Publications by authors named "Cynthia K Rigsby"

98 Publications

Cardiovascular magnetic resonance imaging in children after recovery from symptomatic COVID-19 or MIS-C: a prospective study.

J Cardiovasc Magn Reson 2021 07 1;23(1):86. Epub 2021 Jul 1.

Division of Cardiology, Department of Pediatrics, Ann & Robert H. Lurie Children's Hospital of Chicago, Northwestern University Feinberg School of Medicine, 225 E. Chicago Ave., Box 21, Chicago, IL, 60611, USA.

Background: Cardiac evaluations, including cardiovascular magnetic resonance (CMR) imaging and biomarker results, are needed in children during mid-term recovery after infection with SARS-CoV-2. The incidence of CMR abnormalities 1-3 months after recovery is over 50% in older adults and has ranged between 1 and 15% in college athletes. Abnormal cardiac biomarkers are common in adults, even during recovery.

Methods: We performed CMR imaging in a prospectively-recruited pediatric cohort recovered from COVID-19 and multisystem inflammatory syndrome in children (MIS-C). We obtained CMR data and serum biomarkers. We compared these results to age-matched control patients, imaged prior to the SARS-CoV-2 pandemic.

Results: CMR was performed in 17 children (13.9 years, all ≤ 18 years) and 29 age-matched control patients without SARS-CoV-2 infection. Cases were recruited with symptomatic COVID-19 (11/17, 65%) or MIS-C (6/17, 35%) and studied an average of 2 months after diagnosis. All COVID-19 patients had been symptomatic with fever (73%), vomiting/diarrhea (64%), or breathing difficulty (55%) during infection. Left ventricular and right ventricular ejection fractions were indistinguishable between cases and controls (p = 0.66 and 0.70, respectively). Mean native global T1, global T2 values and segmental T2 maximum values were also not statistically different from control patients (p ≥ 0.06 for each). NT-proBNP and troponin levels were normal in all children.

Conclusions: Children prospectively recruited following SARS-CoV-2 infection had normal CMR and cardiac biomarker evaluations during mid-term recovery. Trial Registration Not applicable.
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http://dx.doi.org/10.1186/s12968-021-00786-5DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8245157PMC
July 2021

A Retrospective Cohort Study of Optimal Contrast for Successful Intussusception Reduction: Institutional Practices Matter.

J Surg Res 2021 Jun 17;267:159-166. Epub 2021 Jun 17.

Division of Pediatric Surgery, Department of Surgery, Northwestern University Feinberg School of Medicine, Ann & Robert H. Lurie Children's Hospital of Chicago, Chicago, Illinois. Electronic address:

Background: The first-line treatment for intussusception is radiologic reduction with either air-contrast enema (AE) or liquid-contrast enema (LE). The purpose of this study was to explore relationships between self-reported institutional AE or LE intussusception reduction preferences and rates of operative intervention and bowel resection.

Methods: Pediatric Health Information System (PHIS) hospitals were contacted to assess institutional enema practices for intussusception. A retrospective study using 2009-2018 PHIS data was conducted for patients aged 0-5 y to evaluate outcomes. Chi-squared tests were used to test for differences in the distribution of surgical patients by hospital management approach.

Results: Of the 45 hospitals, 20 (44%) exclusively used AE, 4 (9%) exclusively used LE, and 21 (46%) used a mixed practice. Of 24,688 patients identified from PHIS, 13,231 (54%) were at exclusive AE/LE hospitals and 11,457 (46%) were at mixed practice hospitals. Patients at AE/LE hospitals underwent operative procedures at lower rates than at mixed practice hospitals (14.8% versus 16.5%, P< 0.001) and were more likely to undergo bowel resection (31.1% versus 27.1%, P= 0.02).

Conclusions: Practice variation exists in hospital-level approaches to radiologic reduction of intussusception and mixed practices may impact outcomes.
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http://dx.doi.org/10.1016/j.jss.2021.05.020DOI Listing
June 2021

Four-dimensional Multiphase Steady-State MRI with Ferumoxytol Enhancement: Early Multicenter Feasibility in Pediatric Congenital Heart Disease.

Radiology 2021 Jul 20;300(1):162-173. Epub 2021 Apr 20.

From the Diagnostic Cardiovascular Imaging Laboratory, Department of Radiological Sciences (K.L.N., T.Y., A.B., P.H., J.P.F.), and Division of Cardiology (K.L.N.), David Geffen School of Medicine at UCLA, 300 Medical Plaza, B119, Los Angeles, CA 90095; VA Greater Los Angeles Healthcare System, Los Angeles, Calif (K.L.N.); Division of Cardiology, Children's Hospital of Philadelphia, Philadelphia, Pa (R.M.G., M.A.F., K.K.W.); Department of Medical Imaging, Ann & Robert H. Lurie Children's Hospital, Chicago, Ill (L.M.G., C.K.R.); and Department of Radiology, Northwestern University Feinberg School of Medicine, Chicago, Ill (L.M.G., C.K.R.).

Background The value of MRI in pediatric congenital heart disease (CHD) is well recognized; however, the requirement for expert oversight impedes its widespread use. Four-dimensional (4D) multiphase steady-state imaging with contrast enhancement (MUSIC) is a cardiovascular MRI technique that uses ferumoxytol and captures all anatomic features dynamically. Purpose To evaluate multicenter feasibility of 4D MUSIC MRI in pediatric CHD. Materials and Methods In this prospective study, participants with CHD underwent 4D MUSIC MRI at 3.0 T or 1.5 T between 2014 and 2020. From a pool of 460 total studies, an equal number of MRI studies from three sites ( = 60) was chosen for detailed analysis. With use of a five-point scale, the feasibility of 4D MUSIC was scored on the basis of artifacts, image quality, and diagnostic confidence for intracardiac and vascular connections ( = 780). Respiratory motion suppression was assessed by using the signal intensity profile. Bias between 4D MUSIC and two-dimensional (2D) cine imaging was evaluated by using Bland-Altman analysis; 4D MUSIC examination duration was compared with that of the local standard for CHD. Results A total of 206 participants with CHD underwent MRI at 3.0 T, and 254 participants underwent MRI at 1.5 T. Of the 60 MRI examinations chosen for analysis (20 per site; median participant age, 14.4 months [interquartile range, 2.3-49 months]; 33 female participants), 56 (93%) had good or excellent image quality scores across a spectrum of disease complexity (mean score ± standard deviation: 4.3 ± 0.6 for site 1, 4.9 ± 0.3 for site 2, and 4.6 ± 0.7 for site 3; < .001). Artifact scores were inversely related to image quality ( = -0.88, < .001) and respiratory motion suppression ( < .001, = -0.45). Diagnostic confidence was high or definite in 730 of 780 (94%) intracardiac and vascular connections. The correlation between 4D MUSIC and 2D cine ventricular volumes and ejection fraction was high (range of = 0.72-0.85; < .001 for all). Compared with local standard MRI, 4D MUSIC reduced the image acquisition time (44 minutes ± 20 vs 12 minutes ± 3, respectively; < .001). Conclusion Four-dimensional multiphase steady-state imaging with contrast enhancement MRI in pediatric congenital heart disease was feasible in a multicenter setting, shortened the examination time, and simplified the acquisition protocol, independently of disease complexity. Clinical trial registration no. NCT02752191 © RSNA, 2021 See also the editorial by Roest and Lamb in this issue.
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http://dx.doi.org/10.1148/radiol.2021203696DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8248952PMC
July 2021

Multi-institution assessment of the use and risk of cardiovascular computed tomography in pediatric patients with congenital heart disease.

