UT Health Austin

Abstract Purpose This study characterizes the current landscape of uveitis specialists and their practice settings in the United States. Methods An anonymous Internet-based survey with questions pertaining to training history and practice characteristics was distributed via REDCap to the American Uveitis Society and Young Uveitis Specialists listservs. Results Forty-eight uveitis specialists in the United States responded to the survey out of 174 uveitis specialists that identify as practicing in the United States. Twenty-five of 48 respondents (52%) completed an additional fellowship. These additional fellowships ranged from surgical retina (12/25, 48%), cornea (8/25, 32%), and medical retina (4/25, 16%). Two-thirds of uveitis specialists managed their own immunosuppression, while one-third comanaged immunosuppression with rheumatologists. Thirty-three of 48 (69%) maintained a surgical practice. Conclusion This is the first survey of uveitis specialists across the United States to provide understanding into training and practice characteristics. These data will provide insight into career planning, practice building, and assist in resource allocation.

COVID White Paper Series

Abstract This education session will review early palliative care in oncology and applications in patients with metastatic breast cancer. This presentation provides an overview of the field of research in early palliative care for patients with advanced solid tumors and discusses considerations specific to breast cancer. The speaker will present current challenges that we face in integrating palliative care into our practice and future areas for clinical innovation and research in palliative care. The speaker will present practical tips for how breast oncologists can deliver early palliative care to patients. The speaker will present guiding principles on when to consider referral to interdisciplinary, specialty palliative care. Citation Format: Tara Kaufmann. Early palliative care [abstract]. In: Proceedings of the 2022 San Antonio Breast Cancer Symposium; 2022 Dec 6-10; San Antonio, TX. Philadelphia (PA): AACR; Cancer Res 2023;83(5 Suppl):Abstract nr ED5-1.

PURPOSE: Pre-transplant (PreTX) diagnoses of congenital heart disease (CHD), including single ventricle (SV) CHD, are known to be associated with immediate post-operative morbidity and mortality. However, the impact on post-discharge health and morbidity has not been elucidated. METHODS: The Pediatric Health Information Survey (PHIS) data was used to identify patients undergoing orthotopic heart transplantation (HT). We assessed hospital encounters for readmission, ICU care, and interventions within 1 year of heart transplantation after discharge from HT. RESULTS: A total of 4087 patients were included in the analysis with the median age of 5.2 years. PreTX diagnosis was CHD in 28%, single ventricle CHD (SV) in 31%, cardiomyopathy, and other causes in 41%. A total of 2698 patients (66%) required hospital readmission within 1 year of discharge, of which 569 required more than two readmissions. The reason for readmission was cardiac in 22%, infectious in 35%, and non-cardiac in 43%. Using multivariable modeling, younger age, CHD, SV, Hispanic race, government insurance, longer post-TX hospital stay, longer ventilation needs, and dialysis use were associated with readmission risk (all p < 0.05). CHD and SV diagnosis, younger age, and longer post-TX stay were also risk factors for ICU-level readmission (all p < 0.05). Regression analysis showed that CHD (HR 2.7) and SV (HR 5.3) were highly predictive of reinterventions within 1 year. Lastly, the morbidity burden was calculated as days alive and outside hospital (DAOH) post TX. Younger age, SV, current era for transplantation, prolonged ventilation, and hospital stay post TX were all associated with lower DAOH. CONCLUSION: CHD and SV have a significant impact on continued morbidity post-TX, including the need for ICU-level readmission and reinterventions. The study also identifies race and post-TX morbidities as other important risk factors for readmissions and reinterventions. We need to study and improve the optimization of patients pre-and post-TX to mitigate this significant and continued risk.

