Long-term studies are needed to assess prognostic significance of these findings.

  View details for DOI 10.1007/s00247-017-4061-0

  View details for Web of Science ID 000432293700010

  View details for PubMedID 29651605

• Comparison of two-dimensional and three-dimensional echocardiographic strain in children with CHD CARDIOLOGY IN THE YOUNG Wisotzkey, B. L., Soriano, B. D., Buddhe, S. 2017; 27 (8): 1557-1565

  Abstract

  In CHD, three-dimensional strain analysis may overcome limitations of Doppler and two-dimensional strain of the left ventricle. The aims of this study were to evaluate feasibility and reproducibility of three-dimensional longitudinal, circumferential, and radial systolic strain by three-dimensional speckle-tracking echocardiography compared with two-dimensional echocardiography.Patients with CHD, biventricular circulation with a systemic left ventricle, and who had two- and three-dimensional imaging performed on the same day from 2010 to 2014 were included. Quantitative two- and three-dimensional strain analyses were performed (two-dimensional cardiac performance analysis version 1.2 and four-dimensional left ventricular analysis version 3.1). Intra- and inter-observer variabilities were calculated on 25 studies.A total of 30 patients, including 19 (61%) males, with a median age of 3.6 years (0.1-22 years) were included. The mean fractional shortening was 34.6±5.3%, and the mean ejection fraction was 62.0±6.4%. Measurement of two- and three-dimensional strain was feasible in >95% of segments. Good correlation was observed between longitudinal and circumferential strain (r=0.92, p⩽0.001 and r=0.87, p⩽0.001), but not radial strain (r=0.29, p=0.2). Intra- and inter-observer agreements were better for three-dimensional compared with two-dimensional strain, and better for both two- and three-dimensional longitudinal and circumferential strains compared with radial strain.Left ventricular three-dimensional strain analysis is feasible in children with CHD. The reproducibility of longitudinal and circumferential strain by three-dimensional analyses is better. Further longitudinal studies are warranted for the potential clinical application of this new technology.

  View details for DOI 10.1017/S1047951117000762

  View details for Web of Science ID 000417419400014

  View details for PubMedID 28502268

• Right ventricular global longitudinal strain in repaired tetralogy of Fallot ECHOCARDIOGRAPHY-A JOURNAL OF CARDIOVASCULAR ULTRASOUND AND ALLIED TECHNIQUES Del Toro, K., Soriano, B. D., Buddhe, S. 2016; 33 (10): 1557-1562

  Abstract

  Echocardiogram has limitations in effectively assessing right ventricular (RV) function in children post tetralogy of Fallot (TOF) repair. We evaluated the utility of speckle tracking echocardiography (STE)-based RV global longitudinal strain (GLS) for the assessment of RV systolic function.All patients with repaired TOF who had both echocardiograms and cardiac MRI (CMR) within a 6-month interval were included. RV volumes and ejection fraction (EF) were obtained by CMRs. Traditional echocardiographic function parameters and RV GLS were compared to CMR-derived RV EF. Subjects were divided into two groups based on CMR RV EF (group I: RV EF ≥45%; and group II: RV EF <45%).A total of 57 subjects were included. Mean age was 13.0±3.6 years and 58% were males. Group I had 39 subjects and group II had 18. Only six of the 18 patients (33%) in group II were identified as having at least mild RV dysfunction by echocardiogram. The mean RV GLS was significantly abnormal in group II (-15.3±3.8%) compared to group I (-20.9±3.3%; P<.001). By ROC analysis, an RV GLS cutoff value of -18% had 78% sensitivity and 77% specificity in identifying RV EF <45% (area under curve .87, P<.001). Intra- and inter-observer reproducibility of RV GLS were good.RV GLS is a simple and effective tool for the assessment of RV systolic function in patients post TOF repair. This technique would help further refine patient selection for timing of CMR and management.