J Cardiovasc Comput Tomogr 2021 Jan 27. Epub 2021 Jan 27.

Minneapolis Heart Institute and Foundation, Minneapolis, MN, USA; Children's Minnesota, Minneapolis, MN, USA. Electronic address:

Background: Cardiac computed tomography (CT) is increasingly used in pediatric patients with congenital heart disease (CHD). Variability of practice and of comprehensive diagnostic risk across institutions is not known.

Methods: Four centers prospectively enrolled consecutive pediatric CHD patients <18 years of age undergoing cardiac CT from January 6, 2017 to 1/30/2020. Patient characteristics, cardiac CT data and comprehensive diagnostic risk were compared by age and institutions. Risk categories included sedation and anesthesia use, vascular access, contrast exposure, cardiovascular medication, adverse events (AEs), and estimated radiation dose.

Results: Cardiac CT was performed in 1045 pediatric patients at a median (interquartile range, IQR) age of 1.7 years (0.3, 11.0). The most common indications were arterial abnormalities, suspected coronary artery anomalies, functionally single ventricle heart disease, and tetralogy of Fallot/pulmonary atresia. Sedation was used in 8% and anesthesia in 11% of patients. Peripheral vascular access was utilized for 93%. Median contrast volume was 2 ​ml/kg. Beta blockers were administered in 11% of cases and nitroglycerin in 2% of cases. The median (IQR) total procedural dose length product (DLP) was 20 ​mGy∗cm (10, 50). Sedation, vascular access, contrast exposure, use of cardiovascular medications and radiation dose estimates varied significantly by institution and age (p ​< ​0.001). Seven minor adverse events (0.7%) and no major adverse events were reported.

Conclusion: Cardiac CT for CHD is safe in pediatric patients when appropriate CT technology and expertise are available. Scans can be acquired at relatively low radiation exposure with few minor adverse events.
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http://dx.doi.org/10.1016/j.jcct.2021.01.003DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8313631PMC
January 2021

Influence of Fetal Diagnosis on Management of Vascular Rings.

Ann Thorac Surg 2021 Jan 29. Epub 2021 Jan 29.

UK HealthCare Kentucky Children's Hospital, Lexington, Kentucky; and Department of Cardiothoracic Surgery, Heart Institute, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio. Electronic address:

Background: There has been an increasing frequency of fetal diagnosis of vascular rings. We compared management strategies and outcomes of infants with fetal diagnosis to those with postnatal diagnosis to inform recommendations regarding optimal management.

Methods: Retrospective review was performed of vascular ring operations from 1/2000 to 6/2019. Standard demographic data (preoperative clinical status, timing of diagnosis, cross-sectional imaging, operative and perioperative details, and clinical outcomes) were collected. Statistical analysis was performed to compare characteristics and outcomes of fetal versus postnatal diagnosis.

Results: There were 190 patients, with 15% (n=29) diagnosed prenatally. Anatomic variants were: double aortic arch (n=66, 14 fetal diagnosis), right aortic arch, aberrant left subclavian artery (n=94, 12 fetal diagnosis), circumflex aorta (n=7, 1 fetal diagnosis), and pulmonary artery sling (n=19, 2 fetal diagnoses). Increasing frequency of fetal diagnosis was noted in the past 10 years. In 2012 1/9 (11%) patients had a fetal diagnosis, in 2018 8/11 (72%) had a fetal diagnosis (p<0.001). Patients with a fetal diagnosis were significantly younger at the time of surgery (13.1 months [20.6] vs. 24.0 months [87.0], p=0.029). There was no difference in postoperative complications or length-of-stay (3 days [1] for fetal diagnosis vs. 4 days [3] for postnatal diagnosis, p=0.50).

Conclusions: Fetal diagnosis leads to the potential for expectant management of vascular ring patients. This has resulted in earlier time of intervention with no increase in postoperative morbidity. This may lead to improved long-term outcomes and potentially alter the natural history for these children.
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http://dx.doi.org/10.1016/j.athoracsur.2021.01.025DOI Listing
January 2021

5D Flow MRI: A Fully Self-gated, Free-running Framework for Cardiac and Respiratory Motion-resolved 3D Hemodynamics.

Radiol Cardiothorac Imaging 2020 Nov 12;2(6):e200219. Epub 2020 Nov 12.

Departments of Radiology, Feinberg School of Medicine (L.E.M., J.C.C., C.K.R., D.K., M.M.) and Biomedical Engineering (L.E.M., J.C.C., D.K., M.M.), Northwestern University, 737 N Michigan Ave, Suite 1600, Chicago, IL 60611; Department of Diagnostic and Interventional Radiology, Lausanne University Hospital and University of Lausanne, Lausanne, Switzerland (J.Y., D.P., L.D.S., C.W.R., M.S.); Center for Biomedical Imaging, Lausanne, Switzerland (J.Y., M.S.); Department of Advanced Clinical Imaging Technology, Siemens Healthineers, Lausanne, Switzerland (D.P.); and Department of Medical Imaging, Ann and Robert H. Lurie Children's Hospital of Chicago, Chicago, Ill (C.K.R.).

Purpose: To implement, validate, and apply a self-gated free-running whole-heart five-dimensional (5D) flow MRI framework to evaluate respiration-driven effects on three-dimensional (3D) hemodynamics in a clinical setting.

Materials And Methods: In this prospective study, a free-running five-dimensional (5D) flow sequence was implemented with 3D radial sampling, self-gating, and a compressed-sensing reconstruction. The 5D flow was evaluated in a pulsatile phantom and adult participants with aortic and/or valvular disease who were enrolled between May and August 2019. Conventional twofold-accelerated four-dimensional (4D) flow of the thoracic aorta with navigator gating was performed as a reference comparison. Continuous parameters were evaluated for parameter normality and were compared between conventional 4D flow and 5D flow using a signed-rank or two-tailed paired test. Differences between respiratory states were evaluated using a repeated-measure analysis of variance or a nonparametric Friedman test.

Results: A total of 20 adult participants (mean age, 49 years ± 17 [standard deviation]; 18 men and two women) were included. In vitro 5D flow results showed excellent agreement with conventional 4D flow-derived values (peak and net flow, <7% difference over all quantified planes). Whole-heart 5D flow data were collected in all participants in 7.65 minutes ± 0.35 (acceleration rate = 36.0-76.9) versus 9.88 minutes ± 3.17 for conventional aortic 4D flow. In vivo, 5D flow demonstrated moderate agreement with conventional 4D flow but demonstrated overestimation in net flow and peak velocity (up to 26% and 12%, respectively) in the ascending aorta and underestimation (<12%) in the arch and descending aorta. Respiratory-resolved analyses of caval veins showed significantly increased net and peak flow in the inferior vena cava in end inspiration compared with end expiration, and the opposite trend was shown in the superior vena cava.

Conclusion: A free-running 5D flow MRI framework consistently captured cardiac and respiratory motion-resolved 3D hemodynamics in less than 8 minutes. © RSNA, 2020.
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http://dx.doi.org/10.1148/ryct.2020200219DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7755133PMC
November 2020

ACR Appropriateness Criteria® Vomiting in Infants.

