tbuckley/cabot-search huggingface dataset:
Abstract 10252: State Wide Pregnancy Outcomes for Adolescents and Adults with Single-Ventricle Congenital Heart Disease - A 14 Year Review
Authors: Andrew Well, Sruthi Ilangovan, Amelia Mercado, Catherine Dawson, Michelle Mizrahi, Hanoch Patt, Gregory Johnson, Charles D Fraser, Carlos M Mery, Ziv Beckerman
Journal: Circulation (2021)

Introduction: With advances in care, survival into adulthood of single-ventricle (SV) palliation is more frequent. Fertility and family planning in this setting is complex. In the general population, maternal mortality is 0.02%, post-partum hemorrhage (PPH) is 3.1% and pre-eclampsia is 7.5%. There are limited data on outcomes of pregnancies of women with SV physiology. This study reviews deliveries of SV mothers in Texas over the past 14 years. Methods: This is a retrospective review of the Texas Inpatient Discharge Dataset, an administrative dataset containing most hospital discharges. All discharges of females≥15 years old between 1/1/2006-12/31/2019 were reviewed. ICD-9/10 diagnosis codes consistent with Hypoplastic Left Heart Syndrome (HLHS), Double Inlet Ventricle (DIV)/Common Ventricle (CV), and Hypoplastic Right Heart Syndrome (HRHS)/Tricuspid Atresia (TA) were included. Pregnancy-related discharges were identified by ICD-9/10 codes. Descriptive and univariate statistics were utilized. Results: A total of 56 pregnancy-related discharges were identified, with 40 (71.4%) deliveries (Table). One (2.5%) was a twin gestation and 4(10%) had fetal demise. Number of annual deliveries increased from 0 in 2006 to 8 in 2019. Median gestational age was 37[IQR: 33.25-38] weeks, 7 (17.5%) had fetal anomalies and 7(17.5%) had poor fetal growth. Twelve (30%) underwent C-Section. Median length of stay was 3.5[2-5] days, 3(7.5%) mothers had PPH, 2(5%) required ventilator support, and there were no in-hospital mortalities. Conclusions: As the number of patients with SV surviving into adulthood increases, accurate counseling on risks of pregnancy is required. While these data cannot evaluate the outcomes for the delivered baby, they suggest favorable short-term outcomes for mothers with no in-hospital mortalities. Further research is needed to both understand outcomes for the delivered babies as well as potential longer-term sequelae for mothers.
Abstract 11186: A Case for Exercise-Induced Arrhythmogenic Right Ventricular Cardiomyopathy
Authors: Emrie D Tomaiko, Sharon Kusne, Peter Weiss, Michael Zawaneh, Wilber Su
Journal: Circulation (2021)

Introduction: Sudden cardiac arrest has been documented in athletes, often secondary to left ventricular (LV) hypertrophy or previously undetected genetic conditions. Some athletes, however, experience these events without evidence of either LV or genetic disease. A hypothetical exercise-induced arrhythmogenic right ventricular cardiomyopathy (ARVC) has been previously described by La Gerche and colleagues, acquired through repeated, intense endurance exercise and associated cardiac remodeling rather than genetic inheritance. We present a case report of an endurance athlete presenting with ventricular tachycardia (VT), suspected to be secondary to exercise-induced ARVC. Results: A 40 year-old male endurance athlete was brought to the emergency department when he collapsed following a race in 2006. Upon arrival, he was in sustained wide-complex tachycardia at a rate of 200-220 BPM. Following conversion with IV amiodarone, left heart catheterization was performed, showing no significant coronary disease and LV ejection fraction (EF) measured 30-35%. He then underwent right ventricular outflow tract ablation. Cardiac MRI was inconclusive for ARVC. Biopsy was performed, negative for amyloidosis but with “focal mild to moderate chronic interstitial inflammatory infiltrates associated with focal muscle fiber necrosis.” Genetic testing was negative for pathologic variants or mutations associated with ARVC. Echocardiogram from 2020 showed EF 40-44% with significant RV enlargement and dysfunction. He required repeat VT ablation in 2010, 2014, and 2017. Arrhythmogenic scar was identified by electrophysiology mapping to be located at the sub-tricuspid RV. In combination with the prior RV outflow tract ablation, this is suggestive of the theoretical exercise-induced ARVC. Conclusions: There is still debate regarding right ventricular remodeling in endurance athletes. Increased volume and pressure in response to demand for cardiac output associated with this intense exercise has been proposed as a possible etiology of RV cardiomyopathy in athletes without identifiable genetic predisposition. Our case report supports this hypothesis.
Abstract 13949: Voronoi Characteristics for the Improvement of Middle Cerebral Aneurysm Rupture Differentiation
Authors: JingFeng Jiang, Kevin Sunderland, Seyedmostafa Rezaeitaleshmahalleh, Tang Tang, Azam S Ahmed, Joseph Gemmete, aditya pandey
Journal: Circulation (2021)

