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Copy number variants (CNVs) are the gain or loss of DNA segments in the genome that can vary in dosage and length. CNVs comprise a large proportion of variation in human genomes and impact health conditions. To detect rare CNV associations, kernel-based methods have been shown to be a powerful tool due to their flexibility in modeling the aggregate CNV effects, their ability to capture effects from different CNV features, and their accommodation of effect heterogeneity. To perform a kernel association test, a CNV locus needs to be defined so that locus-specific effects can be retained during aggregation. However, CNV loci are arbitrarily defined and different locus definitions can lead to different performance depending on the underlying effect patterns. In this work, we develop a new kernel-based test called CONCUR (i.e., copy number profile curve-based association test) that is free from a definition of locus and evaluates CNV-phenotype associations by comparing individuals' copy number profiles across the genomic regions. CONCUR is built on the proposed concepts of "copy number profile curves" to describe the CNV profile of an individual, and the "common area under the curve (cAUC) kernel" to model the multi-feature CNV effects. The proposed method captures the effects of CNV dosage and length, accounts for the numerical nature of copy numbers, and accommodates between- and within-locus etiological heterogeneity without the need to define artificial CNV loci as required in current kernel methods. In a variety of simulation settings, CONCUR shows comparable or improved power over existing approaches. Real data analyses suggest that CONCUR is well powered to detect CNV effects in the Swedish Schizophrenia Study and the Taiwan Biobank.
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Biologia Computacional/métodos , Variações do Número de Cópias de DNA/genética , Algoritmos , Área Sob a Curva , Predisposição Genética para Doença/genética , Variação Genética/genética , Genoma Humano/genética , Estudo de Associação Genômica Ampla/métodos , Genômica/métodos , Humanos , Polimorfismo de Nucleotídeo Único/genética , Análise EspacialRESUMO
BACKGROUND: Patients with nonobstructive coronary artery disease (CAD) have worse outcomes compared with those without CAD; however, few studies have compared the intermediate- and long-term impact of CAD severity as a function of patient sex. METHODS: We evaluated 5-year and long-term all-cause mortality of women and men undergoing elective coronary angiography at a single center by degree of CAD: no CAD (1%-24% stenosis), nonobstructive CAD (25%-69% epicardial stenosis or 25%-49% left main stenosis), or obstructive CAD (epicardial stenosis ≥70% or left main stenosis ≥50%), both overall and after adjusting for baseline clinical risk factors using Cox proportional-hazards models. RESULTS: Between January 1986 and July 2010, 8,766 women and 11,638 men underwent angiography and were followed for a median of 9.2â¯years. The majority (67%) of women had no CAD or nonobstructive CAD, whereas the majority of men had obstructive CAD (56%, Pâ¯<â¯.001). In both sexes, increasing CAD was associated with increased 5-year risk of mortality. Risk-adjusted hazard ratios (vs no CAD) for women were 1.36 (95% CI, 1.16-1.60) and 1.86 (1.61-2.16) for nonobstructive and obstructive CAD, respectively; corresponding hazard ratios for men were 1.24 (1.06-1.45) and 1.38 (1.20-1.59). After risk adjustment, 5-year mortality risk was higher in men than in women at all levels of CAD severity. The relationships between severity of CAD and mortality risk during long-term follow-up in women and men were similar to the 5-year relationships above. CONCLUSIONS: Although women undergoing elective catheterization have less severe CAD than men, nonobstructive CAD is prevalent in both sexes and carries a worse prognosis than no CAD. These data suggest a need for further investigation to establish optimal therapies for this at-risk group of patients with nonobstructive CAD.