  View details for DOI 10.1111/echo.13302

  View details for Web of Science ID 000387156400016

  View details for PubMedID 27543374

• Comparison of left ventricular function assessment between echocardiography and MRI in Duchenne muscular dystrophy PEDIATRIC RADIOLOGY Buddhe, S., Lewin, M., Olson, A., Ferguson, M., Soriano, B. D. 2016; 46 (10): 1399-1408

  Abstract

  Cardiomyopathy in Duchenne muscular dystrophy (DMD) is associated with death in approximately 40% of patients. Echocardiography is routinely used to assess left ventricular (LV) function; however, it has limitations in these patients.We compared echocardiographic measures of cardiac function assessment to cardiac MRI.We included children and young adults with DMD who had MRI performed between January 2010 and July 2015. We measured echocardiographic and MRI parameters of function assessment, including strain. Presence of late gadolinium enhancement (LGE) was assessed by MRI. Subjects were divided into two groups based on MRI left ventricular ejection fraction (LVEF): group I, LVEF ≥55% and group II, LVEF <55%.We included 41 studies in 33 subjects, with 25 in group I and 16 in group II. Mean age of subjects was 13.6 ± 2.8 years and mean duration between echocardiogram and MRI was 7.6 ± 4.1 months. Only 8 of 16 (50%) patients in group II had diminished function on echocardiogram. Echocardiographic images were suboptimal in 16 subjects (39%). Overall, echocardiographic parameters had weak correlation with MRI-derived ejection fraction percentage. MRI-derived myocardial strain assessment has better correlation with MRI ejection fraction as compared to echocardiography-derived strain parameters.Echocardiography-based ventricular functional assessment has weak correlation with MRI parameters in children and young adults with Duchenne muscular dystrophy. While this correlation improves in the subset of subjects with adequate echocardiographic image quality, it remains modest and potentially suboptimal for clinical management. Accordingly, we conclude that MRI should be performed routinely and early in children with DMD, not only for LGE imaging but also for functional assessment.

  View details for DOI 10.1007/s00247-016-3622-y

  View details for Web of Science ID 000382753500005

  View details for PubMedID 27173979

• Right Ventricular Apical Flattening as an Echocardiographic Screening Tool for Right Ventricular Enlargement PEDIATRIC CARDIOLOGY Buddhe, S., Ferguson, M., Arya, B., Soriano, B. D. 2016; 37 (3): 568-574

  Abstract

  Right ventricular dilation is a common complication after tetralogy of Fallot (TOF) repair. Traditional echocardiographic assessments are imprecise due to the RV's location and complex geometry. We propose a novel echocardiographic measurement: RV apical flattening (RVAF) as a screening tool to help identify subjects with severe RV dilation. Patients with repaired TOF who had both echocardiograms and CMR's within 6-month interval at our institution were included in the study. The RVAF was measured in the four-chamber echocardiographic view as the minor length of RV cavity at the level of RV apical endocardium. Subjects were divided into two groups (group I: RVEDVi ≥ 150 ml/m(2) and group II; RVEDVi < 150 ml/m(2)). Echocardiogram and CMR data were compared between groups. A total of 75 subjects were included in the study. Mean age was 12.8 ± 3.6 years. Group I had 36 subjects, and group II had 39 subjects. The mean RVAF was significantly higher in group I (2.7 ± 0.5 cm) compared with group II (1.7 ± 0.4 cm; p < 0.001). There was significant correlation between RVAF and RVEDVi (r = 0.81; p < 0.001). By ROC analysis, an RVAF cutoff value of 2.0 cm had 94 % sensitivity and 77 % specificity in identifying severe RV dilation (area under the curve 0.95). RVAF is a simple and effective echocardiographic screening tool to help identify severe RV dilation. In conjunction with other 2D echocardiographic parameters, this technique would help further refine echocardiography-guided patient selection for timing of CMR and pulmonary valve replacement.

