tbuckley/cabot-search huggingface dataset:
Early Mechanical Alterations in Phospholamban Mutation Carriers
Authors: Karim Taha, Wouter P. te Rijdt, Tom E. Verstraelen, Maarten J. Cramer, Rudolf A. de Boer, Rianne H.A.C.M. de Bruin-Bon, Berto J. Bouma, Folkert W. Asselbergs, Arthur A.M. Wilde, Maarten P. van den Berg, Arco J. Teske
Journal: JACC. Cardiovascular imaging (2020)
Abstract 127: Racial Disparities in Management and Outcomes of Cardiac Arrest Complicating Acute Myocardial Infarction
Authors: Anna Subramaniam, Sri Harsha Patlolla, Saraschandra Vallabhajosyula
Journal: Circulation (2020)

Introduction: Racial disparities in outcomes of acute myocardial infarction (AMI) and cardiac arrest (CA) exist. This study assessed the racial differences in the management and outcomes of CA complicating AMI to better inform clinical care. Hypothesis: We hypothesized that racial minorities would have worse outcomes with AMI-CA as compared to white patients. Methods: During 2012-2017, AMI admissions with a concomitant diagnosis of CA were identified from the National Inpatient Sample. Race was classified as white, black and others (Hispanic, Asian or Pacific Islander, Native American, Others). The primary outcome was racial disparities in in-hospital mortality. Secondary outcomes included racial disparities in invasive procedures and hospitalization characteristics. Results: We identified 3,504,225 admissions for AMI in the study period, of which 182,750 (5.2%) were complicated by CA. 74.8% were white, 10.7% were black and 14.5% belonged to other races. Black and other race AMI-CA admissions received less frequent early coronary angiography (41.4% vs 50.2% vs 52.8%), coronary angiography (61.9% vs 70.2% vs. 73.1% %), PCI (44.6% vs 53.0% vs 58.1%), CABG and mechanical circulatory support compared to white and other races. The mean time to coronary angiography was highest among blacks (3.4 ± 4.2 days) and lowest among whites (3.0 ± 3.7 days). Black and other races had significantly higher unadjusted mortality, however in a multivariable logistic regression analysis with white race as referent, black race was associated with lower in-hospital mortality (OR 0.95 [95% CI 0.91-0.99]; p =0.007) whereas other races had higher in-hospital mortality (OR 1.11 [95% CI 1.08-1.15]; p &lt;0.001) compared to white race. AMI-CA admissions of black race had longer length of hospital stay, higher rates of palliative care consultation, less frequent DNR status use, and fewer discharges to home. Admissions of other races had higher use of DNR status and higher hospitalization costs compared to whites and blacks. Conclusions: Significant racial disparities exist in in-hospital mortality among AMI admissions complicated with CA. Further quantitative and qualitative research into the equitable care of racial minorities with AMI-CA is needed to address this disparity.
Abstract 13493: Mechanism and Prognostic Significance of Cardiothoracic Ratio in Patients With Heart Failure With Preserved Ejection Fraction - Insights From Pursuit-hfpef Registry -
Authors: Masato Okada, KOICHI INOUE, Katsuomi Iwakura, Toshinari Onishi, Shungo Hikoso, Daisaku D Nakatani, Shunsuke Tamaki, Masamichi Yano, Takaharu Hayashi, Akito Nakagawa, Yusuke Nakagawa, Yoshio Yasumura, Takahisa Yamada, Yasushi Sakata
Journal: Circulation (2020)