Introduction: Surgical intervention for detected intracranial aneurysms (IA) cary risks to patients. Analysis of IA geometry may help clinicians differentiate IAs at high rupture risk versus ones likely to remain stable. Yet traditional geometric indices lack sufficient accuracy, stressing a need for novel indices. Hypothesis: Adding novel Voronoi diagram-based indices (see Fig. 1) will improve IA differentiation. Methods: 3D computational models were taken from 47 subjects with one medium-sized (4-10mm) middle cerebral artery (MCA) IA via 3D digital subtraction angiography data. 7 geometric indices for IAs ( e.g. size, aspect ratio) were measured, alongside Voronoi analysis. In terms of the Voronoi diagram/sphere, each IA constitutes one or more spherical cores (large Inscribed sphere(s)) and many small protrusions. 2 variant curves denoting the inscribed spheres to small protrusion size’s relationships can be seen in Fig 1. Support vector machine with cross-validation (100 iterations) assessed Voronoi diagram-based curve’s strength for IA differentiation. The parsimonious model was determined as 3 geometric indices (volume, identified bulb, vessel diameter) and Voronoi characteristics. Results: Conventional IA geometric indices for ruptured vs unruptured differentiation showed limitations: 0.80 AUROC, 0.76 total accuracy. The addition of quantified Voronoi characteristics via both Voronoi curves augmented model strength for differentiation: 0.87 AUROC, 0.86 total accuracy. Conclusions: Preliminary analysis suggests including Voronoi characteristics for IA analyses improves rupture status prediction accuracy for medium-sized MCA aneurysms, which are more difficult to manage. Since Voronoi characteristics can be easily done in the clinical workflow, such an analysis may enrich the differentiation of IAs.
Abstract 11721: FOXK1 is an Essential Transcriptional and Epigenetic Regulator of Cardiovascular Development
Authors: Javier Sierra-Pagan, Nikita Dsouza, Thijs A Larson, Satyabrata Das, Patricia Stan, Wuming Gong, Mary G Garry, Daniel J Garry
Journal: Circulation (2021)