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Doença da Artéria Coronariana/epidemiologia , Previsões , Medição de Risco , Idoso , Causas de Morte/tendências , Angiografia Coronária , Doença da Artéria Coronariana/diagnóstico , Feminino , Seguimentos , Humanos , Masculino , Pessoa de Meia-Idade , Morbidade/tendências , Prognóstico , Estudos Prospectivos , Sistema de Registros , Estudos Retrospectivos , Fatores de Risco , Distribuição por Sexo , Fatores Sexuais , Taxa de Sobrevida/tendências , Estados Unidos/epidemiologiaRESUMO
OBJECTIVE: To evaluate donor gamete utilization, patient satisfaction, and fertility treatment outcomes of patients pursuing treatment with donor gametes stratified by the desired race as well as ethnicity of the gamete donor. DESIGN: Survey study. SETTING: Clinic. PATIENT(S): Patients planning to undergo treatment using donor sperm and/or donor oocytes at a single academic fertility clinic in the Southeastern United States between 2015 and 2020. INTERVENTION(S): None. MAIN OUTCOME MEASURE(S): Utilization rates of donor gametes, satisfaction with donor gamete selection, and fertility treatment outcomes stratified by race and ethnicity of patient, as well as that of their gamete donor. RESULT(S): Four hundred fifty patients were eligible for inclusion and 170 (38%) responded to the survey. Among the respondents, 59% desired a non-Hispanic White gamete donor and 20% desired a non-Hispanic Black gamete donor. Patients seeking a non-Hispanic Black gamete donor had lower odds of using donor gametes (Odds ratio [OR], 0.13; 95% confidence interval [CI], 0.04-0.40) compared with individuals seeking a non-Hispanic White gamete donor. When evaluating satisfaction with donor gamete selection, patients seeking a non-Hispanic Black gamete donor reported lower satisfaction compared with individuals seeking a non-Hispanic White gamete donor (OR, 0.19; 95% CI, 0.09-0.43). When evaluating fertility outcomes, Non-Hispanic Black patients and those using non-Hispaninc Black gamete donors were found to have lower odds of successful conception compared with non-Hispanic White patients (OR, 0.18; 95% CI, 0.07-0.46) and individuals seeking non-Hispanic White gamete donors (OR, 0.26; 95% CI, 0.09-0.75), respectively. CONCLUSION(S): Patients seeking non-Hispanic Black donor gametes have lower utilization rates, less satisfaction with gamete donor selection, and lower odds of conception when compared with those seeking non-Hispanic White gamete donors. These findings highlight the need for more racial diversity within donor gamete banks, as well as within the donor pools available through agencies and fertility clinics.
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BACKGROUND: Patients with diabetes at risk of food insecurity face cost barriers to healthy eating and, as a result, poor health outcomes. Population health management strategies are needed to improve food security in real-world health system settings. We seek to test the effect of a prescription produce program, 'Eat Well' on cardiometabolic health and healthcare utilization. We will also assess the implementation of an automated, affirmative outreach strategy. METHODS: We will recruit approximately 2400 patients from an integrated academic health system in the southeastern United States as part of a two-arm parallel hybrid type 1 pragmatic randomized controlled trial. Patients with diabetes, at risk for food insecurity, and a recent hemoglobin A1c reading will be eligible to participate. The intervention arm receives, 'Eat Well', which provides a debit card with $80 (added monthly) for 12 months valid for fresh, frozen, or canned fruits and vegetables across grocery retailers. The control arm does not. Both arms receive educational resources with diabetes nutrition and self-management materials, and information on existing care management resources. Using an intent-to-treat analysis, primary outcomes include hemoglobin A1C levels and emergency department visits in the 12 months following enrollment. Reach and fidelity data will be collected to assess implementation. DISCUSSION: Addressing food insecurity, particularly among those at heightened cardiometabolic risk, is critical to equitable and effective population health management. Pragmatic trials provide important insights into the effectiveness and implementation of 'Eat Well' and approaches like it in real-world settings. REGISTRATION: ClinicalTrials.gov Identifier: NCT05896644; Clinical Trial Registration Date: 2023-06-09.
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Background: Contamination of work surfaces by used laryngoscopes after endotracheal intubation is a serious infection control concern but no strategies are available to address it. We assessed if contamination of the surfaces after endotracheal intubation would be reduced when providers used a dedicated, self-erected, disposable plastic sleeve (BladePouch) to store the used laryngoscope as compared to using single gloves or double gloves and sheathing the laryngoscope with the outer gloves. Methods: Twenty participants were recruited including attending physicians, trainees and allied health care professionals. They performed endotracheal intubation on a mannequin with oral cavity coated with a dye and stored the used laryngoscope blade using single gloves, double gloves or BladePouch. Each participant used both direct and video laryngoscopes. Following intubation, dye contamination of gloves, gown and work surface was evaluated. Results: There was no difference in the contamination of gloves or gowns between the single gloves, double gloves or BladePouch groups. However, work surface contamination was significantly reduced when using BladePouch compared to single or double gloves (13% vs. 100% vs. 80% respectively, P<0.001). The odds of work surface contamination were significantly lower with BladePouch vs. single or double gloves, even when adjusted for intubation device, role and experience of participants with an adjusted odds ratio of 0.0054 (95% confidence interval: 0.0009-0.0314), P<0.001. Conclusions: In conjunction with standard precautions, the use of a dedicated plastic sleeve to store contaminated laryngoscope blade after endotracheal intubation may reduce the work surface contamination, independent of intubation device, role and experience of providers.