Introduction: The cardiothoracic ratio (CTR) is a widely accepted method of quantifying cardiac size, and carries prognostic information in acquired heart disease. However, little is described in the underlying mechanism and prognostic value in heart failure with preserved ejection fraction (HFpEF). Methods: Analysis was performed from a prospective multicenter observational registry for HFpEF (PURSUIT-HFpEF Registry) conducted in the Osaka region of Japan. A total of 871 patients hospitalized for acute HF (diagnosed by using Framingham criteria) met the inclusion criteria: a left ventricular ejection fraction (LVEF) ≥50% and brain natriuretic peptide (BNP) ≥100 pg/ml. We included 629 patients (age, 81±9 years; men, 46%; atrial fibrillation, 37%) whose follow-up data after survival discharge were available and whose CTR were measured from posterior-anterior chest X-ray. The endpoint was defined as the composite of death, re-hospitalization for HF, and cerebrovascular accident. Results: Median CTR at discharge was 56% (interquartile range: 51-61%). Comparison with echocardiographic data revealed that CTR significantly correlated with increased left atrial size (left atrial volume; r = 0.39, p &lt;0.001) and LV filling pressure (E/e’ ratio; r = 0.13, p = 0.002), but not with LV size (LV end-diastolic volume; r = -0.014; LV end-systolic volume: r = -0.002) and LVEF (r = -0.035). Age (r = 0.16, p &lt;0.001) and log-transformed N-terminal-pro-BNP value (r = 0.21, p &lt;0.001) were also correlated with CTR. Female patients exhibited more higher CTR than male patients (54±7 vs. 58±7%, p &lt;0.001). During a median follow-up of 15 months, 257 patients experienced the endpoint. When patients were classified into the CTR quartile groups, there was a positive correlation between the incidence rate and the quartiles (Q1, 31.0%; Q2, 39.5%; Q3, 35.7%; Q4 55.8%, p &lt;0.001). After adjustment for age and gender, Cox regression analysis revealed that CTR at discharge was a significant predictor of the composite endpoint (hazard ratio per quartile increase; 1.23; 95% confidence interval, 1.10-1.39; p = 0.006). Conclusions: CTR was not associated with LV size but left atrial size in patients with HFpEF. Increased CTR at discharge predicted unfavorable prognosis in patients with HFpEF.
Abstract 13418: Inter-relation of Parity and Coronary Artery Calcium With Cardiovascular Disease Events: The Multi-ethnic Study of Atherosclerosis
Authors: Kimberly Vu, Khoa Nguyen, Jonathan Evans, WENJUN FAN, Morgana Mongraw-chaffin, Matthew J Budoff, Nathan D Wong, Erin D Michos
Journal: Circulation (2020)

Introduction: Coronary artery calcium (CAC) is a measure of subclinical atherosclerosis and predicts cardiovascular disease (CVD) events. Greater parity, or number of live births, has been shown to relate to CVD. We examined whether the relation of parity to CVD events may depend on the presence and extent of subclinical atherosclerosis measured by CAC. Methods: We studied 3151 women free of CVD at baseline in the Multi-Ethnic Study of Atherosclerosis, a prospective study of CVD. Participants were stratified by parity categories of 0-1 (reference), 2-3, and ≥4 and by baseline CAC categories of 0, 1-99, and 100+. We compared the incidence of CVD (myocardial infarction, stroke, resuscitated cardiac arrest, and coronary heart disease deaths) per 1000 years based on parity across levels of CAC over 13 years. Cox regression determined the joint association of parity and CAC on the incidence of CVD. Results: Women with greater parity had a higher prevalence of any CAC and CAC≥100 (p&lt;0.01); among those with CAC, parity related to greater mean CAC scores (175, 184, and 284, respectively) (p&lt;0.01). Women with greater parity also had greater incident CVD (7.1%, 8.7%, and 11.3% for 0-1, 2-3, and ≥4 live births, respectively, p-trend =0.01) and extent of CAC directly related to the incidence of CVD within parity groups. However, the association of parity with CAC was attenuated after adjustment for age, race, income, smoking and other risk factors. Parity also directly related to the incidence of CVD within CAC categories ( Figure ); however, from Cox regression analyses, these relations were attenuated when adjusted for age, ethnicity and other risk factors. Conclusion: In unadjusted analyses, we show parity to be associated with the prevalence of any or significant CAC, extent of CAC among those with CAC&gt;0, as well as the incidence of CVD events, overall and according to the presence and extent of CAC. However, the association of parity with CVD risk was attenuated after adjustment for other factors.
Early-life risk factors for reversible and irreversible airflow limitation in young adults: findings from the BAMSE birth cohort
Authors: Gang Wang, Inger Kull, Anna Bergström, Jenny Hallberg, Petra Um Bergström, Stefano Guerra, Goran Pershagen, Olena Gruzieva, Marianne van Hage, Antonios Georgelis, Christer Janson, Anders Lindén, Erik Melén
Journal: Thorax (2020)