J Am Coll Radiol 2020 Nov;17(11S):S505-S515

Specialty Chair, Riley Hospital for Children Indiana University, Indianapolis, Indiana.

Vomiting in infants under the age of 3 months is one of the most common reasons for parents to seek care from their doctor or present to an emergency room. The imaging workup that ensues is dependent on several factors: age at onset, days versus weeks after birth, quality of emesis, bilious or nonbilious vomiting, and the initial findings on plain radiograph, suspected proximal versus distal bowel obstruction. The purpose of these guidelines is to inform the clinician, based on current evidence, what is the next highest yield and most appropriate imaging study to pursue a diagnosis. The goal is rapid and accurate arrival at a plan for treatment, whether surgical or nonsurgical. The following modalities are discussed for each variant of the symptom: plain radiography, fluoroscopic upper gastrointestinal series, fluoroscopic contrast enema, ultrasound of the abdomen, nuclear medicine gastroesophageal reflux scan. The American College of Radiology Appropriateness Criteria are evidence-based guidelines for specific clinical conditions that are reviewed annually by a multidisciplinary expert panel. The guideline development and revision include an extensive analysis of current medical literature from peer reviewed journals and the application of well-established methodologies (RAND/UCLA Appropriateness Method and Grading of Recommendations Assessment, Development, and Evaluation or GRADE) to rate the appropriateness of imaging and treatment procedures for specific clinical scenarios. In those instances where evidence is lacking or equivocal, expert opinion may supplement the available evidence to recommend imaging or treatment.
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http://dx.doi.org/10.1016/j.jacr.2020.09.002DOI Listing
November 2020

ACR Appropriateness Criteria® Antenatal Hydronephrosis-Infant.

J Am Coll Radiol 2020 Nov;17(11S):S367-S379

Specialty Chair, Riley Hospital for Children Indiana University, Indianapolis, Indiana.

Antenatal hydronephrosis is the most frequent urinary tract anomaly detected on prenatal ultrasonography. It occurs approximately twice as often in males as in females. Most antenatal hydronephrosis is transient with little long-term significance, and few children with antenatal hydronephrosis will have significant obstruction, develop symptoms or complications, and require surgery. Some children will be diagnosed with more serious conditions, such as posterior urethral valves. Early detection of obstructive uropathy is necessary to mitigate the potential morbidity from loss of renal function. Imaging is an integral part of screening, diagnosis, and monitoring of children with antenatal hydronephrosis. Optimal timing and appropriate use of imaging can reduce the incidence of late diagnoses and prevent renal scarring and other complications. In general, follow-up neonatal ultrasound is recommended for all cases of antenatal hydronephrosis, while further imaging, including voiding cystourethrography and nuclear scintigraphy, is recommended for moderate or severe cases, or when renal parenchymal or bladder wall abnormalities are suspected. The American College of Radiology Appropriateness Criteria are evidence-based guidelines for specific clinical conditions that are reviewed annually by a multidisciplinary expert panel. The guideline development and revision include an extensive analysis of current medical literature from peer reviewed journals and the application of well-established methodologies (RAND/UCLA Appropriateness Method and Grading of Recommendations Assessment, Development, and Evaluation or GRADE) to rate the appropriateness of imaging and treatment procedures for specific clinical scenarios. In those instances where evidence is lacking or equivocal, expert opinion may supplement the available evidence to recommend imaging or treatment.
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http://dx.doi.org/10.1016/j.jacr.2020.09.017DOI Listing
November 2020

Evolution and Current Results of a Unified Strategy for Sinus Venosus Surgery.

Ann Thorac Surg 2021 03 11;111(3):980-986. Epub 2020 May 11.

Division of Cardiovascular-Thoracic Surgery, Ann & Robert H. Lurie Children's Hospital of Chicago, Chicago, Illinois; Department of Surgery, Northwestern University Feinberg School of Medicine, Chicago, Illinois. Electronic address:

Background: Given recent reports of percutaneous closure of sinus venosus atrial septal defects, we reviewed our experience with surgical repair. Owing to the high incidence of arrhythmias with the two-patch technique, since 2001 we have used either one-patch repairs or the Warden procedure.

Methods: A retrospective review was performed of pediatric patients undergoing sinus venosus atrial septal defect repair at our institution from January 1, 1990, to July 1, 2018. Standard demographic data such as echocardiographic and cross-sectional imaging along with operative details and clinical echocardiographic outcomes were collected.

Results: The cohort included 144 patients with a median age of 4.3 years (interquartile range, 8.5). Inferior SVASD was present in 24 patients (17%). A single autologous untreated pericardial patch was used for 114 patients (79%), a two-patch technique for 20 patients (14%, last performed in 2000), and a Warden procedure in 10 patients (7%). Median length of stay was 4 days (interquartile range, 2). On echocardiogram follow-up, no patient had pulmonary vein stenosis. One patient who had the Warden procedure required a balloon dilation of the superior caval vein 2 years postoperatively and a stent 3 years later. Two-patch patients were substantially less likely to be in normal sinus rhythm (41%) on postoperative electrocardiograms compared with the other two techniques (81% one-patch and 89% Warden, P = .02).

Conclusions: The great majority of patients with sinus venosus atrial septal defects can be successfully repaired with a single patch of autologous pericardium. We transitioned to using either a single pericardial patch or the Warden procedure, resulting in a higher frequency of normal sinus rhythm on postoperative electrocardiograms.
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http://dx.doi.org/10.1016/j.athoracsur.2020.03.113DOI Listing
March 2021

ACR Appropriateness Criteria® Pneumonia in the Immunocompetent Child.

J Am Coll Radiol 2020 May;17(5S):S215-S225

Specialty Chair, Riley Hospital for Children Indiana University, Indianapolis, Indiana.

Pneumonia is one of the most common acute infections and the single greatest infectious cause of death in children worldwide. In uncomplicated, community-acquired pneumonia in immunocompetent patients, the diagnosis is clinical and imaging has no role. The first role of imaging is to identify complications associated with pneumonia such as pleural effusion, pulmonary abscess, and bronchopleural fistula. Radiographs are recommended for screening for these complications and ultrasound and CT are recommended for confirmation. The second role of imaging is to identify underlying anatomic conditions that may predispose patients to recurrent pneumonia. CT with intravenously administered contrast is recommended for this evaluation. The American College of Radiology Appropriateness Criteria are evidence-based guidelines for specific clinical conditions that are reviewed annually by a multidisciplinary expert panel. The guideline development and revision include an extensive analysis of current medical literature from peer reviewed journals and the application of well-established methodologies (RAND/UCLA Appropriateness Method and Grading of Recommendations Assessment, Development, and Evaluation or GRADE) to rate the appropriateness of imaging and treatment procedures for specific clinical scenarios. In those instances where evidence is lacking or equivocal, expert opinion may supplement the available evidence to recommend imaging or treatment.
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http://dx.doi.org/10.1016/j.jacr.2020.01.033DOI Listing
May 2020

Progress in Improving Readability of RadiologyInfo.org.

AJR Am J Roentgenol 2020 05;214(5):W78

University of Virginia School of Medicine, Charlottesville, VA.