  View details for DOI 10.1007/s00246-015-1316-0

  View details for Web of Science ID 000373308800018

  View details for PubMedID 26667958

• Longitudinal Strain by Speckle Tracking Echocardiography in Pediatric Heart Transplant Recipients CONGENITAL HEART DISEASE Buddhe, S., Richmond, M. E., Gilbreth, J., Lai, W. W. 2015; 10 (4): 362-370

  Abstract

  Heart transplant recipients are at risk for developing left ventricular (LV) dysfunction. While traditional echocardiographic parameters have limitations, speckle tracking echocardiography (STE) is a novel technique shown to be more sensitive and accurate in adult studies for evaluating ventricular systolic and diastolic function.Pediatric heart transplant recipients undergoing routine cardiac catheterization were included. Ratio of mitral early diastolic velocity-to-strain rate during early LV filling (E/SR(E)) and global longitudinal peak systolic strain (GLPSS) was measured by STE imaging. These were compared with wedge pressures by catheterization and traditional echocardiographic parameters.A total of 50 subjects (46% males) were included. Mean age of the subjects was 13.0 ± 6.3 years and time since transplant was 4.1 years (range 0.2-17.1 years). While peak mitral inflow to late diastolic velocity (E/A ratio) was the only traditional diastolic function parameter having significant correlation with pulmonary capillary wedge pressure (PCWP) (r = 0.3; P = .3), STE-derived E/SR(E) had modest correlation with PCWP (r = 0.55; P < .01). Also, while most traditional systolic function parameters were normal, 12 subjects (24%) had GLPSS > -18%. Interestingly, subjects with coronary artery disease (n = 6) had significantly higher E/SR(E) (71.9 ± 28.4) compared to subjects without (45.2 ± 10.8; P < .001).Diastolic function parameters by STE imaging correlate better with gold standard PCWP measurement than traditional echocardiographic parameters. Also, utilizing STE, abnormalities of longitudinal LV systolic function may be more common than previously thought in pediatric heart transplant recipients without acute graft rejection, despite "normal" systolic function by traditional echocardiogram.

  View details for DOI 10.1111/chd.12263

  View details for Web of Science ID 000359861900017

  View details for PubMedID 25919980

• Risk of Congenital Heart Defects in the Offspring of Smoking Mothers: A Population-Based Study JOURNAL OF PEDIATRICS Sullivan, P. M., Dervan, L. A., Reiger, S., Buddhe, S., Schwartz, S. M. 2015; 166 (4): 978-U293

  Abstract

  To conduct a population-based study examining the occurrence of congenital heart defects (CHDs) in relation to maternal smoking during the first trimester of pregnancy.This retrospective case-control study used Washington State birth certificates from 1989 to 2011 and linked hospital discharge International Classification of Diseases, 9th revision, codes to identify singleton nonsyndromic CHD cases and determine maternal prenatal smoking status. We calculated ORs from multivariate logistic regression models to compare maternal first-trimester smoking status (any and daily number of cigarettes) among 14,128 cases, both overall and by phenotype, and 60,938 randomly selected controls frequency matched on birth year.Offspring of mothers reporting cigarette use in the first trimester of pregnancy were more likely to be born with a CHD (aOR 1.16 [1.08-1.24]) independent of demographic characteristics and other prenatal risk factors for CHDs. Maternal smoking was most strongly associated with pulmonary valve anomalies (aOR 1.48 [95% CI: 1.15-1.90]), pulmonary artery anomalies (aOR 1.71 [1.40-2.09]), and isolated atrial septal defects (aOR 1.22 [1.08-1.38]). The association between maternal smoking and CHDs was stronger with increasing number of daily cigarettes and among older (35+ years) mothers compared with younger mothers.We provide evidence that maternal smoking during pregnancy is a risk factor for select CHD phenotypes. Maternal smoking may account for 1.4% of all CHDs. New findings include a strong dose-dependence of the association and augmented risk in older mothers.

  View details for DOI 10.1016/j.jpeds.2014.11.042

  View details for Web of Science ID 000353385700037

  View details for PubMedID 25578997

• Progression of Right Ventricular Dilation in Repaired Tetralogy of Fallot JOURNAL OF MAGNETIC RESONANCE IMAGING Buddhe, S., Shah, A., Lai, W. W. 2015; 41 (3): 730-737