We aimed to determine prevalence and early-life risk factors for reversible and irreversible airflow limitation in young adults from the general population. Among young adults in their 20s, the prevalence was 5.3% for reversible airflow limitation and 2.0% for irreversible airflow limitation. While parental asthma was the only risk factor for development of reversible airflow limitation, the risk factors for development of irreversible airflow limitation were current asthma, childhood respiratory tract infections and asthma, and exposure to air pollution.
Abstract 14322: The Relationship Between Self-care and Health-related Quality Life in Patients With Heart Failure Differs by Age
Authors: Kyoung Suk Lee, Mi Sook Jung
Journal: Circulation (2020)

Background: Although poor self-care is expected to negatively influence health-related quality of life (HRQOL), this relationship is not consistent. The possible reason might be related that older people reconceptualize or change their expectations for HRQOL. Therefore, vigilant monitoring daily symptom status and other self-care activity might not benefit for older patients with HF to improve their HRQOL. Objective: To examine whether the relationship between self-care and HRQOL differs between younger (age &lt;65 years) and older patients (age≥65 years) with HF. Methods: A total of 138 patients were included in this study (60 years, 29% female, 12.3% NYHA functional class III/IV). Patients were asked to complete the Kansas City Cardiomyopathy Questionnaire and the self-care maintenance of the Self-care HF index to measure overall HRQOL and self-care maintenance. To examine the association between self-care maintenance and HRQOL in younger and older patients, two separate linear regressions were performed after controlling for covariates (age, gender, marital status, NYHA functional class, perceived control, and social support). Results: Self-care maintenance and overall HRQOL were not different between younger (n=90) and older patients (n=48). However, self-care maintenance was an independent predictor of overall HRQOL in only older patients, but not their younger counterparts. In older patients, better self-care maintenance was associated with poorer overall HRQOL (β=-0.37, p=0.03). Conclusions: We found that the relationship between self-care and HRQOL is different between younger and older patients with HF. This finding indicates that improving self-care alone does not necessarily improve HRQOL in older patients so that interventions aimed to enhance HRQOL in older patients need to include strategies considering older patients’ conceptualization for HRQOL.
Abstract 13487: Impact of the New Definition of Pulmonary Hypertension in Heart Failure With Preserved Ejection Fraction
Authors: Fusako Sera, Tomohito Ohtani, kei nakamoto, Shungo Hikoso, Daisaku Nakatani, Shunsuke Tamaki, Masamichi Yano, Takaharu Hayashi, Akito Nakagawa, Yusuke Nakagawa, Yoshio Yasumura, Takahisa Yamada, Keiko Yamauchi-Takihara, Yasushi Sakata
Journal: Circulation (2020)