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http://dx.doi.org/10.2214/AJR.19.22362DOI Listing
May 2020

Re: "Readability of Spanish-Language Patient Education Materials From RadiologyInfo.org".

J Am Coll Radiol 2020 05 11;17(5):564-565. Epub 2020 Mar 11.

Ann and Robert H. Lurie Children's Hospital of Chicago, Northwestern Feinberg School of Medicine, Chicago, Illinois.

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http://dx.doi.org/10.1016/j.jacr.2020.02.009DOI Listing
May 2020

Fully automated 3D aortic segmentation of 4D flow MRI for hemodynamic analysis using deep learning.

Magn Reson Med 2020 10 13;84(4):2204-2218. Epub 2020 Mar 13.

Department of Biomedical Engineering, Northwestern University, Chicago, Illinois.

Purpose: To generate fully automated and fast 4D-flow MRI-based 3D segmentations of the aorta using deep learning for reproducible quantification of aortic flow, peak velocity, and dimensions.

Methods: A total of 1018 subjects with aortic 4D-flow MRI (528 with bicuspid aortic valve, 376 with tricuspid aortic valve and aortic dilation, 114 healthy controls) comprised the data set. A convolutional neural network was trained to generate 3D aortic segmentations from 4D-flow data. Manual segmentations served as the ground truth (N = 499 training, N = 101 validation, N = 418 testing). Dice scores, Hausdorff distance, and average symmetrical surface distance were calculated to assess performance. Aortic flow, peak velocity, and lumen dimensions were quantified at the ascending, arch, and descending aorta and compared using Bland-Altman analysis. Interobserver variability of manual analysis was assessed on a subset of 40.

Results: Convolutional neural network segmentation required 0.438 ± 0.355 seconds versus 630 ± 254 seconds for manual analysis and demonstrated excellent performance with a median Dice score of 0.951 (0.930-0.966), Hausdorff distance of 2.80 (2.13-4.35), and average symmetrical surface distance of 0.176 (0.119-0.290). Excellent agreement was found for flow, peak velocity, and dimensions with low bias and limits of agreement less than 10% difference versus manual analysis. For aortic volume, limits of agreement were moderate within 16.3%. Interobserver variability (median Dice score: 0.950; Hausdorff distance: 2.45; and average symmetrical surface distance: 0.145) and convolutional neural network-based analysis (median Dice score: 0.953-0.959; Hausdorff distance: 2.24-2.91; and average symmetrical surface distance: 0.145-1.98 to observers) demonstrated similar reproducibility.

Conclusions: Deep learning enabled fast and automated 3D aortic segmentation from 4D-flow MRI, demonstrating its potential for efficient clinical workflows. Future studies should investigate its utility for other vasculature and multivendor applications.
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http://dx.doi.org/10.1002/mrm.28257DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7724647PMC
October 2020

Highly accelerated, real-time phase-contrast MRI using radial k-space sampling and GROG-GRASP reconstruction: a feasibility study in pediatric patients with congenital heart disease.

NMR Biomed 2020 05 24;33(5):e4240. Epub 2020 Jan 24.

Department of Radiology, Northwestern University Feinberg School of Medicine, Chicago, Illinois.

Retrospective electrocardiogram-gated, 2D phase-contrast (PC) flow MRI is routinely used in clinical evaluation of valvular/vascular disease in pediatric patients with congenital heart disease (CHD). In patients not requiring general anesthesia, clinical standard PC is conducted with free breathing for several minutes per slice with averaging. In younger patients under general anesthesia, clinical standard PC is conducted with breath-holding. One approach to overcome this limitation is using either navigator gating or self-navigation of respiratory motion, at the expense of lengthening scan times. An alternative approach is using highly accelerated, free-breathing, real-time PC (rt-PC) MRI, which to date has not been evaluated in CHD patients. The purpose of this study was to develop a 38.4-fold accelerated 2D rt-PC pulse sequence using radial k-space sampling and compressed sensing with 1.5 × 1.5 × 6.0 mm nominal spatial resolution and 40 ms nominal temporal resolution, and evaluate whether it is capable of accurately measuring flow in 17 pediatric patients (aortic valve, pulmonary valve, right and left pulmonary arteries) compared with clinical standard 2D PC (either breath-hold or free breathing). For clinical translation, we implemented an integrated reconstruction pipeline capable of producing DICOMs of the order of 2 min per time series (46 frames). In terms of association, forward volume, backward volume, regurgitant fraction, and peak velocity at peak systole measured with standard PC and rt-PC were strongly correlated (R > 0.76; P < 0.001). Compared with clinical standard PC, in terms of agreement, forward volume (mean difference = 1.4% (3.0% of mean)) and regurgitant fraction (mean difference = -2.5%) were in good agreement, whereas backward volume (mean difference = -1.1 mL (28.2% of mean)) and peak-velocity at peak systole (mean difference = -21.3 cm/s (17.2% of mean)) were underestimated by rt-PC. This study demonstrates that the proposed rt-PC with the said spatial resolution and temporal resolution produces relatively accurate forward volumes and regurgitant fractions but underestimates backward volumes and peak velocities at peak systole in pediatric patients with CHD.
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http://dx.doi.org/10.1002/nbm.4240DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7165070PMC
May 2020

Eccentric Enlargement of the Aortic Sinuses in Pediatric and Adult Patients with Bicuspid Aortic Valves: A Cardiac MRI Study.

Pediatr Cardiol 2020 Feb 19;41(2):350-360. Epub 2019 Dec 19.

Department of Medical Imaging, Ann & Robert H Lurie Children's Hospital of Chicago, Chicago, IL, USA.

Aortic root size and cusp fusion pattern have been related to disease outcomes in bicuspid aortic valve (BAV). This study seeks to characterize symmetry of the aortic sinuses in adult and pediatric BAV patients and its relationship to valvulopathy and root aortopathy. Aortic sinus-to-commissure (S-C) lengths were measured on cardiac MRI of adult and pediatric BAV patients with right-and-left coronary (RL) or right-and-non-coronary (RN) leaflet fusion and tricuspid aortic valve (TAV) controls. Coefficient of variance (CoV) of S-C lengths was calculated to quantify sinus asymmetry, or eccentricity. BAV cohort included 149 adults (48 ± 15 years) and 51 children (15 ± 5 years). TAV cohort included 40 adults (60 ± 13 years) and 20 children (15 ± 5 years). In adult and pediatric BAV patients, the non-fused aortic sinus was larger than either fused sinus. In RL fusion, the non-coronary S-C distance was larger than right or left S-C distances in adults (n = 121, p < 0.001) and larger than the right S-C distance in children (n = 41, p = 0.013). Sinus eccentricity (CoV) in BAV patients was higher than in TAV patients (p < 0.001) and did not correlate with age (p = 0.12). CoV trended higher in RL adults with aortic regurgitation (AR) compared to those without AR (p = 0.081), but was lower in RN adults with AR than without AR (p = 0.006). CoV did not correlate to root Z scores (p = 0.06-0.55) or ascending aortic (AAo) Z scores in adults (p = 0.45-0.55) but correlated negatively to AAo Z score in children (p = 0.005-0.03). Most adult and pediatric BAV patients with RL and RN leaflet fusion demonstrate eccentric dominance of the non-fused aortic sinus irrespective of age. The degree of eccentricity varies with valve dysfunction and BAV phenotype but does not relate to the degree of aortic root dilatation, nor does eccentricity correlate with ascending aorta dilatation in adults.
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http://dx.doi.org/10.1007/s00246-019-02264-3DOI Listing
February 2020

Altered regional myocardial velocities by tissue phase mapping and feature tracking in pediatric patients with hypertrophic cardiomyopathy.