Abstract Background: Patients treated for early stage breast cancer (BC) have a 30% lifetime risk of developing metastatic disease. Numerous studies have demonstrated that dormant bone marrow disseminated tumor cells (DTCs) are independently associated with risk of recurrence and death, yet interventions targeting these cells are lacking. The PENN-SURMOUNT (Surveillance Markers of Utility for Recurrence after (Neo)adjuvant Therapy) Screening Study was launched in 2016 to screen high risk BC survivors for DTCs using bone marrow aspirate (BMA) and identify eligible DTC positive patients for clinical trials. Given the novelty of this approach, we concurrently developed and pilot tested a PRO measurement strategy to study how the screening method of BMA and disclosure of DTC results impacts early-stage BC patients. Methods: PENN-SURMOUNT is a single center prospective, longitudinal cohort study examining BM and blood biomarkers of MRD among patients within 5 years of BC diagnosis who have high risk criteria (positive axillary nodes, triple negative biology, ER+ with Oncotype Dx ≥ 25 and/or high risk Mammaprint, or pathologic residual disease after neoadjuvant chemotherapy). From May 2019 – August 2021, we recruited patients on SURMOUNT to complete PRO surveys at baseline (T0), after BMA (T1), and after disclosure of DTC results (T2). Surveys were administered in paper form initially, then electronic form starting Feb 2021. PRO survey instruments were selected through literature review, followed by consensus among multidisciplinary clinical and research experts and patient advocates. PRO measures assess recurrence distress (Quality of Life in Adult Cancer Survivors, QLACS), illness intrusiveness (Illness Intrusiveness Ratings Scale, IIRS), and decision making (Decision Regret Scale). Additional survey items assess tolerability of the BMA and patients’ risk perception and cognitive understanding after DTC results disclosure. Descriptive statistics summarize PRO survey compliance and responses at T0, T1, and T2 in the total population and the population who reported longitudinal data for T2. Results: 61 of 66 eligible patients on the SURMOUNT trial enrolled in the PRO pilot study and completed a baseline survey, of which 47 (77%) tested negative for DTCs. Mean completion rates were 0.92 at T0, 0.85 at T1, and 0.56 at T2. After electronic survey implementation, completion rates increased to 0.94 (T0), 0.97 (T1) and 0.81 (T2). At T0, 36 (59%) patients reported a high risk perception of developing BC recurrence at 5 years and 42 (69%) during their lifetime. Mean T0 recurrence distress using the QLACS subscale was 14.6 (SD 6.3) out of possible score 4-28, compared to an expected mean of 11.42 (SD 5.48) in a general survivorship population. Mean T0 illness intrusiveness was 27.3 (SD 13.9) out of possible score 13-91. At T1, approximately 85% of patients agreed that they correctly understood the purpose of the bone marrow procedure and what the procedure would entail. 44 (72%) of patients reported a maximum pain score &amp;lt;= 4 in the week post-procedure and 42 (69%) reported the BMA was same or better than expected tolerability. Exploratory subset analysis of patients with complete longitudinal data at T2 (n = 34) showed average scores of 13.4 (SD 6.0), 30.1 (SD 14.0), and 2.8 (SD 6.2) for recurrence distress, illness intrusive, and decision regret scores (scale 0-100), respectively. At T2, 26 (76%) of patients reported no decision regret for undergoing testing for DTCs; 27 (79%) reported feeling less anxious after DTC results disclosure. Conclusions: Participants of PENN-SURMOUNT perceived risk of recurrence as high. The BMA procedure was well-tolerated and better than expected among the majority of this cohort, and most did not regret having undergone BMA after DTC status disclosure. Longitudinal completion rates were low, limiting assessment of PROs at later time points, a major focus of future work in this setting. Citation Format: Tara Kaufmann, Patrick Chang, Shoshana Rosenberg, Elizabeth Frank, Brian Hobbs, Lauren J. Bayne, Isoris Nivar, Brooke L. Goodspeed, Killian M. Rohn, Emily M. Kugler, Kevin Fox, Susan Domchek, Angela Bradbury, Payal Shah, Hayley Knollman, Rachel C. Jankowitz, Igor Makhlin, Amy S. Clark, Lewis A. Chodosh, Angela DeMichele, Katherine Goodfellow. Pilot study of a patient-reported outcome (PRO) measurement strategy to determine impact of screening for minimal residual disease (MRD) in high-risk breast cancer survivors [abstract]. In: Proceedings of the 2022 San Antonio Breast Cancer Symposium; 2022 Dec 6-10; San Antonio, TX. Philadelphia (PA): AACR; Cancer Res 2023;83(5 Suppl):Abstract nr P5-08-01.