Introduction: The proposed revision of hemodynamic definition of pulmonary hypertension (PH) adopts a lower threshold of mean pulmonary artery pressure (mPAP) &gt; 20 mmHg. In addition, pulmonary vascular resistance (PVR) ≥ 3 Wood units (WU) is included as the definition of pre-capillary component of PH. Heart failure (HF) with preserved ejection fraction (HFpEF) can develop pre-capillary PH as well as post-capillary PH. We aimed to investigate the impact of the proposed definition of PH on clinical diagnosis of PH associated with HFpEF. Methods: From the PURSUIT-HFpEF (Prospective Multicenter Observational Study of Patients with Heart Failure with Preserved Ejection Fraction) registry, 225 patients who were hospitalized with HF and underwent right heart catheterization were categorized according to the current guidelines and the proposed definition of PH: non-PH, isolated post-capillary PH (Ipc-PH), pre-capillary PH, and combined pre- and post-capillary PH (Cpc-PH). In the proposed definition, patients with mPAP &gt; 20 mmHg, PVR &lt; 3 WU, and pulmonary artery wedge pressure ≤ 15 mmHg do not meet criteria for any of the above categories and are categorized as “unclassified PH”. Results: Prevalence of PH was significantly increased in the proposed definition compared to that in the current definition (51% vs 29%, p&lt;0.0001), with a doubled frequency of pre-capillary PH (Fig A). Furthermore, 24 patients (11%) were diagnosed as unclassified PH and accounted for 22% of those with PH by the proposed definition. Among the PH categories in the proposed definition, Cpc-PH category was significantly relevant for worse prognosis at 1 year after discharge in patients with HFpEF (p=0.03 vs non-PH by log-rank test with Bonferroni's correction) (Fig 2). Conclusions: The new definition of PH resulted in a remarkable increase of prevalence of PH in HFpEF with a quite a few patients with unclassified PH and doubled frequency of pre-capillary PH.
Abstract 126: Intravenous Versus Intraosseous Epinephrine Administration for Pediatric Patients with Out-of-hospital Cardiac Arrest
Authors: Masashi Okubo, Cameron Dezfulian, Francis X Guyette, Christian Martin-Gill, Sylvia Owusu-Ansah, Clifton W Callaway
Journal: Circulation (2020)

Introduction: The 2015 American Heart Association Guidelines for Cardiopulmonary Resuscitation recommend intravenous (IV) or intraosseous (IO) epinephrine administration for pediatric patients with out-of-hospital cardiac arrest (OHCA). However, it is unknown whether the route of epinephrine administration affects patient outcomes. Our objective was to evaluate the association between the route of epinephrine administration and survival. Methods: We conducted a secondary analysis of the Resuscitation Outcomes Consortium Epistry, a prospective multicenter OHCA registry from 2011 through 2015 in North America. We included pediatric patients (≤18 years) with OHCA for whom emergency medical services (EMS) providers attempted resuscitation and administered epinephrine via IV or IO. We excluded patients who received endotracheal epinephrine, received both IV and IO epinephrine, received IV epinephrine with failed IO access, and received IO epinephrine with failed IV access. The primary outcome was survival to hospital discharge. We used multivariable logistic regression and adjusted for age, sex, initial rhythm, location of arrest, witness status, receiving layperson cardiopulmonary resuscitation, 9-1-1 call to EMS arrival, and advanced airway management. We also conducted a propensity score matching analysis with the same covariates. Results: Of the eligible 831 pediatric patients with OHCA, 226 (27.2%) received IV epinephrine and 605 (72.8%) IO epinephrine. Median interval between 9-1-1 call and epinephrine administration was 16.4 minutes (interquartile range [IQR] 12.9-21.0) in IV group and 16.2 minutes (IQR 12.8-20.0) in IO group. In the logistic regression model, the adjusted odds ratio (OR) of the IO group for survival to hospital discharge was 0.99 (95% confidence interval [CI] 0.41-2.40), compared with the IV group. Similarly, in the propensity score analysis, 218 patients underwent matching with good balance (standardized differences &lt;0.25 for all covariates) and the OR of the IO group for survival to hospital discharge was 1.00 (95% CI 0.38-2.62). Conclusions: We observed no significant difference in survival to hospital between pediatric patients with OHCA who received epinephrine via IV and IO routes.
Abstract 13585: Genomics-first Evaluation of Phenotypes Associated With Pathogenic or Likely Pathogenic Transthyretin Variants
Authors: Brendan Carry, Katelyn Young, Melissa Kelly, Amy C Sturm, Jose D Avila, Brandon Fornwalt, Christopher M Haggerty
Journal: Circulation (2020)

