Intervention for critical aortic stenosis in Hutchinson-Gilford progeria syndrome.

Hutchinson-Gilford Progeria Syndrome (HGPS) is an ultra-rare genetic premature aging disease that is historically fatal in teenage years, secondary to severe accelerated atherosclerosis. The only approved treatment is the farnesyltransferase inhibitor lonafarnib, which improves vascular structure and function, extending average untreated lifespan of 14.5 years by 4.3 years (30%). With this longer lifespan, calcific aortic stenosis (AS) was identified as an emerging critical risk factor for cardiac death in older patients. Intervention to relieve critical AS has the potential for immediate improvement in healthspan and lifespan. However, HGPS patient-device size mismatch, pervasive peripheral arterial disease, skin and bone abnormalities, and lifelong failure to thrive present unique challenges to intervention. An international group of experts in HGPS, pediatric and adult cardiology, cardiac surgery, and pediatric critical care convened to identify strategies for successful treatment. Candidate procedures were evaluated by in-depth examination of 4 cases that typify HGPS clinical pathology. Modified transcatheter aortic valve replacement (TAVR) and left ventricular Apico-Aortic Conduit (AAC) placement were deemed high risk but viable options. Two cases received TAVR and 2 received AAC post-summit. Three were successful and 1 patient died perioperatively due to cardiovascular disease severity, highlighting the importance of intervention timing and comparative risk stratification. These breakthrough interventions for treating critical aortic stenosis in HGPS patients could rewrite the current clinical perspective on disease course by greatly improving late-stage quality of life and increasing lifespan. Expanding worldwide medical and surgical competency for this ultra-rare disease through expert information-sharing could have high impact on treatment success.

Clinical situations for which 3D printing is considered an appropriate representation or extension of data contained in a medical imaging examination: neurosurgical and otolaryngologic conditions.

BACKGROUND: Medical three dimensional (3D) printing is performed for neurosurgical and otolaryngologic conditions, but without evidence-based guidance on clinical appropriateness. A writing group composed of the Radiological Society of North America (RSNA) Special Interest Group on 3D Printing (SIG) provides appropriateness recommendations for neurologic 3D printing conditions. METHODS: A structured literature search was conducted to identify all relevant articles using 3D printing technology associated with neurologic and otolaryngologic conditions. Each study was vetted by the authors and strength of evidence was assessed according to published guidelines. RESULTS: Evidence-based recommendations for when 3D printing is appropriate are provided for diseases of the calvaria and skull base, brain tumors and cerebrovascular disease. Recommendations are provided in accordance with strength of evidence of publications corresponding to each neurologic condition combined with expert opinion from members of the 3D printing SIG. CONCLUSIONS: This consensus guidance document, created by the members of the 3D printing SIG, provides a reference for clinical standards of 3D printing for neurologic conditions.

Clinical situations for which 3D Printing is considered an appropriate representation or extension of data contained in a medical imaging examination: vascular conditions.

BACKGROUND: Medical three-dimensional (3D) printing has demonstrated utility and value in anatomic models for vascular conditions. A writing group composed of the Radiological Society of North America (RSNA) Special Interest Group on 3D Printing (3DPSIG) provides appropriateness recommendations for vascular 3D printing indications. METHODS: A structured literature search was conducted to identify all relevant articles using 3D printing technology associated with vascular indications. Each study was vetted by the authors and strength of evidence was assessed according to published appropriateness ratings. RESULTS: Evidence-based recommendations for when 3D printing is appropriate are provided for the following areas: aneurysm, dissection, extremity vascular disease, other arterial diseases, acute venous thromboembolic disease, venous disorders, lymphedema, congenital vascular malformations, vascular trauma, vascular tumors, visceral vasculature for surgical planning, dialysis access, vascular research/development and modeling, and other vasculopathy. Recommendations are provided in accordance with strength of evidence of publications corresponding to each vascular condition combined with expert opinion from members of the 3DPSIG. CONCLUSION: This consensus appropriateness ratings document, created by the members of the 3DPSIG, provides an updated reference for clinical standards of 3D printing for the care of patients with vascular conditions.