PURPOSE: The detrimental effects of endogenous endophthalmitis on visual acuity have been well studied; however, other complications of the central nervous system have not been robustly investigated. Our objective was to investigate central nervous system involvement associated with endogenous endophthalmitis. METHODS: We analyzed hospitalized patients with endogenous endophthalmitis in two hospitals from January 2010 to November 2020. We documented patient characteristics and outcomes related to endogenous endophthalmitis, including relevant neurologic complications. A neuroradiologist reviewed any available central nervous system imaging. RESULTS: A total of 62 patients were included (42 [68%] males and 20 [32%] female). Of those included, 14 patients (23%) had at least one neurologic complication associated with the central nervous system. Of those 14 patients, 8 (57%) had ischemic/embolic brain infarction(s), 7 (50%) had encephalopathy, 4 (29%) had cerebral hemorrhage(s), 2 (14%) had meningitis, 1 (7%) had a brain abscess, and 1 (7%) had an anoxic brain injury. Of the patients who had central nervous system complications, a majority of eight patients (57%) had positive blood cultures, two (14%) had positive intraocular cultures, while two (14%) were positive in both. There was no statistically significant difference in demographics, comorbidities, source of infection, or organism between patients with and without central nervous system complications. CONCLUSION: The incidence of comorbid central nervous system disease in our cohort was higher than previously reported in the literature. Although we identified no specific risk factors for the development of central nervous system disease in endogenous endophthalmitis, we found that cases with more severe central nervous system complications were associated with culture positivity.

INTRODUCTION: Congenital heart disease (CHD) patients experience risks for renal failure, including low cardiac output, exposure to nephrotoxic agents, and surgical interventions. Outcomes of kidney transplantation in CHD patients remain underexplored. METHODS: A retrospective review of the Pediatric Health Information System database from 1/1/04-10/30/23. All patients < 18 years who underwent kidney transplantation were included. Multi-organ transplants were excluded. International Classification of Diseases 9th and 10th editions were utilized to identify patients with a diagnosis consistent with CHD. RESULTS: A total of 7586 patients were identified, with 3109 (41%) female, 3833 (51%) white non-Hispanic, and a median age of 13 [IQR:7-16] years at transplant. A CHD diagnosis was present in 181 (2%). CHD patients were more likely to have mechanical ventilation > 96 h (n = 54 (30%) vs. n = 1264 (17%), p < 0.001) and red blood cell transfusion (n = 48 (27%) vs. n = 1452 (20%, p = 0.026)). On multivariable analysis, CHD was associated with a 9% (95% CI: 2.5-17.1) increase in length of stay (p = 0.007) and was not associated with an increased risk for 30-day readmission (OR: 0.88; CI: 0.62-1.26, p = 0.509). Over a median follow-up of 2.6 [IRQ: 0.7-5.7] years, no difference in re-transplant free survival was evident. CONCLUSIONS: CHD patients have increased inhospital resource utilization compared to non-CHD, but no difference in long-term outcomes. However, differences in willingness to transplant cannot be assessed with this data. Further work is needed to better understand the impact of CHD on management decisions and outcomes across the kidney disease continuum.

In the setting of a normal first-trimester ultrasound, an amniocentesis may be a better option than chorionic villous sampling for invasive diagnostic testing after a cell-free DNA high risk for trisomy 13, given the high rates of confined placental mosaicism. In unaffected fetuses, other evaluations should be considered depending on the cell-free DNA results, including maternal karyotyping for monosomy X, uniparental disomy testing for chromosomes with imprinted genes, serial growth scans for trisomy 16, and a workup for maternal malignancy for multiple aneuploidies or autosomal monosomy.

