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 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.

Single-center prognostic validation of the risk assessment of the 2015 ESC/ERS guidelines in patients with pulmonary arterial hypertension in Japan

2020 ◽  
Vol 98 (9) ◽  
pp. 653-658 ◽  
Author(s):  
Ryo Imai ◽  
Shiro Adachi ◽  
Masahiro Yoshida ◽  
Shigetake Shimokata ◽  
Yoshihisa Nakano ◽  
...  
Keyword(s):  
Risk Assessment ◽  
Pulmonary Arterial Hypertension ◽  
High Risk ◽  
Arterial Hypertension ◽  
Risk Group ◽  
Low Risk ◽  
World Health ◽  
Right Atrial ◽  
Walking Distance ◽  
Pulmonary Arterial

The 2015 European Society of Cardiology/European Respiratory Society guidelines for the diagnosis and treatment of pulmonary hypertension include a multidimensional risk assessment for patients with pulmonary arterial hypertension (PAH). However, prognostic validations of this risk assessment are limited, especially outside Europe. Here, we validated the risk assessment strategy in PAH patients in our institution in Japan. Eighty consecutive PAH patients who underwent right heart catheterization between November 2006 and December 2018 were analyzed. Patients were classified as low, intermediate, or high risk by using a simplified version of the risk assessment that included seven variables: World Health Organization functional class, 6-min walking distance, peak oxygen consumption, brain natriuretic peptide, right atrial pressure, mixed venous oxygen saturation, and cardiac index. The high-risk group showed significantly higher mortality than the low- or intermediate-risk group at baseline (P < 0.001 for both comparisons), and the mortalities in the intermediate- and low-risk groups were both low (P = 0.989). At follow-up, patients who improved to or maintained a low-risk status showed better survival than those who did not (P = 0.041). Our data suggest that this risk assessment can predict higher mortality risk and long-term survival in PAH patients in Japan.

Incorporation of renal function in mortality risk assessment for pulmonary arterial hypertension

2020 ◽  
Vol 39 (7) ◽  
pp. 675-685 ◽  
Author(s):  
Jason G.E. Zelt ◽  
Alomgir Hossain ◽  
Louise Y. Sun ◽  
Sanjay Mehta ◽  
George Chandy ◽  
...  
Keyword(s):  
Risk Assessment ◽  
Renal Function ◽  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Mortality Risk ◽  
Pulmonary Arterial

scholarly journals Risk Assessment in Pulmonary Arterial Hypertension Patients: The Long and Short of it

2018 ◽  
Vol 16 (3) ◽  
pp. 125-135 ◽  
Author(s):  
Raymond L. Benza ◽  
Lisa Carey Lohmueller ◽  
Jidapa Kraisangka ◽  
Manreet Kanwar
Keyword(s):  
Risk Factors ◽  
Risk Assessment ◽  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Disease Progression ◽  
Risk Prediction ◽  
Response To Treatment ◽  
Pulmonary Vasculature ◽  
Term Survival ◽  
Pulmonary Arterial

Pulmonary arterial hypertension (PAH) is a chronic and rapidly progressive disease that is characterized by extensive narrowing of the pulmonary vasculature, leading to increases in pulmonary vascular resistance, subsequent right ventricular dysfunction, and eventual death. There are currently multiple approved drugs—developed as single or combination therapies in the last few years—that have improved outcome and functionality in PAH. However, despite improvement in short-term survival with these new effective therapies, PAH remains an incurable disease with a median survival of 7 years (Figure 1).1 This chronic disease state may be characterized by morbid events such as hospitalizations that herald rapid disease progression and account for a significant disease burden (Figure 2).23 Physician ability to predict PAH disease progression is critical for determining optimal care of patients. Accurate risk assessment allows clinicians to determine the patient's prognosis, identify treatment goals, and monitor disease progression and the patient's response to treatment. Risk assessment for PAH patients should include a range of clinical, hemodynamic, and exercise parameters, performed in a serial fashion over the treatment course. Patient risk stratification can also help physicians better allocate treatment resources in settings where they are scarce. If widely adopted, risk prediction can enhance the consistency of treatment approaches across PAH practitioners and improve the timeliness of referral for lung transplantation. Hence, along with advancing PAH treatment options, comprehensive risk prediction is essential to make individualized treatment decisions in the current treatment era. Several tools are currently available for assessing risk in PAH (Figure 3). These include the 2015 European Society of Cardiology/European Respiratory Society pulmonary hypertension guidelines' risk variables,4 the French registry equation,5 the National Institutes of Health risk equation,6 or a risk score such as the one derived from the Registry to Evaluate Early And Long-term PAH Disease Management.1 These registries and evaluations of clinical trial sets have provided important insights into the importance of both modifiable (eg, 6-minute walk distance, functional class, brain natriuretic peptide, and nonmodifiable (eg, age, gender, PAH etiology) risk factors that predict survival. The following review explores commonly cited risk factors, both modifiable and nonmodifiable, and their implications for patient outcomes.