Background: Congenital heart disease (CHD) is the most common genetic birth defect and has considerable morbidity and mortality. Therefore, it is essential to define the mechanisms governing the specification and differentiation of mesodermal and cardiac progenitors to develop targeted therapies for CHD. FOXK1 is a forkhead/winged helix transcription factor known to regulate cell cycle kinetics, myogenic stem cell proliferation and tumorigenesis. During development, FOXK1 expression is restricted to mesodermal progenitors, somites and the heart. The role for FOXK1 in the developing heart is unknown and warrants investigation. In the present study, we describe a novel role for FOXK1 as an essential regulator of cardiovascular development. Approach and Results: We used a mouse embryoid body (EB) system to differentiate WT and Foxk1 null ESCs into mesodermal and cardiac progenitor cells. Flow cytometry analysis for FLK1+PDGFRa+ showed that in the absence of FOXK1, the cardiac lineage was significantly affected. Bulk RNAseq analysis of D3 and D5 EBs showed a significant induction of the cardiac molecular program in WT EBs compared to the Foxk1 null EBs. Since forkhead transcription factors have been shown to be important epigenetic regulators (i.e. pioneer factors), we performed ATACseq of D3 and D5 EBs, which showed that the chromatin landscape nearby known important regulators of cardiogenesis (Isl1, Gata4, Hand1, Hand2, etc.) was significantly relaxed in WT EBs compared to Foxk1 null EBs. Furthermore, we demonstrated that in the absence of Foxk1 , cardiac differentiation was markedly impaired by assaying for cTnT expression and cardiac contractility which was essentially absent in Foxk1 null EBs compared to WT EBs at D10 of differentiation. Additionally, using transcriptional, Co-IP and pulldown assays we demonstrated that FOXK1 is an important regulator of Notch and Wnt/β-catenin signaling pathways during cardiogenesis. These results were further supported as the Foxk1 null embryo was lethal at E9.5 and had perturbed heart development. Conclusions: These results identify FOXK1 as an essential transcriptional and epigenetic regulator of cardiovascular development using EB assays and gene disruption technologies.
Abstract 13333: Systolic Blood Pressure Is An Independent Risk Factor For Diabetic Retinopathy In Both Hypertensive And Normotensive Patients
Authors: Yu-Ting Li, Yi Wang, Xiu-Jing Hu, Bedru H. Mohammed, Xiao-Ling Liang, Jose Hernandez, Wen-Yong Huang, Harry H.X. Wang
Journal: Circulation (2021)

Introduction: Treatments with targets of systolic blood pressure (BP) &lt;140 mmHg reduce the onset, or decrease the progression of diabetic retinopathy (DR) - a most common microvascular complication in type 2 diabetic patients with established hypertension. However, the debate on further intensive vs. standard BP control targets in limiting the onset and progression of diabetic microvascular complications remains to be an area of controversy. We aimed to explore whether the extent to which systolic BP levels were associated with the presence of DR in type 2 diabetic patients with normal BP were similar to that in patients with coexisting hypertension. Hypothesis: We assessed the hypothesis that systolic BP levels were not associated with DR in normotensive patients. Methods: This was a baseline analysis of a cohort of adult Chinese patients diagnosed with type 2 diabetes who attended ophthalmoscopic exam with primary care referral in the context of a Specialist-Generalist Working Alliance in Guangzhou, China. A hospital-based, standardised investigation included a dilated, comprehensive eye examination, a measurement of BP values and anthropometric parameters, and a clinical laboratory test for haemoglobin A1c and lipid profiles. A binary logistic regression analysis was conducted to examine patient-level risk factors associated with DR. Results: The mean age of all study participants (N=2,510) was 64.8 years, and more than half were females. More than one in three patients (40.4%) were normotensive. The average duration of diabetes was 8.6 years, and one in five patients were clinically diagnosed with DR. A higher systolic BP level was significantly associated with DR (adjusted odds ratio [aOR]=1.019, 95%CI: 1.012-1.027; p&lt;0.001 for hypertensives, and aOR=1.020, 95%CI: 1.005-1.036; p=0.009 for normotensives), after adjusting for diabetes duration, sex, lifestyle behaviours, and HbA1c levels. Conclusions: In conclusion, we found that increased systolic BP level was an independent, significant risk factor associated with DR in both hypertensive and normotensive diabetic patients. Physicians, patients and caregivers are recommended to maintain regular monitoring of BP in all diabetic patients irrespective of the coexistence of hypertension.
Abstract 9459: Cardiac Troponin I and the Risk of Cardiovascular or Non-Cardiovascular Death in Patients Visiting Emergency Department
Authors: Jong Eun Park, Jin-Ho Choi, Sung Yeon Hwang, Minseok Song
Journal: Circulation (2021)