BACKGROUND: Paediatric cardiac electrophysiologists are essential in CHD inpatient care, but their involvement is typically limited to consultation with individual patients. In our integrated heart centre, an electrophysiologist reviews all cardiac inpatient telemetry over the preceding 24 hours and participates in daily multidisciplinary morning report. This study investigates the impact of the strategy of consistent, formalised electrophysiologist presence at multidisciplinary morning report. METHODS: This is a single-centre, prospective, observational study of electrophysiologist participation in patient encounters during heart centre multidisciplinary morning report from 10/20/2021 to 10/31/2022. Multidisciplinary morning report includes discussion of all intensive care and non-intensive care cardiac patients. An encounter was defined as reporting on one patient for one day. Electrophysiologists were initially blinded to observations. RESULTS: Two electrophysiologists were observed over 215 days encompassing 6413 patient encounters. Electrophysiologists made comments on 581(9.1%) encounters in 234 unique patients with diverse diagnoses, equating to a median of 3[interquartile range:1-4] encounters per day. These included identifications of arrhythmias and describing electrocardiographic findings. Recommendation to change management occurred in 282(48.5%) encounters, most commonly regarding medications (n = 142, 24.4%) or pacemaker management (n = 48, 8.3%). Of the 581 encounters, there were 61(10.5%) in which they corrected another physician's interpretation of rhythm or electrocardiogram. CONCLUSION: Routine electrophysiologist involvement in multidisciplinary morning report provides significant, frequent, and timely input in patient management by identifying precise rhythm-related diagnoses and allowing nuanced, patient-specific medication and pacemaker management of all cardiac patients, not just those consulted. Electrophysiologist presence at multidisciplinary morning report is a vital resource and this practice should be considered at integrated paediatric cardiac centres.

Abstract Background: Women undergoing treatment for breast cancer experience both disease- and treatment-related symptoms. Remote symptom management programs allow real time symptom documentation, earlier intervention, and opportunities to improve quality of life and decrease symptom burden. This study describes patient-reported outcomes (PROs) in women undergoing treatment for early stage and metastatic breast cancer. Methods: Women with breast cancer using Carevive’s remote symptom management (RSM) program completed weekly surveys to assess the presence of 14 common symptoms over 16 weeks. Symptoms assessed were anxiety, decreased appetite, fatigue, general pain, mouth sores, muscle pain, nausea, vomiting, numbness, sadness, shortness of breath, diarrhea, constipation, and insomnia. When a symptom was reported, additional questions were asked regarding symptom severity, frequency, and interference using the National Cancer Society’s Patient Reported Outcomes-Common Terminology Criteria for Adverse Events (PRO-CTCAE). The PRO-CTCAE produced composite scores, which classified each symptom reporting event as mild, moderate-severe, or severe. A mild symptom classification generates an electronic care plan with recommendations for symptom management; moderate-severe and severe classifications trigger an alert to the care team. Descriptive analyses summarize PROs for early stage and metastatic patients. Symptom burden was assessed by calculating the frequency distribution of each patient’s highest reported composite score for each symptom by month (Table 1). Results: Between September 2020 and April 2022, 280 women enrolled in the RSM program; 201 of these women had complete staging information for analysis. 80% (n=160) had early stage (0-III) and 20% (n=41) had metastatic (IV) disease. 32% (n=64) were less than 50 years old and 68% (n=137) were age 50 or older. 58% (n=116) were hormone receptor (HR) positive/HER2 negative, 22% (n=45) HR+ or -/HER2+ and 19% (n=39) HR-/HER2-. In Month 1, patients with metastatic disease most frequently reported moderate to severe symptoms for general pain (51%), nausea (32%), decreased appetite (22%), and diarrhea (29%). In Month 1, patients with early stage disease most frequently reported moderate to severe symptoms for general pain (32%) and diarrhea (28%). In Month 1, general pain was the most frequently reported symptom for both early stage (34%) and metastatic (51%) groups. In both groups over 16 weeks, nausea, diarrhea, and constipation were among the five most reported symptoms along with muscle pain for early stage patients and shortness of breath for metastatic patients. The frequency of all symptoms decreased over 16 weeks, but there remained cases of moderate-severe and severe symptom intensity through Week 16 for several symptoms. Conclusion: Women with metastatic and early stage breast cancer both report severe symptoms during treatment. Early stage patients may have different symptom profiles and unmet needs not captured by common PROs. Future work should further evaluate symptom profiles of early stage patients to understand how to best use PRO monitoring in the curative intent setting. Percentage of Moderate-Severe and Severe Symptom Reports By Month and Symptom for Women with Early Stage and Metastatic Breast Cancer MO=Month Citation Format: Tara Kaufmann, Aaron Galaznik, Nicholas Coombs, Gabrielle B. Rocque. Patient-Reported Symptom Burden in Women Undergoing Treatment for Early Stage and Metastatic Breast Cancer [abstract]. In: Proceedings of the 2022 San Antonio Breast Cancer Symposium; 2022 Dec 6-10; San Antonio, TX. Philadelphia (PA): AACR; Cancer Res 2023;83(5 Suppl):Abstract nr P5-07-12.