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INTRODUCTION: Detection of adverse reactions to drugs and biologic agents is an important component of regulatory approval and post-market safety evaluation. Real-world data, including insurance claims and electronic health records data, are increasingly used for the evaluation of potential safety outcomes; however, there are different types of data elements available within these data resources, impacting the development and performance of computable phenotypes for the identification of adverse events (AEs) associated with a given therapy. OBJECTIVE: To evaluate the utility of different types of data elements to the performance of computable phenotypes for AEs. METHODS: We used intravenous immunoglobulin (IVIG) as a model therapeutic agent and conducted a single-center, retrospective study of 3897 individuals who had at least one IVIG administration between 1 January 2014 and 31 December 2019. We identified the potential occurrence of four different AEs, including two proximal AEs (anaphylaxis and heart rate alterations) and two distal AEs (thrombosis and hemolysis). We considered three different computable phenotypes: (1) an International Classification of Disease (ICD)-based phenotype; (2) a phenotype-based on EHR-derived contextual information based on structured data elements, including laboratory values, medication administrations, or vital signs; and (3) a compound phenotype that required both an ICD code for the AE in combination with additional EHR-derived structured data elements. We evaluated the performance of each of these computable phenotypes compared with chart review-based identification of AEs, assessing the positive predictive value (PPV), specificity, and estimated sensitivity of each computable phenotype method. RESULTS: Compound computable phenotypes had a high positive predictive value for acute AEs such as anaphylaxis and bradycardia or tachycardia; however, few patients had both ICD codes and the relevant contextual data, which decreased the sensitivity of these computable phenotypes. In contrast, computable phenotypes for distal AEs (i.e., thrombotic events or hemolysis) frequently had ICD codes for these conditions in the absence of an AE due to a prior history of such events, suggesting that patient medical history of AEs negatively impacted the PPV of computable phenotypes based on ICD codes. CONCLUSIONS: These data provide evidence for the utility of different structured data elements in computable phenotypes for AEs. Such computable phenotypes can be used across different data sources for the detection of infusion-related adverse events.
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Anafilaxia , Imunoglobulinas Intravenosas , Humanos , Imunoglobulinas Intravenosas/efeitos adversos , Estudos Retrospectivos , Registros Eletrônicos de Saúde , Hemólise , Fenótipo , AlgoritmosRESUMO
Importance: Health care workers (HCWs) have been experiencing substantial stress and burnout, and evidence-based mitigation strategies are needed. Transcendental Meditation (TM) is a mantra meditation practice with potential efficacy in reducing stress. Objective: To assess the efficacy of TM practice in reducing stress among HCWs over a 3-month period. Design, Setting, and Participants: This single-center open-label randomized clinical trial was conducted among HCWs at an academic medical center from November 19, 2020, to August 31, 2021. Inclusion criteria comprised a score of 6 points or greater on the Subjective Units of Distress Scale and an increase of 5% or greater in baseline heart rate or an increase of 33% or greater in galvanic skin response after exposure to a stressful script. Exclusion criteria included the use of antipsychotic or ß blocker medications, current suicidal ideation, or previous TM training. Of 213 HCWs who participated in prescreening, 95 attended in-person visits, resulting in 80 eligible participants who were randomized to receive a TM intervention (TM group) or usual treatment (control group). Interventions: The TM group practiced TM for 20 minutes twice daily over a 3-month period. The control group received usual