  Abstract

  To evaluate factors associated with rapid rate of progression (ROP) of right ventricular (RV) dilation by cardiac MRI in repaired tetralogy of Fallot (TOF) patients.All patients with repaired TOF with two MRIs were included. RV volumes and function were assessed by MRIs performed on a GE 1.5 Tesla (T) platform. The ROP of RV dilation was calculated as the difference between the last and first RV indexed end-diastolic volumes (iEDV) divided by the time difference. Subjects were divided into two groups: Group I-rapid ROP (top quartile of ROP) and Group II-slower ROP (lower three quartiles).A total of 61 subjects were included. Mean age was 18.0 ± 9.7 years and duration between MRIs 3.4 ± 2.1 years. Median ROP for RV iEDV was 2.0 (-12.7 to 27.8) mL/m(2) /year. Fifteen subjects were in Group I and 46 in Group II. RV iEDV, RV ejection fraction, RV indexed end-systolic volume (iESV) were significantly different between groups. By multivariable analysis, RV iESV was the only independent parameter associated with rapid RV dilation (P < 0.01).There was no significant change in RV iEDV in majority of repaired TOF subjects. RV iESV was the best parameter associated with more rapid RV dilation.

  View details for DOI 10.1002/jmri.24610

  View details for Web of Science ID 000349967700019

  View details for PubMedID 24591270

• Predictors of left ventricular remodeling after aortic valve replacement in pediatric patients with isolated aortic regurgitation. Congenital heart disease Buddhe, S., Du, W., Walters, H. L., Delius, R., Pettersen, M. D. 2013; 8 (2): 167-73

  Abstract

  To identify the risk factors that could predict postoperative outcome after aortic valve replacement in pediatric patients with isolated aortic regurgitation (AR).There is controversy regarding the appropriate timing of surgery in asymptomatic or minimally symptomatic patients with isolated AR. In the pediatric age group, there are limited studies in this regard and most of them are on combined aortic valve stenosis and regurgitation.All patients with biventricular physiology and morphologic left ventricle (LV) who underwent aortic valve surgery for AR from January 1988 to July 2010 were included in the study. Demographic, clinical, and echocardiographic data were collected at presurgical visit, early postoperative, 1 year, and most recent follow-up.Among 53 patients (36 males), 18 had LV end-diastolic diameter (LVEDD) z-score >4 standard deviation (SD) (group I) and 35 had LVEDD <4 SD (group II). Forty-one had long-term follow-up. Mean age at surgery was 11.6 ± 5.9 years; mean follow-up was 6.9 ± 5.6 years. Preoperative LVEDD >4 SD predicted persistent LV dilation (>2 SD) at early post-op (P < .05) and 1 year follow-up (P = .09). Preoperative decreased LV function (fractional shortening <28%) was the only significant predictor of persistent LV dysfunction at most recent follow-up and requirement for repeat interventions (P < .01). Most have reduction of LV dimensions in the immediate postoperative period to normal limits.In children with AR, preoperative LV dysfunction and extreme LV dilation (>4 SD) are significant predictors of incomplete LV remodeling or persistent LV dysfunction.

  View details for DOI 10.1111/j.1747-0803.2012.00703.x

  View details for PubMedID 22931553

• Managing Cardiovascular Risk in Overweight Children and Adolescents PEDIATRIC DRUGS Dhuper, S., Buddhe, S., Patel, S. 2013; 15 (3): 181-190

  Abstract

  The scientific, medical, and lay communities are currently confronted with a serious medical and public health problem related to the marked non-remitting worldwide epidemic of obesity. This ever-increasing prevalence of obesity is accompanied by a host of inherently associated co-morbidities. As a result, obesity is fast becoming the major cause of premature death in the developed world. As pediatric and adult cardiologists, we have seen a dramatic increase in office referrals of overweight and obese children and adolescents, who already have obesity-related degenerative disease processes such as hypertension, dyslipidemia, the metabolic syndrome, and type 2 diabetes mellitus, as well as manifestations of early preclinical atherosclerotic cardiovascular disease, not previously observed in this age group. This article presents a review of the literature and recent scientific statements and recommendations issued by the American Heart Association (AHA) and the American Academy of Pediatrics (AAP) regarding the metabolic abnormalities associated with obesity, including newer identification and treatment strategies for obesity, dyslipidemia, and early subclinical coronary artery disease seen in high-risk children and adolescents.