Background: To identify the magnitude and temporal relationship between cardiac troponin I (TnI) and the risk of cardiac or non-cardiac death. Methods: Consecutive patients were included with maximal TnI value within 24 hours of their emergency department visit. Multivariate analyses using parameters selected by Bayesian information criterion was performed to investigate the impact of TnI on the event rate, time-dependent risk, and dose-dependent risk of cardiac or non-cardiac death within 360 days. We investigated the association between cardiac troponin I (TnI) and the risk of cardiac or non-cardiac death according to period. Results: A total of 36806 patients were included. There were 5,472 (14.9%) all-cause deaths including 881 (2.4%) cardiac and 4,591 (12.5%) non-cardiac deaths. In patients with positive TnI defined as the ≥99 th percentile of upper normal limit, the cumulative risk of cardiac and non-cardiac death were 4.4- and 1.4- fold higher compared to negative TnI, respectively. In competing risk analysis, positive TnI was linked to 2.4- and 1.2-fold higher risks of cardiac and non-cardiac death, respectively. TnI value showed positive relationship with the risk of both cardiac and non-cardiac death. In time-dependent risk analysis, the excess risk of cardiac death was concentrated in early few weeks. Conclusions: Even a small increase in TnI was associated with increased risk of both cardiac and non-cardiac death, which was concentrated in the early period after emergency department visit.
QOLP-37. MOOD DISTURBANCE IN PATIENTS WITH CENTRAL NERVOUS SYSTEM (CNS) TUMORS DURING THE COVID-19 PANDEMIC
Authors: Lily Polskin, Valentina Pillai, Elizabeth Vera, Alvina Acquaye, Nicole Briceno, Anna Choi, Alexa Christ, Ewa Grajkowska, Varna Jammula, Heather Leeper, Jason Levine, Matthew Lindsley, Jennifer Reyes, Kayla Roche, James Rogers, Michael Timmer, Lisa Boris, Eric Burton, Nicole Lollo, Marissa Panzer, Marta Penas-Prado, Brett Theeler, Jing Wu, Mark Gilbert, Terri Armstrong, Amanda King
Journal: Neuro-Oncology (2021)

Abstract BACKGROUND Primary CNS tumors are associated with uncertainty likely contributing to mood disturbance that is common throughout the disease trajectory. The intersection of the COVID-19 pandemic with a CNS tumor diagnosis may further impact the anxiety/depression experienced in this population. This study assessed key anxiety/depression symptoms in patients with CNS tumors prior to and during the COVID year. METHODS Patient reported outcomes (PROs), including the PROMIS Anxiety and Depression Short Forms and EQ-5D-3L, were collected at the time of clinical or telehealth evaluation from the COVID year (March 2020-February 2021) and were compared to assessments through February 2020 (a NOB-normative sample), reflecting what we would typically see in our regular clinic evaluations. RESULTS The COVID sample (N = 178) was primarily White (82%), male (55%), median age of 45 (range 18–79), and KPS ³ 90 (50%). The majority had high grade (70%) brain (83%) tumors with ³ 1 prior recurrence (60%) and 25% were on active treatment. Visits were primarily conducted via telehealth (64%) and 20% had progression at assessment. Compared to the NOB-normative sample, patients reported significantly higher depression scores (moderate-severe, 17% vs. 12%, p &amp;lt; 0.05), but not anxiety (18% vs. 16%). Eleven percent reported both moderate-severe anxiety and depressive symptoms (8% pre-COVID). Overall health assessed by the EQ-5D-3L was similar to the normative sample in all dimensions, apart from impact of moderate/extreme mood disturbance, which was more prevalent in the COVID year (53% vs. 43%, p &amp;lt; 0.05%). CONCLUSION Patients with CNS tumors are at risk for significant symptoms of depression and anxiety; this risk was heightened during the COVID year. Further evaluation of clinical factors associated with risk are underway. These findings highlight the need for assessments and interventions that can be administered via telehealth to address the mental health needs of this vulnerable population. Radiobiology
Abstract 10836: Outcomes of Biventricular Pacing versus Conventional Pacing (DDD Mode) in Patients with a Normal Left Ventricular Ejection Fraction
Authors: Siddharth Shah, Kuldeep Shah, Mohit K Turagam, Rahul Bhardwaj, Tahmeed Contractor, Ravi Mandapati, Andrea Natale, Dhanunjaya Lakkireddy, Jalaj Garg
Journal: Circulation (2021)