Generalization optimizing machine learning to improve CT scan radiomics and assess immune checkpoint inhibitors' response in non-small cell lung cancer: a multicenter cohort study.

Background: Recent developments in artificial intelligence suggest that radiomics may represent a promising non-invasive biomarker to predict response to immune checkpoint inhibitors (ICIs). Nevertheless, validation of radiomics algorithms in independent cohorts remains a challenge due to variations in image acquisition and reconstruction. Using radiomics, we investigated the importance of scan normalization as part of a broader machine learning framework to enable model external generalizability to predict ICI response in non-small cell lung cancer (NSCLC) patients across different centers. Methods: Radiomics features were extracted and compared from 642 advanced NSCLC patients on pre-ICI scans using established open-source PyRadiomics and a proprietary DeepRadiomics deep learning technology. The population was separated into two groups: a discovery cohort of 512 NSCLC patients from three academic centers and a validation cohort that included 130 NSCLC patients from a fourth center. We harmonized images to account for variations in reconstruction kernel, slice thicknesses, and device manufacturers. Multivariable models, evaluated using cross-validation, were used to estimate the predictive value of clinical variables, PD-L1 expression, and PyRadiomics or DeepRadiomics for progression-free survival at 6 months (PFS-6). Results: The best prognostic factor for PFS-6, excluding radiomics features, was obtained with the combination of Clinical + PD-L1 expression (AUC = 0.66 in the discovery and 0.62 in the validation cohort). Without image harmonization, combining Clinical + PyRadiomics or DeepRadiomics delivered an AUC = 0.69 and 0.69, respectively, in the discovery cohort, but dropped to 0.57 and 0.52, in the validation cohort. This lack of generalizability was consistent with observations in principal component analysis clustered by CT scan parameters. Subsequently, image harmonization eliminated these clusters. The combination of Clinical + DeepRadiomics reached an AUC = 0.67 and 0.63 in the discovery and validation cohort, respectively. Conversely, the combination of Clinical + PyRadiomics failed generalizability validations, with AUC = 0.66 and 0.59. Conclusion: We demonstrated that a risk prediction model combining Clinical + DeepRadiomics was generalizable following CT scan harmonization and machine learning generalization methods. These results had similar performances to routine oncology practice using Clinical + PD-L1. This study supports the strong potential of radiomics as a future non-invasive strategy to predict ICI response in advanced NSCLC.

3D printing exposure and perception in radiology residency: survey results of radiology chief residents.

RATIONALE AND OBJECTIVES: The purpose of this study is to summarize a survey of radiology chief residents focused on 3D printing in radiology. MATERIALS AND METHODS: An online survey was distributed to chief residents in North American radiology residencies by subgroups of the Association of University Radiologists. The survey included a subset of questions focused on the clinical use of 3D printing and perceptions of the role of 3D printing and radiology. Respondents were asked to define the role of 3D printing at their institution and asked about the potential role of clinical 3D printing in radiology and radiology residencies. RESULTS: 152 individual responses from 90 programs were provided, with a 46% overall program response rate (n = 90/194 radiology residencies). Most programs had 3D printing at their institution (60%; n = 54/90 programs). Among the institutions that perform 3D printing, 33% (n = 18/54) have structured opportunities for resident contribution. Most residents (60%; n = 91/152 respondents) feel they would benefit from 3D printing exposure or educational material. 56% of residents (n = 84/151) believed clinical 3D printing should be centered in radiology departments. 22% of residents (n = 34/151) believed it would increase communication and improve relationships between radiology and surgery colleagues. A minority (5%; 7/151) believe 3D printing is too costly, time-consuming, or outside a radiologist's scope of practice. CONCLUSIONS: A majority of surveyed chief residents in accredited radiology residencies believe they would benefit from exposure to 3D printing in residency. 3D printing education and integration would be a valuable addition to current radiology residency program curricula.

Radiological Society of North America (RSNA) 3D Printing Special Interest Group (SIG) clinical situations for which 3D printing is considered an appropriate representation or extension of data contained in a medical imaging examination: breast conditions.