  View details for DOI 10.1007/s40272-013-0011-y

  View details for Web of Science ID 000320426100003

  View details for PubMedID 23580344

• Primary Endocardial Fibroelastosis Presenting in a Young Child as Incessant Ventricular Tachycardia and Dilated Cardiomyopathy TEXAS HEART INSTITUTE JOURNAL Arya, S. O., Karpawich, P. P., Gupta, P., Buddhe, S., Singh, H. R., Hussein, Y., Gowda, S. T. 2012; 39 (5): 714-718

  Abstract

  Endocardial fibroelastosis is a cardiomyopathy not commonly seen in the present age. We describe the case of a 4-year-old girl who presented with sustained incessant ventricular tachycardia, a severely dilated left ventricle, and cardiac dysfunction refractory to all medical management and even to ablation; she eventually underwent cardiac transplantation. The diagnosis was made only after histopathologic examination of the explanted heart showed clear evidence of endocardial fibroelastosis. Through this report, we would like to highlight the fact that primary endocardial fibroelastosis can masquerade as idiopathic dilated cardiomyopathy and that associated frequent premature ventricular contractions and nonsustained ventricular tachycardia require close monitoring. Progressive ventricular dilation and ventricular dysfunction can convey a poor prognosis. Sustained recalcitrant ventricular tachycardia in these patients can be a life-threatening event that requires emergent mechanical support and heart transplantation.

  View details for Web of Science ID 000309796700029

  View details for PubMedID 23109776

  View details for PubMedCentralID PMC3461649

• Radiofrequency and cryoablation therapies for supraventricular arrhythmias in the young: five-year review of efficacies. Pacing and clinical electrophysiology : PACE Buddhe, S., Singh, H., Du, W., Karpawich, P. P. 2012; 35 (6): 711-7

  Abstract

  Cryoablation (Cryo) has augmented radiofrequency (RF) as the ablation energy choice for most supraventricular tachycardias (SVT). Although initial acute results and more recent, but limited, 3-36-month follow-up studies have been reported, more longer follow-up information is required to determine actual efficacy.Data from patients with structurally normal hearts who underwent reentrant forms of SVT ablation at our institution from January 2005 to December 2009 were reviewed. These included demographics, clinical and electrophysiologic findings, and ablative energies used. Following apparent acute success, all patients were then reevaluated for any potential recurrences of SVT or preexcitation up to 5 years later.A total of 155 patients (83 male) were reviewed (mean age 13.4 ± 3.7 years). Ablations were predominantly right-sided (75%). Atrioventricular reciprocating tachycardia was seen in 74% and atrioventricular node reciprocating tachycardia (AVNRT) in 17% of patients. For concerns of atrioventricular node integrity, Cryo ± RF was user-preferred for anteroseptal accessory fiber locations and AVNRT. Acute success rate was 98% and chronic 83.2% over the next 5 years. Among patients with accessory pathways, recurrence was pathway number and location dependent: significantly higher (P < 0.05) if they were right anterior-anteroseptal, multiple, or with a broad-distribution pattern. There were no significant differences in recurrence rates with use of RF or its combination with Cryo.Radiofrequency ablation and Cryo are both effective therapies for pediatric patients. Although use of Cryo with RF in combination may enhance safety while affording comparable success, risk of recurrence still persists in the current era among patients depending on accessory pathways connection location and characteristics.

  View details for DOI 10.1111/j.1540-8159.2012.03372.x

  View details for PubMedID 22452328

• Impact of pulmonary hypertension on transplant outcomes in pediatric cardiomyopathy patients. Pediatric transplantation Buddhe, S., Du, W., L'Ecuyer, T. 2012; 16 (4): 367-72