Risk assessment in pulmonary arterial hypertension (PAH): Insights from the GRIPHON study

2019 ◽  
Author(s):  
HA Ghofrani ◽  
O Sitbon ◽  
K Chin ◽  
R Channick ◽  
L Di Scala ◽  
...  
Keyword(s):  
Risk Assessment ◽  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Pulmonary Arterial

scholarly journals Study of the effect of Occlutech Atrial Flow Regulator on symptoms, hemodynamics, and echocardiographic parameters in advanced pulmonary arterial hypertension

2021 ◽  
Vol 11 (1) ◽  
pp. 204589402198996
Author(s):  
Kothandam Sivakumar ◽  
Gopalavilasam R. Rohitraj ◽  
Monica Rajendran ◽  
Nithya Thivianathan
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
High Risk ◽  
Arterial Hypertension ◽  
Right Heart Failure ◽  
Right Atrial ◽  
Echocardiographic Parameters ◽  
Procedural Complications ◽  
Pulmonary Arterial ◽  
Flow Regulator ◽  
Risk Predictors

Optimal sized balloon atrial septostomy improves hemodynamics in advanced pulmonary arterial hypertension. Occlutech Atrial Flow Regulator is designed to provide an atrial septal fenestration diameter titrated according to the age and right atrial pressures. This observational study analyzed symptoms, exercise distance, oxygen saturations, hemodynamics and echocardiographic parameters after Atrial Flow Regulator implantation in patients with syncope or right-heart failure. Patients with high-risk predictors of mortality during septostomy were scrutinized. Thirty-nine patients (9 children) with syncope (34/39) or right-heart failure (27/39) underwent Atrial Flow Regulator implantation without procedural complications. Six-minute walk distance increased from 310 ± 158.2 to 376.4 ± 182.6 m, none developed syncope. Oxygen saturations reduced from 96.4 ± 6.4% to 92 ± 4.9% at rest and further to 80.3 ± 5.9% on exercise. Right atrial pressures reduced from 9.4 ± 5 (2–27) mmHg to 6.9 ± 2.6 (1–12) mmHg, while cardiac index increased from 2.4 ± 0.8 (0.98–4.3) to 3 ± 1 (1.1–5.3) L/min/m2 and systemic oxygen transport increased from 546.1 ± 157.9 (256.2–910.5) to 637.2 ± 191.1 (301.3–1020.2) ml/min. Echocardiographic improvement included significant reduction of pericardial effusion and inferior caval congestion at a median follow-up of 37 months. Overall survival improved except two early and one late deaths in high-risk patients. Five of seven patients with advanced disease and key hemodynamic predictors of mortality survived. Acute hemodynamic benefits in pulmonary arterial hypertension after Atrial Flow Regulator were improved cardiac output, systemic oxygen transport, and reduced right atrial pressures. Improvement of symptoms especially syncope, exercise duration, and right ventricular systolic function as well as device patency were sustained on mid-term follow-up. Implantation was safe in all including young children without procedural complications. Mortality was noted only in patients who had high-risk predictors and patients at advanced stage of the disease.

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