Pediatr Radiol 2020 02 28;50(2):168-179. Epub 2019 Oct 28.

Department of Radiology, Feinberg School of Medicine, Northwestern University, Chicago, IL, USA.

Background: Hypertrophic cardiomyopathy (HCM) is associated with heart failure, atrial fibrillation and sudden death. Reduced myocardial function has been reported in HCM despite normal left ventricular (LV) ejection fraction. Additionally, LV fibrosis is associated with elevated T1 and might be an outcome predictor.

Objective: To systematically compare tissue phase mapping and feature tracking for assessing regional LV function in children and young adults with HCM and pediatric controls, and to evaluate structure-function relationships among myocardial velocities, LV wall thickness and myocardial T1.

Materials And Methods: Seventeen pediatric patients with HCM and 21 age-matched controls underwent cardiac MRI including standard cine imaging, tissue phase mapping (two-dimensional cine phase contrast with three-directional velocity encoding), and modified Look-Locker inversion recovery to calculate native global LV T1. Maximum LV wall thickness was measured on cine images. LV radial, circumferential and long-axis myocardial velocity time courses, as well as global and segmental systolic and diastolic peak velocities, were quantified from tissue phase mapping and feature tracking.

Results: Both tissue phase mapping and feature tracking detected significantly decreased global and segmental diastolic radial and long-axis peak velocities (by 12-51%, P<0.001-0.05) in pediatric patients with HCM vs. controls. Feature tracking peak velocities were lower than directly measured tissue phase mapping velocities (mean bias = 0.3-2.9 cm/s). Diastolic global peak velocities correlated moderately with global T1 (r = -0.57 to -0.72, P<0.01) and maximum wall thickness (r = -0.37 to -0.61, P<0.05).

Conclusion: Both tissue phase mapping and feature tracking detected myocardial velocity changes in children and young adults with HCM vs. controls. Associations between impaired diastolic LV velocities and elevated T1 indicate structure-function relationships in HCM.
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http://dx.doi.org/10.1007/s00247-019-04549-4DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6982608PMC
February 2020

Multicenter Safety and Practice for Off-Label Diagnostic Use of Ferumoxytol in MRI.

Radiology 2019 12 22;293(3):554-564. Epub 2019 Oct 22.

From the Diagnostic Cardiovascular Imaging Research Laboratory, Department of Radiological Sciences, David Geffen School of Medicine at UCLA, 300 Medical Plaza, Suite B119, Los Angeles, CA 90095 (K.L.N., T.Y., P.H., J.P.F.); Division of Cardiology, David Geffen School of Medicine at UCLA and VA Greater Los Angeles Healthcare System, Los Angeles, Calif (K.L.N., N.K.); Department of Radiology (I.H.Z., M.R.B.), Center for Advanced Magnetic Resonance Development (I.H.Z., M.R.B.), and Division of Gastroenterology, Department of Medicine (M.R.B.), Duke University Medical Center, Durham, NC; Department of Diagnostic Radiology and Neurology, Oregon Health Sciences University, Portland, Ore (C.G.V.); British Heart Foundation Centre for Cardiovascular Science, University of Edinburgh, Edinburgh, Scotland (S.I.S., D.E.N.); Department of Imaging, Cedars-Sinai Medical Center, Los Angeles, Calif (R.S.); Department of Medical Imaging, Ann & Robert H. Lurie Children's Hospital of Chicago, Chicago, Ill (C.K.R., L.M.G.); Department of Radiology, Northwestern University Feinberg School of Medicine, Chicago, Ill (C.K.R., L.M.G.); Institute of Cardiovascular and Medical Sciences, University of Glasgow, Scotland (S.S., A.R.); Division of Cardiology, Department of Pediatrics and Radiology, Children's Hospital of Philadelphia, Philadelphia, Pa (K.K.W., M.A.F.); Department of Radiology, University of Wisconsin, Madison, Wis (L.M.G., M.L.S.); Department of Radiology, University of California, San Francisco and VA San Francisco Healthcare System, San Francisco, Calif (D.S., M.D.H.); Department of Radiology, Weill Medical College of Cornell University, New York, NY (M.R.P.); Department of Radiology, NHS Greater Glasgow and Clyde, and Institute of Cardiovascular and Medical Sciences, University of Glasgow, Glasgow, Scotland (G.H.R.); and Department of Neurology and Neurosurgery, Oregon Health Sciences University and VA Portland Healthcare System, Portland, Ore (E.A.N.).

Background Ferumoxytol is approved for use in the treatment of iron deficiency anemia, but it can serve as an alternative to gadolinium-based contrast agents. On the basis of postmarketing surveillance data, the Food and Drug Administration issued a black box warning regarding the risks of rare but serious acute hypersensitivity reactions during fast high-dose injection (510 mg iron in 17 seconds) for therapeutic use. Whereas single-center safety data for diagnostic use have been positive, multicenter data are lacking. Purpose To report multicenter safety data for off-label diagnostic ferumoxytol use. Materials and Methods The multicenter ferumoxytol MRI registry was established as an open-label nonrandomized surveillance databank without industry involvement. Each center monitored all ferumoxytol administrations, classified adverse events (AEs) using the National Cancer Institute Common Terminology Criteria for Adverse Events (grade 1-5), and assessed the relationship of AEs to ferumoxytol administration. AEs related to or possibly related to ferumoxytol injection were considered adverse reactions. The core laboratory adjudicated the AEs and classified them with the American College of Radiology (ACR) classification. Analysis of variance was used to compare vital signs. Results Between January 2003 and October 2018, 3215 patients (median age, 58 years; range, 1 day to 96 years; 1897 male patients) received 4240 ferumoxytol injections for MRI. Ferumoxytol dose ranged from 1 to 11 mg per kilogram of body weight (≤510 mg iron; rate ≤45 mg iron/sec). There were no systematic changes in vital signs after ferumoxytol administration ( > .05). No severe, life-threatening, or fatal AEs occurred. Eighty-three (1.9%) of 4240 AEs were related or possibly related to ferumoxytol infusions (75 mild [1.8%], eight moderate [0.2%]). Thirty-one AEs were classified as allergiclike reactions using ACR criteria but were consistent with minor infusion reactions observed with parenteral iron. Conclusion Diagnostic ferumoxytol use was well tolerated, associated with no serious adverse events, and implicated in few adverse reactions. Registry results indicate a positive safety profile for ferumoxytol use in MRI. © RSNA, 2019
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http://dx.doi.org/10.1148/radiol.2019190477DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6884068PMC
December 2019

Myocardial velocity, intra-, and interventricular dyssynchrony evaluated by tissue phase mapping in pediatric heart transplant recipients.

J Magn Reson Imaging 2020 04 12;51(4):1212-1222. Epub 2019 Sep 12.

Department of Radiology, Feinberg School of Medicine, Northwestern University, Chicago, Illinois, USA.