Introduction: Cardiac resynchronization therapy (CRT) is beneficial in patients with heart failure with reduced ejection fraction and normal sinus rhythm. However, its role in patients with pacing indication, narrow QRS, and normal ejection fraction remains controversial. Objectives: We aimed to evaluate the impact of biventricular pacing (BiV) versus conventional right ventricular (RV) pacing (DDD mode, ventricular pacing avoidance algorithm) in patients with normal ejection fraction. Methods: A comprehensive literature search in PubMed, Google Scholar, and Cochrane Review from inception to November 15, 2020, was performed. Outcomes studies were - major adverse cardiovascular events (MACE, composite of cardiovascular death, hospitalization for heart failure or any cardiovascular cause), cardiovascular mortality, change in left ventricular ejection fraction (LVEF, %), left ventricular end-systolic volume (LVESV, mL) and left ventricular end-diastolic volume (LVEDV, mL). Results: Four randomized trials (n=457, BiV group=229, mean age 71.6±4.4 years, RV pacing=228, mean age 70.3±4.38 years) met inclusion criteria. There was a significant reduction in MACE (RR 0.68, 95% CI 0.46-0.99, p=0.04), LVESV [mean difference (MD) -6.42, 95%CI -10.27- -2.57; p=0.001], LVEDV (MD -6.12,95% CI -9.30 to -2.94; p=0.0002) in patients with BiV pacing compared to RV pacing. There was no reduction in cardiovascular mortality and a trend towards improvement in LVEF (MD 3.99,95% -0.12-8.10; p=0.06) with BiV pacing compared to RV pacing during the follow-up period (1.95 years). Conclusion: BiV pacing may be a feasible option for permanent pacing in patients with normal LVEF and may attenuate untoward effects of conventional RV pacing on the left ventricular function.
Abstract 13026: What Causes Speedy Growth (Within 3 to 6 Months) of Vulnerable Plaques in Acute Coronary Syndrome: An Angiographic and Machine Learning Analysis
Authors: Thach N Nguyen, Thoa Le, Minh H Le, Ngoc H Vuong, DO H LE, Nguyet T Nguyen, Trang T Le, Thoai H Nguyen, Thinh Cao, Gianluca Rigatelli, Marco Zuin, Hung N Pham, Dung T Ho, Ernest Talarico, Nghia T Nguyen Thuong
Journal: Circulation (2021)

Introduction: Based on damages by flow abnormalities seen in pipes, our research is built on the hypothesis that the flow disturbances start and grow atherosclerotic plaques. Our strategy is to use knowledge and practice of fluid mechanics to identify the different types of abnormal coronary flows and correlate them with the appearance and growth of lesions. Hypothesis: In patient with fast tract ACS when a normal segment becomes severely stenotic in a matter of 3 to 6 months, what kind of abnormal flow could trigger this speedy growth of a plaque ? Methods: Patients were selected if there was a near normal segment in the first angiogram and severe lesion in the 2nd angiogram 3 to 6 months later. LDL cholesterol level and HbA1c were also measured. In our cardiac catheterization laboratories, in the past 5 years, all patients underwent a special new dynamic coronary angiogram protocol. The contrast was injected until the coronary artery was completely opacified. As the injection of contrast stopped, the blood in white color moved in and displaced the contrast in black. The morphologies, movements and interactions of the blood flow in white color above a black background were reviewed frame by frame by investigators. The main measurements were the types of flow, their durations and direction at the index segment of the 1rst angiogram. At the same time, an artificial intelligence (AI) program (mainly Machine Learning (ML) algorithm) searched by 2 models built on Python. Model 1 was based on U-net and Densenet-121 for vessel segmentation. Model 2 was used for classification of flow (laminar, turbulent, antegrade, retrograde or stagnant). Model 2 was trained based on the convolutional neural network. Results: Ten dual angiograms were selected. At the index segment of the 1st angiogram, 80% showed turbulent and retrograde flow. The duration of this turbulent flow lasted 80% of one cardiac cycle (diastole and systole). The duration of retrograde flow lasted 90% of one systolic phase. A high level of LDL cholesterol (120mg%) was statistically significant while the HbA1c was not. Conclusions: In patient with fast tract ACS when a near normal segment became severely stenotic in a matter of 3 to 6 months, prolonged turbulent and retrograde flow with high LDL cholesterol were the culprits.
BRMP-03. Pituitary carcinoma: a case of dramatic response to immunotherapy (ipilimumab + nivolumab) after failure with temozolomide
Authors: Sanjit Shah, Saima Manzoor, Yehudit Rothman, Matthew Hagen, Luke Prater, Karl Golnik, Abdelkader Mahammedi, Andrew Lin, Ruchi Bhabhra, Jonathan Forbes, Soma Sengupta
Journal: Neuro-Oncology (2021)