The use of medical 3D printing has expanded dramatically for breast diseases. A writing group composed of the Radiological Society of North America (RSNA) Special Interest Group on 3D Printing (SIG) provides updated appropriateness criteria for breast 3D printing in various clinical scenarios. Evidence-based appropriateness criteria are provided for the following clinical scenarios: benign breast lesions and high-risk breast lesions, breast cancer, breast reconstruction, and breast radiation (treatment planning and radiation delivery).

Recover Wisely From COVID-19: Responsible Resumption of Nonurgent Radiology Services.

RATIONALE AND OBJECTIVES: Following state and institutional guidelines, our Radiology department launched the "Recover Wisely" for all nonurgent radiology care on May 4, 2020. Our objective is to report our practice implementation and experience of COVID-19 recovery during the resumption of routine imaging at a tertiary academic medical center. MATERIALS AND METHODS: We used the SQUIRE 2.0 guidelines for this practice implementation. Recover Wisely focused on a data driven, strategic rescheduling and redesigning patient flow process. We used scheduling simulations and meticulous monitoring and control of outpatient medical imaging volumes to achieve a linear restoration to our pre-COVID imaging studies. We had a tiered plan to address the backlog of rescheduled patients with gradual opening of our imaging facilities, while maintaining broad communication with our patients and referring clinicians. RESULTS: Recover Wisely followed our anticipated linear modeling. Considering the last 10 weeks in the recovery, outpatient growth was linear with an increase of approximately 172 cases per week, (R2 =0.97). We achieved an overall recovery of 102% in week 10, as compared to average weekly pre-COVID outpatient volumes. The modalities recovered as follows in outpatient volumes: CT (113%), MRI (101%), nuclear medicine including PET (138%), mammograms (97%), ultrasound (99%) and interventional radiology (106%). When compared to identical 2019 calendar weeks (May 4, 2020-July 10, 2020), the total 2020 radiology volume was 11% reduced from the 2019 volume. The reduction in total weighted relative value units was 8% in this time period, as compared to 2019. CONCLUSION: Our department utilized a data-driven, team approach based on our guiding principles to "Recover Wisely." We created and implemented a methodology that achieved a linear increase in outpatient studies over a 10-week recovery period.

Clinical situations for which 3D printing is considered an appropriate representation or extension of data contained in a medical imaging examination: adult cardiac conditions.

BACKGROUND: Medical 3D printing as a component of care for adults with cardiovascular diseases has expanded dramatically. A writing group composed of the Radiological Society of North America (RSNA) Special Interest Group on 3D Printing (SIG) provides appropriateness criteria for adult cardiac 3D printing indications. METHODS: A structured literature search was conducted to identify all relevant articles using 3D printing technology associated with a number of adult cardiac indications, physiologic, and pathologic processes. Each study was vetted by the authors and graded according to published guidelines. RESULTS: Evidence-based appropriateness guidelines are provided for the following areas in adult cardiac care; cardiac fundamentals, perioperative and intraoperative care, coronary disease and ischemic heart disease, complications of myocardial infarction, valve disease, cardiac arrhythmias, cardiac neoplasm, cardiac transplant and mechanical circulatory support, heart failure, preventative cardiology, cardiac and pericardial disease and cardiac trauma. CONCLUSIONS: Adoption of common clinical standards regarding appropriate use, information and material management, and quality control are needed to ensure the greatest possible clinical benefit from 3D printing. This consensus guideline document, created by the members of the RSNA 3D printing Special Interest Group, will provide a reference for clinical standards of 3D printing for adult cardiac indications.

Radiological Society of North America (RSNA) 3D Printing Special Interest Group (SIG) clinical situations for which 3D printing is considered an appropriate representation or extension of data contained in a medical imaging examination: abdominal, hepatobiliary, and gastrointestinal conditions.