treatment, which consisted of access to wellness resources. Main Outcomes and Measures: The primary outcome was change in acute psychological distress measured by the Global Severity Index. Secondary outcomes included changes in burnout (measured by the Maslach Burnout Inventory), insomnia (measured by the Insomnia Severity Index), and anxiety (measured by the Generalized Anxiety Disorder-7 scale). Results: Among 80 participants, 66 (82.5%) were women, with a mean (SD) age of 40 (11) years. One participant (1.3%) was American Indian or Alaska Native, 5 (6.3%) were Asian, 12 (15.0%) were Black, 59 (73.8%) were White, and 3 (3.8%) were of unknown or unreported race; 4 participants (5.0%) were Hispanic, and 76 (95.0%) were non-Hispanic. A total of 41 participants were randomized to the TM group, and 39 were randomized to the control group. Participants in the TM group did not show a statistically significant decrease in psychological distress on the Global Severity Index compared with those in the control group (-5.6 points vs -3.8 points; between-group difference, -1.8 points; 95% CI, -4.2 to 0.6 points; P = .13). Compared with the control group, the TM group had significantly greater reductions in the secondary end points of emotional exhaustion (Maslach Burnout Inventory subscore: -8.0 points vs -2.6 points; between-group difference, -5.4 points; 95% CI, -9.2 to -1.6 points; P = .006), insomnia (Insomnia Severity Scale score: -4.1 points vs -1.9 points; between-group difference, -2.2 points; 95% CI, -4.4 to 0 points; P = .05), and anxiety (Generalized Anxiety Disorder-7 score: -3.1 points vs -0.9 points; between-group difference, -2.2 points; 95% CI, -3.8 to -0.5; P = .01) at 3 months. A total of 38 participants (92.7%) in the TM group adhered to home practice. Conclusions and Relevance: In this randomized clinical trial, TM practice among HCWs over a 3-month period did not result in a statistically significant reduction in the primary outcome of acute psychological distress compared with usual treatment but significantly improved the secondary outcomes of burnout, anxiety, and insomnia. These findings suggest that TM may be a safe and effective strategy to alleviate chronic stress among HCWs. Trial Registration: ClinicalTrials.gov identifier: NCT04632368.
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Antipsicóticos , Esgotamento Profissional , Meditação , Distúrbios do Início e da Manutenção do Sono , Adulto , Esgotamento Profissional/prevenção & controle , Feminino , Pessoal de Saúde , Humanos , MasculinoRESUMO
OBJECTIVE: For the diagnosis of COVID-19, the yield of nasopharyngeal (NP) swabs is unclear, and bronchoalveolar lavage (BAL) is obtained to confirm the diagnosis. We assessed the utilisation of bronchoscopy for COVID-19 diagnosis in a multicenter study and compared the diagnostic yield of BAL versus NP swabs. METHODS: This retrospective study included all patients who were admitted with clinical presentation concerning for COVID-19 and underwent BAL from 1 March to 31 July 2020 at four tertiary care centres in North America. We also compared concordance of BAL with NP swabs for diagnosis of COVID-19 infection. RESULTS: Fifty-three patients, with clinical suspicion for COVID-19 and admitted for respiratory failure, underwent bronchoscopy to collect BAL for SARS-CoV-2 testing. During the same period, 2039 bronchoscopies were performed on patients not infected with COVID-19. Of 42 patients with NP swabs and BAL collected within ≤7 days, 1 was NP swab negative but positive by BAL for SARS-CoV-2 (n=1/42 (2.4%)). Across a wide array of testing platforms, the overall agreement between NP swabs and BAL results was 97.6% (95% CI: 93.0% to 100%) with Cohen's k of 0.90 (95% CI: 0.69 to 1.00). The sensitivity, specificity, positive and negative predictive values of NP swabs compared with BAL were 83.3% (95% CI: 53.5% to 100%), 100%, 100% and 97.3% (95% CI: 92.1% to 100%), respectively. CONCLUSIONS: BAL was used infrequently to assess COVID-19 in busy institutions. NP swabs have a high concordance with BAL for COVID-19 testing, but negative NP swabs should be confirmed with BAL when clinical suspicion is high.