Background: Endomyocardial biopsy (EMB) is the standard method for detecting allograft rejection in pediatric heart transplants (Htx). As EMB is invasive and carries a risk of complications, there is a need for a noninvasive alternative for allograft monitoring.

Purpose: To quantify left and right ventricular (LV & RV) peak velocities, velocity twist, and intra-/interventricular dyssynchrony using tissue phase mapping (TPM) in pediatric Htx compared with controls, and to explore the relationship between global cardiac function parameters and the number of rejection episodes to these velocities and intra-/interventricular dyssynchrony.

Study Type: Prospective.

Subjects: Twenty Htx patients (age: 16.0 ± 3.1 years, 11 males) and 18 age- and sex-matched controls (age: 15.5 ± 4.3 years, nine males).

Field Strength/sequence: 5T; 2D balanced cine steady-state free-precession (bSSFP), TPM (2D cine phase contrast with three-directional velocity encoding).

Assessment: LV and RV circumferential, radial, and long-axis velocity-time curves, global and segmental peak velocities were measured using TPM. Short-axis bSSFP images were used to measure global LV and RV function parameters.

Statistical Tests: A normality test (Lilliefors test) was performed on all data. For comparisons, a t-test was used for normally distributed data or a Wilcoxon rank-sum test otherwise. Correlations were determined by a Pearson correlation.

Results: Htx patients had significantly reduced LV (P < 0.05-0.001) and RV (P < 0.05-0.001) systolic and diastolic global and segmental long-axis velocities, reduced RV diastolic peak twist (P < 0.01), and presented with higher interventricular dyssynchrony for long-axis and circumferential motions (P < 0.05-0.001). LV diastolic long-axis dyssynchrony (r = 0.48, P = 0.03) and RV diastolic peak twist (r = -0.64, P = 0.004) significantly correlated with the total number of rejection episodes.

Data Conclusion: TPM detected differences in biventricular myocardial velocities in pediatric Htx patients compared with controls and indicated a relationship between Htx myocardial velocities and rejection history.

Level Of Evidence: 2 Technical Efficacy Stage: 3 J. Magn. Reson. Imaging 2020;51:1212-1222.
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http://dx.doi.org/10.1002/jmri.26916DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7065939PMC
April 2020

Altered 4-D magnetic resonance imaging flow characteristics in complex congenital aortic arch repair.

Pediatr Radiol 2020 01 31;50(1):17-27. Epub 2019 Aug 31.

Northwestern University Feinberg School of Medicine, Chicago, IL, USA.

Background: Interrupted aortic arch (IAA) is a rare but severe congenital abnormality often associated with bicuspid aortic valve (BAV). Complex re-interventions are often needed despite surgical advances, but the impact of aortic hemodynamics in repaired patients is unknown.

Objective: Investigate effect of IAA repairs on aortic hemodynamics, wall shear stress and flow derangements via 4-D flow MRI.

Materials And Methods: We retrospectively analyzed age- and gender-matched cohorts (IAA [n=6], BAV alone [n=6], controls [n=6]) undergoing cardiac MRI including 4-D flow. Aortic dimensions were measured from standard MR angiography. We quantified peak systolic velocities, regurgitant fractions and wall shear stress in the ascending aorta (AAo), transverse arch and descending aorta (DAo) from 4-D flow, and we graded helix/vortex flow patterns from 3-D blood flow visualization.

Results: Children and young adults with IAA had a wide range of arch dimensions, peak systolic velocities, regurgitant fractions and flow grades. Peak transverse arch systolic velocities were higher in patients with IAA versus controls (P=0.02). Flow derangements in the AAo were found in patients with IAA (median grade=2, 5/6 patients, P=0.04) and BAV (median grade=3, 5/6 patients, P=0.03) versus controls. Flow derangements in the DAo were only seen in patients with IAA (median grade=1, 5/6 patients, P=0.04), and 5/6 people with IAA had helical flow in head and neck vessels. Wall shear stress was increased in people with IAA along the superior transverse arch and proximal DAo versus controls (P=0.02).

Conclusion: Complex congenital aortic arch repairs can change aortic hemodynamics. Associated cardiac defects can further alter findings. Studies are warranted to investigate clinical implications in larger cohorts.
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http://dx.doi.org/10.1007/s00247-019-04507-0DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6943192PMC
January 2020

Pediatric Tracheal Surgery: A 25-Year Review of Slide Tracheoplasty and Tracheal Resection.

Ann Thorac Surg 2020 01 7;109(1):148-153. Epub 2019 Aug 7.

Division of Cardiovascular-Thoracic Surgery, Ann & Robert H. Lurie Children's Hospital of Chicago, Chicago, Illinois; Department of Surgery, Northwestern University Feinberg School of Medicine, Chicago, Illinois. Electronic address:

Background: The purpose of this study was to assess the outcomes of slide tracheoplasty and tracheal resection in pediatric patients and analyze the data for predictors of outcomes.

Methods: A retrospective review of tracheal surgery from January 1, 1993 to May 1, 2018 was performed. Demographic data, operative details, perioperative data, and clinical outcomes were collected. The study investigators' management strategy has evolved over time, with less rigid bronchoscopy, more reliance on postoperative computed tomographic imaging, and the use of inhaled Ciprodex (combination of ciprofloxacin and dexamethasone) since 2007.

Results: The study included 41 patients, with a median age of 4.1 months and a median weight of 4.2 kg. There were 6 neonates and 24 infants. Slide tracheoplasty was performed in 27 patients (66%), and resection with end-to-end anastomosis was performed in 14 (34%). Eleven patients (27%) had a pulmonary artery sling. Simultaneous intracardiac repairs requiring cross-clamp and cardioplegia were performed in 9 patients (22%). Lung agenesis (n = 6) or severe hypoplasia (n = 2) was present in 8 patients (20%). Complications included tracheostomy in 5 patients (12%) and in-hospital death in 3 patients (7%). There were no cases of mediastinitis. Inhaled Ciprodex was used to decrease granulation tissue at suture lines. Median intubation time was 7 days, and median length of stay was 25.0 days. There was no difference in outcomes when comparing intracardiac repairs with the remaining patients, lung agenesis or hypoplasia vs the remaining cohort, or neonates vs infants.

Conclusions: Slide tracheoplasty and tracheal resection are effective operative strategies for infants and children with tracheal stenosis, including patients with lung agenesis/hypoplasia. Simultaneous repair of intracardiac anomalies and pulmonary artery sling is recommended.
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http://dx.doi.org/10.1016/j.athoracsur.2019.06.042DOI Listing
January 2020

Comprehensive MR Analysis of Cardiac Function, Aortic Hemodynamics and Left Ventricular Strain in Pediatric Cohort with Isolated Bicuspid Aortic Valve.

Pediatr Cardiol 2019 Oct 24;40(7):1450-1459. Epub 2019 Jul 24.

Department of Medical Imaging, Ann & Robert H Lurie Children's Hospital of Chicago, Chicago, IL, USA.