BACKGROUND: Medical 3D printing has demonstrated value in anatomic models for abdominal, hepatobiliary, and gastrointestinal conditions. A writing group composed of the Radiological Society of North America (RSNA) Special Interest Group on 3D Printing (SIG) provides appropriateness criteria for abdominal, hepatobiliary, and gastrointestinal 3D printing indications. METHODS: A literature search was conducted to identify all relevant articles using 3D printing technology associated with a number of abdominal pathologic processes. Each included study was graded according to published guidelines. RESULTS: Evidence-based appropriateness guidelines are provided for the following areas: intra-hepatic masses, hilar cholangiocarcinoma, biliary stenosis, biliary stones, gallbladder pathology, pancreatic cancer, pancreatitis, splenic disease, gastric pathology, small bowel pathology, colorectal cancer, perianal fistula, visceral trauma, hernia, abdominal sarcoma, abdominal wall masses, and intra-abdominal fluid collections. CONCLUSION: This document provides initial appropriate use criteria for medical 3D printing in abdominal, hepatobiliary, and gastrointestinal conditions.

Medical 3D Printing Cost-Savings in Orthopedic and Maxillofacial Surgery: Cost Analysis of Operating Room Time Saved with 3D Printed Anatomic Models and Surgical Guides.

RATIONALE AND OBJECTIVE: Three-dimensional (3D) printed anatomic models and surgical guides have been shown to reduce operative time. The purpose of this study was to generate an economic analysis of the cost-saving potential of 3D printed anatomic models and surgical guides in orthopedic and maxillofacial surgical applications. MATERIALS AND METHODS: A targeted literature search identified operating room cost-per-minute and studies that quantified time saved using 3D printed constructs. Studies that reported operative time differences due to 3D printed anatomic models or surgical guides were reviewed and cataloged. A mean of $62 per operating room minute (range of $22-$133 per minute) was used as the reference standard for operating room time cost. Different financial scenarios were modeled with the provided cost-per-minute of operating room time (using high, mean, and low values) and mean time saved using 3D printed constructs. RESULTS: Seven studies using 3D printed anatomic models in surgical care demonstrated a mean 62 minutes ($3720/case saved from reduced time) of time saved, and 25 studies of 3D printed surgical guides demonstrated a mean 23 minutes time saved ($1488/case saved from reduced time). An estimated 63 models or guides per year (or 1.2/week) were predicted to be the minimum number to breakeven and account for annual fixed costs. CONCLUSION: Based on the literature-based financial analyses, medical 3D printing appears to reduce operating room costs secondary to shortening procedure times. While resource-intensive, 3D printed constructs used in patients' operative care provides considerable downstream value to health systems.

Reference values for mid-diastolic right ventricular volume in population referred for cardiac computed tomography: An additional diagnostic value to cardiac computed tomography.

BACKGROUND: While an assessment of the right ventricular (RV) size remains challenging, the entire RV is can be imaged on coronary computed tomography angiography (CCTA) studies. With prospective ECG-triggering, the RV end diastolic volume (RVEDV) cannot be measured; however, the RV mid-diastolic volume (RVMDV) can still be measured accurately from routine CCTA data sets. The objective of this study is to establish normal reference values for RVMDV. METHODS: Right ventricular mid-diastolic volumes were measured in 4855 consecutive patients undergoing prospectively ECG-triggered coronary CTA. All patients with known cardiac or pulmonary disease (coronary artery disease, myocardial infarction, revascularization, heart failure, pulmonary hypertension, congenital heart disease, valvular heart disease, atrial fibrillation, implantable cardiac defibrillator implantation, cardiac transplant, or cardiac surgery) or smoking history (3313 patients) were excluded. RESULTS: 1542 patients were analyzed (mean age 56.4 ± 11.1 years, mean BSA 1.96 ± 0.26 and 47% male). The mean RVMDV for men and women was 168.6 ± 37.6 mL and 117.6 ± 26.4 mL, respectively. Mean BSA-indexed RVMDV was 80.0 ± 15.3 mL/m2 and 64.1 ± 12.2 mL/m2 for men and women, respectively. The presence of hypertension and diabetes did not have an impact on these values. RVMDV and BSA-indexed RVMDV were lower in women and in older individuals. CONCLUSION: Normal reference ranges for RVMDV were established using prospectively ECG-triggered coronary CTA studies. This data can be used to identify patients with abnormal RV volumes and potentially RV dysfunction, adding incremental diagnostic value to routine CCTA studies.