  Abstract

  Controversy exists over whether PHTN in heart transplant candidates increases post-transplant mortality. We performed analysis of data reported to UNOS for children who underwent primary heart transplantation for cardiomyopathy from January 1994 to June 2010. Patients were divided into two groups depending on their pre-transplant TPG: no-PHTN (TPG ≤ 12 mmHg) and PHTN (TPG >12 mm Hg). A total of 6139 children underwent transplantation of whom 2456 (40%) were for cardiomyopathies; 1322 (54%) of these had catheterization data available. The PHTN group (mean TPG 19.5 ± 8.6) had 312 patients and no-PHTN (TPG 6.7 ± 4.0) had 1010. Mortality at one month (4.5% vs. 2.3%) and three months (6.1% vs. 3.1%) post-transplant was significantly higher in the PHTN than the no-PHTN group with an odds ratio of 2 (p < 0.05). There was no significant effect of PHTN on early mortality in children <1 yr age. There was no significant improvement in early survival for transplants performed after compared to before 2003 in patients with PHTN despite availability of pulmonary dilators. Pre-transplant PHTN increases early post-transplant mortality in pediatric cardiomyopathy patients above one yr of age. There has been no significant improvement in the outcome of this group over the last seven yr.

  View details for DOI 10.1111/j.1399-3046.2012.01678.x

  View details for PubMedID 22471690

• NT-proBNP Levels Improve the Ability of Predicting a Hemodynamically Significant Patent Ductus Arteriosus in Very Low-Birth-Weight Infants JOURNAL OF CLINICAL NEONATOLOGY Buddhe, S., Dhuper, S., Kim, R., Weichbrod, L., Mahdi, E., Shah, N., Kona, S., Sokal, M. 2012; 1 (2): 82-86

  Abstract

  A hemodynamically significant Patent ductus arteriosus (HsPDA) in premature infants is known to be associated with significant morbidity. Recently brain natriuretic peptides and superior mesenteric artery (SMA)-resistive indices have been used to effectively diagnose HsPDA.To assess the sensitivity and specificity of N-terminal proBNP (NT-proBNP) in predicting an HsPDA diagnosed by clinical and echocardiographic criteria including pulsatility index (PI) of SMA.All preterm neonates <1500 g were evaluated with echocardiograms and NT-proBNP levels on the 3(rd) to 5(th) day of life and then every week until the echo showed either a closed PDA or non-HsPDA.Sixty-nine babies with mean gestational age of 27 weeks were included in the study. NT-proBNP levels were significantly higher in the HsPDA group (n=22) with a mean±SEM of 24420±3190 compared to 3072±332 in the non-HsPDA group (n=47) (P<0.001). NT-pro BNP level of 5900 pg/ml had 96% sensitivity and 90% specificity of predicting HsPDA.With frequently changing hemodynamics in low-birth weight infants, including NT-proBNP and PI of SMA improve the ability of assessing the effects of a HsPDA and will help timing of intervention.

  View details for DOI 10.4103/2249-4847.96758

  View details for Web of Science ID 000218496800008

  View details for PubMedID 24027696

  View details for PubMedCentralID PMC3743145

• Association of Lipid Abnormalities With Measures and Severity of Adiposity and Insulin Resistance Among Overweight Children and Adolescents JOURNAL OF CLINICAL HYPERTENSION Dhuper, S., Sakowitz, S., Daniels, J., Buddhe, S., Cohen, H. W. 2009; 11 (10): 594-600

  Abstract

  Obesity and lipid abnormalities in children may increase premature cardiovascular disease risk, but the relationship of dyslipidemia with adiposity among obese children is not well defined. The authors performed a cross-sectional analysis of children and adolescents (N=698) in 3 age groups (3-8 years, 9-11 years, and 12-18 years; 53% female, 81% African American, and 16% Hispanic) attending an obesity treatment program. More than 50% of the sample had abnormal levels of triglycerides (TG) or high-density lipoprotein (HDL) cholesterol or both. Only HDL cholesterol and TG were significantly associated with adiposity measures and insulin resistance (measured by homeostasis model assessment [HOMA]) and only in adolescents. All measures of adiposity, adjusted for age and sex, among adolescents were modest predictors of abnormal TG and HDL cholesterol, but these associations were attenuated when adjusting for HOMA. Despite the high prevalence of dyslipidemia in overweight children and adolescents, severity of adiposity appears to be a poor predictor of lipid values except among adolescents. Insulin resistance may in part mediate the relationship of adiposity and dyslipidemia among obese adolescents.

  View details for DOI 10.1111/j.1559-4572.2009.00056.x

  View details for Web of Science ID 000270588700009

  View details for PubMedID 19817943

  View details for PubMedCentralID PMC8673010