Introduction: Hereditary transthyretin ( TTR ) amyloidosis as a cause of cardiomyopathy has historically been challenging to diagnose. With increased genetic testing, quantification of penetrance and disease associations through genomics-first approach provides a first step towards improved diagnosis and treatment. Methods: In the Geisinger MyCode Community Health Initiative, we identified pathogenic and likely pathogenic (P/LP) variants in TTR from 135,878 individuals with exome sequence data linked to electronic health records (DiscovEHR cohort) using ANNOVAR. We compared diagnoses and imaging findings between TTR variant carriers/noncarriers using SAIGE mixed linear models. Results: We observed 7 P/LP TTR variants (6 P, 1 LP) in 158 individuals (0.12%; 142 P, 16 LP). Median carrier age was 52 (IQR: 37-61) and 43% were male. The most common variant was V122I (i.e., p.V142I; 114, 0.08%). Most TTR variant carriers were of African ancestry (58%), all of whom carried V122I, consistent with the frequency of this variant in that population (2.6%). Observed associations with cardiac amyloidosis (odds ratio (OR): 2.8E 20 , p = 0.03) and non-specific amyloidosis (OR: 4E 4 , p = 0.03) were significant, although prevalence was low (n = 1 and 2 TTR carriers, respectively). No associations were observed with other specified diagnoses (Figure) or imaging findings. Considering only individuals aged ≥60, we observed a significant association with non-ischemic, non-dilated cardiomyopathy diagnoses (OR: 5.8, p=0.04) involving carriers of V122I (n = 3), V30M (p.V50M), T60A (p.T80A), and I68L (p.I88L; n = 1 each). Conclusion: Genomics-first screening of a clinical population for P/LP variants in TTR has a moderate to high yield depending on population ancestry. In the absence of symptoms or family history, initiating routine surveillance may be most appropriate after age 60. Deeper phenotyping is warranted to better characterize penetrance and ramifications for management.
Abstract 13046: Treatment of Highly Sensitized Patients Before Heart Transplant Associated With Long-term Post-transplant Benefit
Authors: Timothy Ho, Jignesh K Patel, Keith Nishihara, Michelle M Kittleson, David H Chang, Evan P Kransdorf, Lawrence S Czer, Xiaohai Zhang, Jon A Kobashigawa
Journal: Circulation (2020)

Introduction: Highly sensitized patients awaiting heart transplantation (HTx) are disadvantaged by a narrow donor pool due to incompatible potential donors. Desensitization therapy has been demonstrated to be effective in lowering antibodies and thereby enlarging the donor pool. By desensitization, the calculated panel reactive antibody (cPRA) has been reported to decrease and result in a shorter waitlist time. It has not been established whether desensitization before transplant can have impact after HTx outcomes. Methods: Between 2010 and 2015, we assessed 34 patients awaiting HTx who had a PRA &gt;50% and underwent desensitization therapy. Desensitization therapy included combinations of IVIG, rituximab, plasmapheresis, and bortezomib. These patients who underwent HTx were then assessed for outcomes, which included 5-year survival, freedom from cardiac allograft vasculopathy (CAV), freedom from non-fatal major adverse cardiac events (NF-MACE), and freedom from donor specific antibody (DSA) development and 1-year freedom from rejection (any treated rejection (ATR), acute cellular rejection (ACR), antibody mediated rejection (AMR)). Patients who underwent desensitization were compared to a similar group of patients with PRA &gt;50% who did not undergo desensitization and underwent HTx. Results: Patients who underwent desensitization compared to those who did not had significantly greater freedom from 5-year development of CAV (94.1 vs 78.8%, p=0.039). There was no significant difference in 5-year survival, 5-year freedom from NF-MACE or DSA development, and 1-year freedom from ATR, ACR, AMR. Conclusions: Desensitization in highly sensitized patients awaiting HTx appears to have long ranging effects to modulate the immune system after HTx, resulting in less development of CAV. Further investigation should be pursued to understand the mechanism of this intervention.
Abstract 14226: Resilience in Young Adults With CHD: Predictors and Impact on Quality of Life
Authors: Alana Cianciulli, Richard Ittenbach, Gabrielle Combs, Isabel Randazzo, Daniel J Licht, Lyla Hampton, Marsha Gerdes, Judy C Bernbaum, Nancy Burnham, James W Gaynor
Journal: Circulation (2020)