We present a case of a three-month-old male who presented with a cervical esophageal duplication cyst requiring early surgical intervention. The patient presented with feeding difficulties, poor weight gain, and respiratory distress. Due to the position of the cervical esophageal duplication cyst and airway compression, this unique case required a multidisciplinary surgical approach involving both otolaryngology and cardiothoracic surgery.

This case report discusses the management of a 15-year-old Jehovah's Witness (JW) with DiGeorge syndrome, complex congenital heart disease (CHD), and severe neuromuscular scoliosis requiring major orthopedic surgery. Refusal of blood transfusions required preoperative optimization and advanced blood conservation strategies. The patient underwent multiple cardiac surgeries and spinal fusion. This case emphasizes multidisciplinary coordination and blood management strategies for surgical cases with religious restrictions.

Background Despite advances in congenital heart surgery (CHS) and improvements in outcomes, disparities continue to exist. Social Drivers of Health (SDoH) have been associated with many aspects of health and wellness including congenital heart disease. The goal was to evaluate the impact of individual and neighborhood-level SDoH on outcomes following CHS. Methods Retrospective review of the Society of Thoracic Surgeons Congenital Heart Surgery Database from 2014 to 2022 linked via ZIP code with the Centers for Disease Control and Prevention Social Vulnerability Index (SVI). ZIP codes were ranked by their SVI score with the first quintile the least vulnerable and the fifth the most vulnerable. The primary outcome was STS perioperative mortality. Results A total of 49 centers with ≥85% ZIP code completion rate were included. Those with missing ZIP codes ( n = 1329) or missing data ( n = 5651) were excluded resulting in a final cohort of 16,238 (69.5%, 11,285/16,238) cases. Of the final cohort, 8,977/16,238 (55.3%) were Non-Hispanic White and 7084/16,238 (43.6%) had private insurance. There were 448/16,238 (2.8%) STS perioperative mortalities. In the fully adjusted model, individual Non-Hispanic Black race (OR:1.76 [95% CI: 1.20-2.52]), Non-Hispanic Other race (OR: 2.39 [95% CI: 1.10-4.99]), Medicaid (OR:1.55 [95% CI: 1.04-2.30]), None/Self/Charity Insurance (OR:2.60 [95% CI: 1.09-5.93]), as well as increasing SVI quintile with SVI2 (OR: 1.22 [95% CI: 0.86-1.75]), SVI3 (OR: 1.71 [95% CI: 1.07-2.73]), SVI4 (OR: 1.92 [95% CI: 1.04-3.53]) and SVI5 (OR: 3.09 [95% CI: 1.36-6.87]), had increased odds of STS perioperative mortality. Conclusions After adjusting for clinical characteristics, both individual and neighborhood Social Drivers of Health conferred increased odds of perioperative mortality following CHS. These results spotlight the disparities driven by individual circumstances beyond clinical characteristics and reinforce the need to intervene at both the individual and community levels to improve outcomes.