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Líquido da Lavagem Broncoalveolar/virologia , Broncoscopia/estatística & dados numéricos , COVID-19/diagnóstico , SARS-CoV-2/isolamento & purificação , Idoso , Teste para COVID-19 , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Nasofaringe/virologia , América do Norte , Valor Preditivo dos Testes , Estudos RetrospectivosRESUMO
We assessed the effect of implementing a protocol-directed strategy to determine when patients can be liberated from venovenous extracorporeal membrane oxygenation on extracorporeal membrane oxygenation duration, time to initiation of first sweep-off trial, duration of mechanical ventilation, ICU length of stay, hospital length of stay, and survival to hospital discharge. DESIGN: Single-center retrospective before and after study. SETTING: The medical ICU at an academic medical center. PATIENTS: One-hundred eighty patients with acute respiratory distress syndrome managed with venovenous extracorporeal membrane oxygenation at a single institution from 2013 to 2019. INTERVENTIONS: In 2016, our institution implemented a daily assessment of readiness for a trial off extracorporeal membrane oxygenation sweep gas ("sweep-off trial"). When patients met prespecified criteria, the respiratory therapist performed a sweep-off trial to determine readiness for discontinuation of venovenous extracorporeal membrane oxygenation. MEASUREMENTS AND MAIN RESULTS: Sixty-seven patients were treated before implementation of the sweep-off trial protocol, and 113 patients were treated after implementation. Patients managed using the sweep-off trial protocol had a significantly shorter extracorporeal membrane oxygenation duration (5.5 d [3-11 d] vs 11 d [7-15.5 d]; p < 0.001), time to first sweep-off trial (2.5 d [1-5 d] vs 7.0 d [5-11 d]; p < 0.001), duration of mechanical ventilation (15.0 d [9-31 d] vs 25 d [21-33 d]; p = 0.017), and ICU length of stay (18 d [10-33 d] vs 27.0 d [21-36 d]; p = 0.008). There were no observed differences in hospital length of stay or survival to hospital discharge. CONCLUSIONS: In patients with acute respiratory distress syndrome managed with venovenous extracorporeal membrane oxygenation at our institution, implementation of a daily, respiratory therapist assessment of readiness for a sweep-off trial was associated with a shorter time to first sweep-off trial and shorter duration of extracorporeal membrane oxygenation. Among survivors, the postassessment group had a reduced duration of mechanical ventilation and ICU lengths of stay. There were no observed differences in hospital length of stay or inhospital mortality.
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Unplanned hospital readmissions represent a significant health care value problem with high costs and poor quality of care. A significant percentage of readmissions could be prevented if clinical inpatient teams were better able to predict which patients were at higher risk for readmission. Many of the current clinical decision support models that predict readmissions are not configured to integrate closely with the electronic health record or alert providers in real-time prior to discharge about a patient's risk for readmission. We report on the implementation and monitoring of the Epic electronic health record-"Unplanned readmission model version 1"-over 2 years from 1/1/2018-12/31/2019. For patients discharged during this time, the predictive capability to discern high risk discharges was reflected in an AUC/C-statistic at our three hospitals of 0.716-0.760 for all patients and 0.676-0.695 for general medicine patients. The model had a positive predictive value ranging from 0.217-0.248 for all patients. We also present our methods in monitoring the model over time for trend changes, as well as common readmissions reduction strategies triggered by the score.
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International variability in infarct size following acute anterior ST-elevation myocardial infarction without shock treated with primary percutaneous coronary intervention (PCI) has been little studied. Patients enrolled in the Counterpulsation to Reduce Infarct Size pre-PCI for Acute Myocardial Infarction international randomized trial were analyzed according to their region of enrollment: United States (US) (nâ¯=â¯60), Europe/Australia (EU/AU) (nâ¯=â¯104), or India (nâ¯=â¯123). Cardiac magnetic resonance imaging was performed 3-5 days after PCI to assess infarct size, expressed as percentage of left ventricular mass, and analyzed by an imaging core laboratory. The relation between infarct size and region was modelled using multivariable linear regression adjusting for time from symptom onset to first hospital contact, myocardial infarction severity, and baseline characteristics. Infarct size was 36.4% of left ventricular mass in US patients (95% confidence interval [CI] 31.5 to 41.4), 36.5% (95% CI 32.6 to 40.4) in EU/AU patients, and 44.7% (95% CI 41.1 to 48.2) in patients from India (pâ¯=â¯0.01). In multiplicity-adjusted regression analysis, mean infarct size in patients from India was higher than in US patients (mean difference of 8.3%; 95% CI 0.7 to 15.8; pâ¯=â¯0.03), and EU/AU patients (mean difference of 8.2%; 95% CI 1.6 to 14.8; pâ¯=â¯0.01). There was no significance difference in infarct size between patients from the EU/AU and the US (mean difference of 0.1%; 95% CI -7.5 to 7.4; pâ¯=â¯0.99). ln conclusion, in patients with anterior ST-elevation myocardial infarction without cardiogenic shock treated with primary PCI, infarct size was larger in India compared to the United States and EU/AU, even after adjustment for performance metrics, including time to treatment, and other potential confounders. Future studies are needed to better elucidate this discrepancy.