Bicuspid aortic valve (BAV) disease demonstrates a range of clinical presentations and complications. We aim to use cardiac MRI (CMR) to evaluate left ventricular (LV) parameters, myocardial strain and aortic hemodynamics in pediatric BAV patients with and without aortic stenosis (AS) or regurgitation (AR) compared to tricuspid aortic valve (TAV) controls. We identified 58 pediatric BAV patients without additional cardiovascular pathology and 25 healthy TAV controls (15.3 ± 2.2 years) who underwent CMR with 4D flow. BAV cohort included subgroups with no valvulopathy (n = 13, 14.3 ± 4.7 years), isolated AS (n = 19, 14.5 ± 4.0 years), mixed valve disease (AS + AR) (n = 13, 17.1 ± 3.2 years), and prior valvotomy/valvuloplasty (n = 13, 13.9 ± 3.2 years). CMR data included LV volumetric and mass indices, myocardial strain and aortic hemodynamics. BAV patients with no valvulopathy or isolated AS had similar LV parameters to controls excepting cardiac output (p < 0.05). AS + AR and post-surgical patients had abnormal LV volumetric and mass indices (p < 0.01). Post-surgical patients had decreased global longitudinal strain (p = 0.02); other subgroups had comparable strain to controls. Patients with valvulopathy demonstrated elevated velocity and wall shear stress (WSS) in the ascending aorta (AAo) and arch (p < 0.01), while those without valve dysfunction had only elevated AAo velocity (p = 0.03). Across the cohort, elevated AAo velocity and WSS correlated to higher LV mass (p < 0.01), and abnormal hemodynamics correlated to decreased strain rates (p < 0.045). Pediatric BAV patients demonstrate abnormalities in LV parameters as a function of valvular dysfunction, most significantly in children with AS + AR or prior valvotomy/valvuloplasty. Correlations between aortic hemodynamics, LV mass and strain suggest valvular dysfunction could drive LV remodeling. Multiparametric CMR assessment in pediatric BAV may help stratify risk for cardiac remodeling and dysfunction.
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http://dx.doi.org/10.1007/s00246-019-02157-5DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6786923PMC
October 2019

Diagnostic performance of cardiovascular magnetic resonance native T1 and T2 mapping in pediatric patients with acute myocarditis.

J Cardiovasc Magn Reson 2019 07 15;21(1):40. Epub 2019 Jul 15.

Division of Pediatric Cardiology, Ann & Robert H Lurie Children's Hospital of Chicago Northwestern University, 737 N. Michigan Avenue, Suite 1600 225 E Chicago Avenue, Box 21, Chicago, IL, 60611, USA.

Background: Multiple studies in adult patients suggest that tissue mapping performed by cardiovascular magnetic resonance (CMR) has excellent diagnostic accuracy in acute myocarditis, however, these techniques have not been studied in depth in children.

Methods: CMR data on 23 consecutive pediatric patients from 2014 to 2017 with a clinical diagnosis of acute myocarditis were retrospectively analyzed and compared to 39 healthy controls. The CMR protocol included native T1, T2, and extracellular volume fraction (ECV) in addition to standard Lake Louise Criteria (LLC) parameters on a 1.5 T scanner.

Results: Mean global values for novel mapping parameters were significantly elevated in patients with clinically suspected acute myocarditis compared to controls, with native T1 1098 ± 77 vs 990 ± 34 ms, T2 52.8 ± 4.6 ms vs 46.7 ± 2.6 ms, and ECV 29.8 ± 5.1% vs 23.3 ± 2.6% (all p-values < 0.001). Ideal cutoff values were generated using corresponding ROC curves and were for global T1 1015 ms (AUC 0.936, sensitivity 91%, specificity 86%), for global T2 48.5 ms (AUC 0.908, sensitivity 91%, specificity 74%); and for ECV 25.9% (AUC 0.918, sensitivity 86%, specificity 89%). While the diagnostic yield of the LLC was 57% (13/23) in our patient cohort, 70% (7/10) of patients missed by the LLC demonstrated abnormalities across all three global mapping parameters (native T1, T2, and ECV) and another 20% (2/10) of patients demonstrated at least one abnormal mapping value.

Conclusions: Similar to findings in adults, pediatric patients with acute myocarditis demonstrate abnormal CMR tissue mapping values compared to controls. Furthermore, we found CMR parametric mapping techniques measurably increased CMR diagnostic yield when compared with conventional LLC alone, providing additional sensitivity and specificity compared to historical references. Routine integration of these techniques into imaging protocols may aid diagnosis in children.
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http://dx.doi.org/10.1186/s12968-019-0550-7DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6631973PMC
July 2019

ACR Appropriateness Criteria Developmental Dysplasia of the Hip-Child.

J Am Coll Radiol 2019 May;16(5S):S94-S103

Specialty Chair, Riley Hospital for Children Indiana University, Indianapolis, Indiana.

Developmental dysplasia of the hip (DDH) is the most common hip pathology in infants. Although its exact pathophysiology remains incompletely understood, its long-term prognosis depends not only on the severity of the dysphasia, but also on the timely implementation of appropriate treatment. Unrecognized and untreated hip subluxations and dislocations inevitably lead to early joint degeneration while overtreatment can produce iatrogenic complications, including avascular necrosis of the femoral head. In the past two decades, imaging has become an integral part of the clinical screening, diagnosis, and monitoring of children with DDH. Optimal timing for imaging and appropriate use of imaging can reduce the incidence of late diagnoses and prevent iatrogenic complications. In general, ultrasound of the hips is recommended in infants under the age of 4 months while pelvic radiography is recommended in older infants due to the fact that the femoral head ossific nucleus typically is not formed until 4 to 6 months of age. The American College of Radiology Appropriateness Criteria are evidence-based guidelines for specific clinical conditions that are reviewed annually by a multidisciplinary expert panel. The guideline development and revision include an extensive analysis of current medical literature from peer reviewed journals and the application of well-established methodologies (RAND/UCLA Appropriateness Method and Grading of Recommendations Assessment, Development, and Evaluation or GRADE) to rate the appropriateness of imaging and treatment procedures for specific clinical scenarios. In those instances where evidence is lacking or equivocal, expert opinion may supplement the available evidence to recommend imaging or treatment.
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http://dx.doi.org/10.1016/j.jacr.2019.02.014DOI Listing
May 2019

ACR Appropriateness Criteria Suspected Appendicitis-Child.

J Am Coll Radiol 2019 May;16(5S):S252-S263

Specialty Chair, Riley Hospital for Children Indiana University, Indianapolis, Indiana.

Acute appendicitis represents the most common abdominal surgical urgency/emergency in children. Imaging remains a central tool in the diagnosis of acute appendicitis and has been shown to facilitate management and decrease the rate of negative appendectomies. The initial consideration for imaging in a child with suspected acute appendicitis is based on clinical assessment, which can be facilitated with published scoring systems. The level of clinical risk (low, intermediate, high) and the clinical scenario (suspicion for complication) define the need for imaging and the optimal imaging modality. In some situations, no imaging is required, while in others ultrasound, CT, or MRI may be appropriate. This review frames the presentation of suspected acute appendicitis in terms of the clinical risk and also discusses the unique situations of the equivocal or nondiagnostic initial ultrasound examination and suspected appendicitis with suspicion for complication (eg, bowel obstruction). The American College of Radiology Appropriateness Criteria are evidence-based guidelines for specific clinical conditions that are reviewed annually by a multidisciplinary expert panel. The guideline development and revision include an extensive analysis of current medical literature from peer reviewed journals and the application of well-established methodologies (RAND/UCLA Appropriateness Method and Grading of Recommendations Assessment, Development, and Evaluation or GRADE) to rate the appropriateness of imaging and treatment procedures for specific clinical scenarios. In those instances where evidence is lacking or equivocal, expert opinion may supplement the available evidence to recommend imaging or treatment.
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http://dx.doi.org/10.1016/j.jacr.2019.02.022DOI Listing
May 2019

Clinical decision support: practical implementation at two pediatric hospitals.

