scholarly journals Prognostication in Pulmonary Arterial Hypertension and Use of Current Risk Prediction Models

2015 ◽  
Vol 14 (1) ◽  
pp. 37-43 ◽  
Author(s):  
Richa Agarwal
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Risk Prediction ◽  
Patient Outcomes ◽  
Prediction Models ◽  
Risk Prediction Models ◽  
Patient Demographics ◽  
Target Individual ◽  
Individualized Approach ◽  
Pulmonary Arterial

Significant therapeutic advances in the field of pulmonary arterial hypertension (PAH), increased awareness and diagnosis, and changing patient demographics in the contemporary era have facilitated the development of better prognostic tools for predicting survival. However, overall patient outcomes remain poor, and measurement of most prognostic factors still occurs at the time of initial PAH diagnosis or enrollment into clinical trials. Treatment of PAH patients requires an individualized approach based on disease severity and burden of risk factors to improve patient outcomes. This article will focus on the use of risk prediction models to map and target individual disease trajectories to avoid future morbid and mortal events.

scholarly journals The angiostatic peptide endostatin enhances mortality risk prediction in pulmonary arterial hypertension

2021 ◽  
pp. 00378-2021
Author(s):  
Catherine E. Simpson ◽  
Megan Griffiths ◽  
Jun Yang ◽  
Melanie K. Nies ◽  
R. Dhananjay Vaidya ◽  
...  
Keyword(s):  
Risk Assessment ◽  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Risk Prediction ◽  
Mortality Risk ◽  
Prediction Models ◽  
Independent Predictor ◽  
Right Atrial ◽  
Risk Prediction Models ◽  
Pulmonary Arterial

Currently available noninvasive markers for assessing disease severity and mortality risk in pulmonary arterial hypertension (PAH) are unrelated to fundamental disease biology. Endostatin, an angiostatic peptide known to inhibit pulmonary artery endothelial cell migration, proliferation, and survival in vitro, has been linked to adverse hemodynamics and shortened survival in small PAH cohorts. This observational cohort study sought to assess 1) the prognostic performance of circulating endostatin levels in a large, multicenter PAH cohort, and 2) the added value gained by incorporating endostatin into existing PAH risk prediction models.Endostatin ELISAs were performed on enrollment samples collected from 2017 PAH subjects with detailed clinical data, including survival times. Endostatin associations with clinical variables, including survival, were examined using multivariable regression and Cox proportional hazards models. Extended survival models including endostatin were compared to null models based on the REVEAL risk prediction tool and ESC/ERS low risk criteria using likelihood ratio tests, Akaike and Bayesian information criteria, and C-statistics.Higher endostatin was associated with higher right atrial pressure, mean pulmonary arterial pressure, and pulmonary vascular resistance and with shorter six-minute walk distance (p<0.01). Mortality risk doubled for each log-higher endostatin (hazard ratio 2.3, 95% confidence interval 1.6 to 3.4, p<0.001). Endostatin remained an independent predictor of survival when incorporated into existing risk prediction models. Adding endostatin to REVEAL-based and ESC/ERS criteria-based risk assessment strategies improved mortality risk prediction.Endostatin is a robust, independent predictor of mortality in PAH. Adding endostatin to existing PAH risk prediction strategies improves PAH risk assessment.

scholarly journals EXPRESS: Improving Communication Between Healthcare Providers and Pulmonary Arterial Hypertension Patients: A Survey of Patient Preferences

2021 ◽  
pp. 204589402110158
Author(s):  
Jacqueline Brewer ◽  
Mary Bartlett ◽  
David Harris ◽  
Christine C Hui
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Patient Outcomes ◽  
Patient Preferences ◽  
Treatment Options ◽  
Healthcare Providers ◽  
Effective Communication ◽  
Item Questionnaire ◽  
Important Health ◽  
Pulmonary Arterial