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Infarto Miocárdico de Parede Anterior/diagnóstico , Imagem Cinética por Ressonância Magnética/métodos , Miocárdio/patologia , Intervenção Coronária Percutânea/métodos , Idoso , Infarto Miocárdico de Parede Anterior/epidemiologia , Infarto Miocárdico de Parede Anterior/cirurgia , Austrália/epidemiologia , Angiografia Coronária , Europa (Continente)/epidemiologia , Feminino , Seguimentos , Humanos , Incidência , Índia/epidemiologia , Masculino , Pessoa de Meia-Idade , Prognóstico , Estudos Retrospectivos , Índice de Gravidade de Doença , Taxa de Sobrevida/tendências , Estados Unidos/epidemiologiaRESUMO
Greater optimism regarding recovery from chronic illness is associated with improved quality of life and clinical outcomes. We performed a post-hoc analysis on the association between optimism and outcomes in Ranolazine in Patients with Incomplete Revascularization after Percutaneous Coronary Intervention (RIVER-PCI), a randomized trial in patients with chronic angina pectoris who had incomplete revascularization following percutaneous coronary intervention. At baseline, patients answered how much they agreed with the phrase, "I am optimistic about my future and returning to a normal lifestyle." We evaluated the association between baseline optimism and time to ischemia-driven hospitalization or revascularization using a Cox model, and the association between baseline optimism and change in frequency of angina pectoris using a mixed measures model. Of 2,389 patients, 782 (33.2%) were very optimistic ("strongly agree"), 1,000 (42.4%) were optimistic ("agree"), 451 (19.1%) were neutral ("undecided"), and 123 (5.2%) were not optimistic ("disagree" or "strongly disagree"). Very optimistic patients had a lower prevalence of co-morbidities and less severe angina at baseline than less optimistic patients. The rate of ischemia-driven revascularization or hospitalization was higher in neutral and not optimistic patients compared with very optimistic patients; this finding persisted after adjustment for co-morbidities and baseline angina frequency (hazard ratio 1.42, 95% confidence interval 1.14 to 1.77 for neutral vs very optimistic; hazard ratio 1.38, 95% confidence interval 0.98 to 1.94 for not optimistic vs very optimistic). Neutral and not optimistic patients also had less improvement in angina than very optimistic patients. In conclusion, in patients with angina, those with more self-reported optimism had better health status outcomes. Whether structured interventions targeting optimism improve outcomes in these patients warrants further study.
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Angina Pectoris/terapia , Nível de Saúde , Revascularização Miocárdica/métodos , Qualidade de Vida , Ranolazina/uso terapêutico , Idoso , Angina Pectoris/epidemiologia , Angina Pectoris/psicologia , Doença Crônica , Método Duplo-Cego , Feminino , Seguimentos , Humanos , Masculino , Pessoa de Meia-Idade , Morbidade/tendências , Prognóstico , Bloqueadores dos Canais de Sódio/uso terapêutico , Estados Unidos/epidemiologiaRESUMO
In pulmonary hypertension (PH), measurement of various echocardiographic parameters that assess right heart function is recommended by current clinical guidelines. Limited data exists on the combined value of clinical and echocardiographic parameters in precapillary PH in the modern era of therapy. We examined the association of clinical and echocardiographic parameters with surrogate outcomes (6-minute walk distance) and hard outcomes (hospitalization or death) in patients with precapillary PH. A cohort of patients with an established diagnosis of precapillary PH who underwent transthoracic echocardiography at the Duke Echo Lab were prospectively enrolled from 2010 to 2014. Univariable and multivariable models were constructed to examine the relation of clinical and echocardiographic parameters with surrogate and hard outcomes. Of the 98 patients with analyzable echocardiograms with good image quality, 85 were woman, mean age was 59.4 years, and 47% had ≥World Health Organization functional class III symptoms. The mean 6-minute walk distance was 354(±132) m, and 83% were on pulmonary arterial hypertension medications. At 24 months, the cumulative incidence rate for hospitalization or death was 47%. In univariable analyses, the REVEAL (Registry to Evaluate Early and Long-term PAH Disease Management) risk score (HR 1.72 per 1 SD (2.81) increment, 95% CI 1.34, 2.22; p=<0.001), RV global longitudinal strain (RVGLS) (HR 1.54 per 1 SD (5.31) worsening, 95% CI , 2.12; p=0.008) and log-2 NT proBNP (HR 1.43 per 1-fold increase, 95% CI 1.25, 1.63; p=<0.001) were significantly associated with hospitalization or death.