Pediatr Radiol 2019 04 29;49(4):486-492. Epub 2019 Mar 29.

Department of Radiology, Vanderbilt University Medical Center, Monroe Carell Jr. Children's Hospital at Vanderbilt, Nashville, TN, USA.

Clinical decision support has been identified by the United States government as a method to decrease inappropriate imaging exams and promote judicious use of imaging resources. The adoption of this method will be incentivized by requiring appropriate use criteria to qualify for Medicare reimbursement starting in January 2020. While Medicare reimbursement is unlikely to directly impact pediatric imaging because of largely disparate patient populations, insurance providers typically use Medicare to benchmark their reimbursement guidelines. Therefore soon after their adoption these guidelines could become relevant to pediatric imaging. In this article we discuss how pediatric imaging was initially underrepresented in the clinical decision support realm, and how this was addressed by a subcommittee involving both American College of Radiology and Society for Pediatric Radiology members. We also present the experience of implementing clinical decision support software at two standalone pediatric hospitals and summarize the lessons learned from these deployments.
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http://dx.doi.org/10.1007/s00247-018-4322-6DOI Listing
April 2019

Clinical decision support: the role of ACR Appropriateness Criteria.

Pediatr Radiol 2019 04 29;49(4):479-485. Epub 2019 Mar 29.

Department of Radiology and Imaging Sciences, Riley Hospital for Children, Indiana University School of Medicine, Indianapolis, IN, USA.

Clinical decision support is a way to decrease inappropriate imaging exams and promote judicious use of imaging resources. The adoption of clinical decision support will be incentivized by requiring the use of approved mechanisms to qualify for Medicare reimbursement starting in January 2020. Insurance providers base their reimbursement policies on Medicare, so clinical decision support could soon become relevant to pediatric imaging. We present the process behind the American College of Radiology (ACR) Appropriateness Criteria (a set of appropriate use criteria developed by the ACR) that will form the basis for software that can be used to fulfill the criteria for clinical decision support. For most organizations, this software is expected to be the easiest way to implement clinical decision support. Clinical decision support will affect how providers order imaging exams. This article should help readers understand how clinical decision support is expected to change the practice of the ordering providers, how the ACR Appropriateness Criteria are related to clinical decision support and how the ACR Appropriateness Criteria are developed. This will help the interpreting radiologist better communicate with the referring clinician, including informing the latter about how the clinical decision support software is making decisions.
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http://dx.doi.org/10.1007/s00247-018-4298-2DOI Listing
April 2019

Adherence to the 2011 American Academy of Pediatrics Urinary Tract Infection Guidelines for Voiding Cystourethrogram Ordering by Clinician Specialty.

Urology 2019 04 6;126:180-186. Epub 2019 Feb 6.

Department of Urology, Northwestern University, Chicago, IL; Division of Pediatric Urology, Ann & Robert H. Lurie Children's Hospital of Chicago, Chicago, IL. Electronic address:

Objective: To evaluate rates of guideline adherence and associations with voiding cystourethrogram result. The American Academy of Pediatrics guidelines recommend voiding cystourethrogram after abnormal renal ultrasound or 2 febrile urinary tract infections. It is unclear whether guideline adherence increases vesicoureteral reflux detection. Additionally, guidelines targeting children 2-24 months are often applied to other ages.

Methods: Children undergoing voiding cystourethrogram from January 2012 to December 2013 at 1 institution were retrospectively reviewed. Children with known genitourinary abnormalities were excluded. The primary outcome was guideline adherence. Univariate and multivariate analyses were performed. Subgroup analysis of children 2-24 months was completed.

Results: Voiding cystourethrograms from 365 children were included in the primary analysis, including 187 (51.2%) aged 2-24 months. Overall, 60.3% of voiding cystourethrograms were ordered in accordance with the guidelines. Urologists/nephrologists were more likely to adhere to ordering guidelines than pediatricians/others (76.4% vs 51.7%, odds ratio 3.0 [1.9-4.9], P <.001). Subgroup analysis in children 2-24 months revealed similar findings (76.4% vs 51.5%, odds ratio 3.0 [1.5-6.2], P = .002). Voiding cystourethrograms were abnormal in 31.8% overall and 26.2% aged 2-24 months. Guideline adherence was associated with increased likelihood of abnormal voiding cystourethrogram among all children (P = .02), but not among children 2-24 months (P = .95). Older age, white race, and guideline adherence remained significantly associated with abnormal voiding cystourethrogram in a multiple logistic regression model.

Conclusions: Guideline adherence was more likely among urologists/nephrologists than pediatricians/others and was not associated with abnormal voiding cystourethrogram among children 2-24 months. Multicenter evaluation is necessary to determine if ordering recommendations should be revised.
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http://dx.doi.org/10.1016/j.urology.2018.12.044DOI Listing
April 2019

Impact of age and cardiac disease on regional left and right ventricular myocardial motion in healthy controls and patients with repaired tetralogy of fallot.

Int J Cardiovasc Imaging 2019 Jun 4;35(6):1119-1132. Epub 2019 Feb 4.

Department of Radiology, Feinberg School of Medicine, Northwestern University, 737 N. Michigan Avenue, Suite 1600, Chicago, IL, 60611, USA.

The assessment of both left (LV) and right ventricular (RV) motion is important to understand the impact of heart disease on cardiac function. The MRI technique of tissue phase mapping (TPM) allows for the quantification of regional biventricular three-directional myocardial velocities. The goal of this study was to establish normal LV and RV velocity parameters across a wide range of pediatric to adult ages and to investigate the feasibility of TPM for detecting impaired regional biventricular function in patients with repaired tetralogy of Fallot (TOF). Thirty-six healthy controls (age = 1-75 years) and 12 TOF patients (age = 5-23 years) underwent cardiac MRI including TPM in short-axis locations (base, mid, apex). For ten adults, a second TPM scan was used to assess test-retest reproducibility. Data analysis included the calculation of biventricular radial, circumferential, and long-axis velocity components, quantification of systolic and diastolic peak velocities in an extended 16 + 10 LV + RV segment model, and assessment of inter-ventricular dyssynchrony. Biventricular velocities showed good test-retest reproducibility (mean bias ≤ 0.23 cm/s). Diastolic radial and long-axis peak velocities for LV and RV were significantly reduced in adults compared to children (19-61%, p < 0.001-0.02). In TOF patients, TPM identified significantly reduced systolic and diastolic LV and RV long-axis peak velocities (20-50%, p < 0.001-0.05) compared to age-matched controls. In conclusion, tissue phase mapping enables comprehensive analysis of global and regional biventricular myocardial motion. Changes in myocardial velocities associated with age underline the importance of age-matched controls. This pilot study in TOF patients shows the feasibility to detect regionally abnormal LV and RV motion.
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http://dx.doi.org/10.1007/s10554-019-01544-6DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6535126PMC
June 2019
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