Abstract INTRODUCTION Pituitary carcinoma (PC) accounts for just 0.1% of all pituitary tumors, often recurs following resection, and has a median reported survival of 1 year. Current treatment guidelines are not standardized but combine surgical resection, radiation therapy, and chemotherapy [1]. Temozolomide is the only chemotherapeutic with documented effectiveness, and the only recommended agent for aggressive pituitary carcinomas in ESE clinical guidelines [3]. CASE: A 57-year-old male presented with visual deterioration over a three-month period. Ophthalmologic evaluation revealed bitemporal visual field deficits. MRI brain W/WO demonstrated a sellar mass suspected to be pituitary macroadenoma with displacement of the stalk and optic nerve impingement (Figure 1a). The patient underwent stereotactic endoscopic transsphenoidal resection of the mass [2]. Postoperative MRI demonstrated gross total resection (Figure 1b). Pathology revealed a sparsely granulated corticotroph-adenoma with malignant transformation (early in-situ PC). Immunohistochemistry showed LOE of MLH1 and PMS2 in the tumor cells; Genetic analysis revealed MGMT methylation. Proton therapy was recommended given the elevated Ki67 index (75%) and p53 positivity. Before radiotherapy, there was no evidence of residual tumor or metastasis radiographically. He received 6600cGy of radiation over 33 fractions. Surveillance MRI showed recurrence at 21 months postoperatively, and temozolomide was initiated. However, MRI demonstrated marked progression after 3 cycles, and at 44 months, he developed a new 6th nerve palsy (Figure1c). Next-generation sequencing using the MSK-IMPACT platform identified somatic mutations in MLH1 Y548lfs*9 and TP53 R337C[4]. Immunotherapy with ipilimumab/nivolumab was initiated [5], and the patient noted resolution of his third nerve palsy soon after. MRI demonstrated a dramatic response with only minimal residual tumor burden (Figure1d). CONCLUSION PC is a rare tumor with frequent recurrence and a short median expected length of survival. Here we demonstrate the utility of immunotherapy in a single case report of PC. This treatment helped our patient survive well beyond the expected median life expectancy of this aggressive disease.
Abstract 13133: Heterogeneous Uptake of [99mTc]microspheres and 201Tl in Skeletal Muscles in Porcine Model of Hindlimb Ischemia
Authors: Supum Lee, Pedro Gil de Rubio Cruz, Stephanie Thorn, Zhao Liu, christi hawley, Marina Mamarian, Tymoteusz Turlejski, Ling Jian Meng, Chi Liu, Albert Sinusas
Journal: Circulation (2021)

Introduction: There is a need for quantitative methods to evaluate skeletal muscle blood flow in PAD patients. We assessed skeletal muscle blood flow heterogeneity at rest or vasodilator stress usi...