Introduction: Resilience is the ability to recover after stressful events and is believed to help reduce the long-term adverse effects. The role of resilience as a determinant of neurodevelopmental (ND) outcomes and quality of life (QOL) in young adults with congenital heart disease (CHD) has not been fully delineated. Hypothesis: Higher resilience in young adults with CHD is associated with better QOL and can be predicted by patient factors and ND status in the preschool period. Methods: Prospective evaluation of subjects ≥ 18 years of age enrolled in a study of genetic determinants of ND outcome after repair of CHD at age ≤ 6 months. The subjects had previously undergone a ND evaluation (executive function, attention/impulsivity and social skills) at 4 years of age. For this study, subjects completed the RS-14, a questionnaire that captures the constructs of resilience; and the SF-36, a functional health status questionnaire. Factors associated with resilience were determined using generalized linear (gamma) regression. Results: The RS-14 and SF-36 were completed by 151 subjects (74 male and 77 female, mean age 19.5 ± 1.1 years). The median resilience score of 79 (CI 0.95 = 77, 82) was significantly greater than the young adult population median of 76.0, p = 0.006. Resilience was graded as moderate or higher in 103 subjects (68%). Higher resilience scores were significantly correlated with higher QOL assessed by the SF-36 Mental Component Summary, r = 0.40, p &lt; 0.001. By simple regression, Caucasian race was associated with higher resilience scores, p = 0.03. Prematurity, low birth weight, presence of a genetic anomaly, and severity of CHD were not associated with higher or lower scores, all p &gt; 0.2. No operative management factors were associated with higher or lower scores, all p &gt; 0.28. Better performance for executive function ( p = 0.03) and social skills ( p = 0.02) at the 4-year ND evaluation were associated with higher resilience. Hyperactivity was associated with lower resilience scores, p = 0.02. Conclusions: Many young adults with CHD demonstrate good resilience. Higher resilience is associated with better QOL. Performance on ND testing in the preschool period may identify patients with lower resilience and offer an opportunity for intervention.
Response to: 'Correspondence on 'Impact of COVID-19 pandemic on patients with large-vessels vasculitis in Italy: a monocentric survey' by Comarmond et al
Authors: Alessandro Tomelleri, Silvia Sartorelli, Elena Marina Baldissera, Lorenzo Dagna, Corrado Campochiaro
Journal: Annals of the Rheumatic Diseases (2020)

We read with interest the correspondence by Comarmond et al 1 on our study about the impact of novel COVID-19 on Italian patients with large-vessels vasculitis (LVV).2 COVID-19 has been declared a global pandemic by WHO in March 2020. Since then, the number of patients diagnosed with SARS-CoV-2 infection has rapidly grown and today more than 46 million cases worldwide have been identified.3

International and National surveys on different groups of patients with chronic inflammatory diseases have been performed to evaluate the impact of COVID-19 pandemic among frail patients.4 5 After a first study by our group, based in Milan, Italy,2 Comarmond et al reported the results of a survey conducted among patients affected by LVV, followed up at Pitie-Salpetriere Hospital (Paris, France).1 Both studies were performed during the so-called ‘first wave’ of the pandemic, in areas characterised by a high rate of infection, and among patients followed up in tertiary referral centres. In addition, the number of patients included and the ratio between patients affected by the two …