Effective communication within the patient-provider relationship is a key aspect of shared decision-making and associated with several positive patient outcomes. Although previous studies suggest that patients’ and providers’ conceptualization of what constitutes effective communication differ, there is no available literature discussing patient preferences for communication. The objective of this study was to determine the words and phrases pulmonary arterial hypertension (PAH) patients prefer to hear when discussing their disease with their physician. A total of 227 PAH patients completed a survey that included a 20-item questionnaire specifically designed to assess patient preference for words and phrases when discussing their disease and treatment; statistically significant differences were observed across all items of the questionnaire. Patients preferred their physician ask them how they have “been feeling” (63%) rather than how they have “been doing”. In addition, 96% of patients indicated that they wanted to hear that this is “…the best medicine for you” rather than this is“ the best medicine.” Considerably more patients (60%) indicated they want their physician to say, “We want you to have fewer symptoms” rather than “We want you to feel more normal.” They also indicated they wanted the “most effective” medicine (82%) rather than the “most aggressive” medicine (7%). The results of this study suggest that PAH patients have strong preferences for the language their providers use when discussing their disease and treatment options. Given the role that effective communication has on important health outcomes, PAH providers need to consider these findings when communicating with patients.

Patient Outcomes in Pulmonary Arterial Hypertension With Nonclassic Response to Acute Vasodilator Testing

CHEST Journal ◽  
2013 ◽  
Vol 144 (4) ◽  
pp. 870A
Author(s):  
Stephen Halliday ◽  
Anna Hemnes ◽  
Ivan Robbins ◽  
Evan Brittain
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Patient Outcomes ◽  
Pulmonary Arterial

scholarly journals Prediction of organ involvement and survival in systemic sclerosis patients in the first 5 years from diagnosis

2019 ◽  
Vol 5 (1) ◽  
pp. 57-65 ◽  
Author(s):  
Wieneke MT van den Hombergh ◽  
Hanneke KA Knaapen-Hans ◽  
Frank HJ van den Hoogen ◽  
Patricia Carreira ◽  
Oliver Distler ◽  
...  
Keyword(s):  
Systemic Sclerosis ◽  
Pulmonary Arterial Hypertension ◽  
Interstitial Lung Disease ◽  
Arterial Hypertension ◽  
Lung Disease ◽  
Vital Capacity ◽  
Operating Characteristic ◽  
Prediction Models ◽  
Organ Involvement ◽  
Pulmonary Arterial

Background: Organ involvement often occurs in early systemic sclerosis and has been related to premature death. Identifying patients at diagnosis at risk of developing early organ involvement would be useful to optimize screening and management strategies. Objective: To develop prediction models for the 5-year development of interstitial lung disease, pulmonary arterial hypertension and death. Methods: A European multicentre inception cohort was created. For modelling, predefined clinical variables with known predictive value at diagnosis were used. Univariate and multivariate regression analysis were done to select baseline predictors and build the prediction models. The models were tested using the area under the receiver operating characteristic curve comparing observed and expected frequencies. Results: Of 735 patients, 23% developed interstitial lung disease, 8% developed pulmonary arterial hypertension 12% died. The interstitial lung disease model included diffuse cutaneous systemic sclerosis (OR = 1.8), systemic sclerosis disease duration < 3 years (OR = 1.4), puffy fingers (OR = 1.6), and anti-topoisomerase-I-antibodies (OR = 1.8). The pulmonary arterial hypertension model included age > 65 years (OR = 3.2), forced vital capacity < 70% (OR = 2.5) and diffusing capacity of the lung for carbon monoxide < 55% (OR = 1.9). Death was predicted best by age > 65 years (OR = 4.1), male gender (OR = 1.9), no anti-centromere antibodies (OR = 0.5), proteinuria (OR = 1.9), forced vital capacity < 70% (OR = 1.8) and pulmonary arterial hypertension at diagnosis (OR = 10.1). The area under the receiver operating characteristic was 0.66 (95% CI 0.64–0.67), 0.66 (95% CI 0.64–0.68) and 0.70 (95% CI 0.69–0.72), respectively. Conclusion: We have shown that it is possible to predict interstitial lung disease, pulmonary arterial hypertension and death using established variables already available at the moment of systemic sclerosis diagnosis. Discriminatory performance of the models was suboptimal. Further research including new variables is necessary to improve performance.

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