Point of Care Clinical Risk Score to Improve the Negative Diagnostic Utility of an Agatston Score of Zero: Averting the Need for Coronary Computed Tomography Angiography.

BACKGROUND: Coronary artery calcification is a marker of underlying atherosclerotic vascular disease. The absence of coronary artery calcification is associated with a low prevalence of obstructive coronary artery disease (CAD), but it cannot be ruled out completely. We sought to develop a clinical tool that can be added to Agatston score of zero to rule out obstructive CAD with high accuracy. METHODS: We developed a clinical score retrospectively from a cohort of 4903 consecutive patients with an Agatston score of zero. Patients with prior diagnosis of CAD, coronary percutaneous coronary intervention, or surgical revascularization were excluded. Obstructive CAD was defined as any epicardial vessel diameter narrowing of ≥50%. The score was validated using an external cohort of 4290 patients with an Agatston score of zero from a multinational registry. RESULTS: The score consisted of 7 variables: age, sex, typical chest pain, dyslipidemia, hypertension, family history, and diabetes mellitus. The model was robust with an area under the curve of 0.70 (95% CI, 0.65-0.76) in the derivation cohort and 0.69 (95% CI, 0.65-0.72) in the validation cohort. Patients were divided into 3 risk groups based on the score: low (≤6), intermediate (7-13), and high (≥14). Patients who score ≤6 have a negative likelihood ratio of 0.42 for obstructive CAD, whereas those who score ≥14 have a positive likelihood ratio of >5.5 for obstructive CAD. The outcome was ruled out in >98% of patients with a score ≤6 in the validation cohort. CONCLUSIONS: We developed a score that may be used to identify the likelihood of obstructive CAD in patients with an Agatston score of zero, which may be used to direct the need for additional testing. However, the results of this retrospective analysis are hypothesis generating and before clinical implementation should be validated in a trial with a prospectively collected data.

Changes in tissue gadolinium biodistribution measured in an animal model exposed to four chelating agents.

PURPOSE: This study investigated the potential to reduce gadolinium levels in rodents after repetitive IV Gadodiamide administration using several chelating agents. MATERIALS AND METHODS: The following six groups of rats were studied. Group 1: Control; Group 2: Gadodiamide only; Group 3: Meso-2,3-Dimercaptosuccinic acid (DMSA) + Gadodiamide; Group 4: N-Acetyl-L-cysteine (NAC) + Gadodiamide; Group 5: Coriandrum sativum extract + Gadodiamide; and Group 6: Deferoxamine + Gadodiamide. Brain, kidney, and blood samples were evaluated via inductively coupled plasma mass spectrometry. The brain was also evaluated histologically. RESULTS: Kidney gadolinium levels in Groups 4 and 5 were approximately double that of Group 2 (p = 0.033 for each). There was almost no calcification in rat hippocampus for Group 4 rodents when compared with Groups 2, 3, 5 and 6. CONCLUSION: Our preliminary study shows that excretion to the kidney has a higher propensity in NAC and Coriandrum sativum groups. It may be possible to change the distribution of gadolinium by administrating several agents. NAC may lower Gadodiamide-induced mineralization in rat hippocampus.

ACR Appropriateness Criteria® Imaging for Transcatheter Aortic Valve Replacement.

Aortic stenosis is a common valvular condition with increasing prevalence in aging populations. When severe and symptomatic, the downstream prognosis is poor without surgical or transcatheter aortic valve replacement. Transcatheter aortic valve replacement is now considered a viable alternative to surgical aortic valve replacement in patients considered high and intermediate risk for surgery. Pre-intervention imaging with echocardiography and CT are essential for procedure planning and device selection to help optimize clinical outcomes with MR angiography playing largely a complementary role. Modern 3-D cross-sectional imaging has consistently shown to help reduce procedural complications from vascular access injury to paravalvular regurgitation and coronary 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.