Skip to main content
Top
Published in: Advances in Therapy 9/2019

Open Access 01-09-2019 | Hypertension | Original Research

Assessment of Risk of Disease Progression in Pulmonary Arterial Hypertension: Insights from an International Survey of Clinical Practice

Authors: Jonathan E. Simons, Elena B. Mann, Adam Pierozynski

Published in: Advances in Therapy | Issue 9/2019

Login to get access

Abstract

Introduction

Current guidelines for the management of pulmonary arterial hypertension (PAH) recommend regular multi-parametric assessment of a patient’s risk of clinical worsening or death, with the goal of achieving/maintaining a low-risk status. This international survey investigated how physicians currently assess risk and compared their clinical gestalt (judgement of risk) with the risk calculated using an algorithm based on the European Society of Cardiology (ESC)/European Respiratory Society (ERS) pulmonary hypertension guidelines and demonstrated to provide accurate mortality estimates.

Methods

PAH-treating physicians from Europe and the United States were surveyed on (1) how frequently they evaluated the recommended risk-assessment parameters and (2) to complete patient record forms (PRFs) with details on 5–7 recent adult PAH patients receiving treatment. For each PRF, physicians provided (1) their gestalt judgement of current risk and (2) details of the risk-assessment parameters measured. In accordance with the published method, measurements for ≥ 2 (of 6 selected) variables were required to calculate risk. Each variable was assigned a score of 1, 2 or 3 according to whether the measurement was within the thresholds for the low-, intermediate- or high-risk categories, as defined in the ESC/ERS guidelines. The average score represented the patient’s calculated risk.

Results

In total, 90 physicians (52 cardiologists, 38 pulmonologists) completed the survey, providing a total of 623 PRFs; of these, 365 (59%) included ≥ 2 measurements required to calculate risk. Among these patients, the percentages assigned to low-, intermediate- and high-risk categories based on gestalt/calculation were 32%/15%, 45%/68% and 22%/17%, respectively. Overall, there was concordance between the gestalt and calculated risk category for 45% of patients. The greatest level of disparity was for patients judged to be at low risk, where 80% were assigned to higher risk categories based on their calculated risk.

Conclusions

The results of this survey demonstrate that multi-parametric risk assessment is being performed in clinical practice, but not always to the extent recommended in the current guidelines. Further study on the utility of more regular measurement is required.

Funding

Actelion Pharmaceuticals Ltd.
Appendix
Available only for authorised users
Literature
1.
go back to reference Farber HW, Miller DP, Poms AD, et al. Five-year outcomes of patients enrolled in the REVEAL Registry. Chest. 2015;148(4):1043–54.CrossRefPubMed Farber HW, Miller DP, Poms AD, et al. Five-year outcomes of patients enrolled in the REVEAL Registry. Chest. 2015;148(4):1043–54.CrossRefPubMed
2.
go back to reference Pulido T, Channick RN, Delcroix M, et al. Long-term survival and safety with macitentan in patients with pulmonary arterial hypertension: results from the SERAPHIN Study and its open-label extension. Am J Respir Crit Care Med. 2017;195:A2294. Pulido T, Channick RN, Delcroix M, et al. Long-term survival and safety with macitentan in patients with pulmonary arterial hypertension: results from the SERAPHIN Study and its open-label extension. Am J Respir Crit Care Med. 2017;195:A2294.
3.
go back to reference Galiè N, Gaine S, Channick R, et al. Long-term survival and safety with selexipag in patients with pulmonary arterial hypertension: results from the GRIPHON study and its open-label extension. Eur Heart J. 2018;39:631–2.CrossRef Galiè N, Gaine S, Channick R, et al. Long-term survival and safety with selexipag in patients with pulmonary arterial hypertension: results from the GRIPHON study and its open-label extension. Eur Heart J. 2018;39:631–2.CrossRef
4.
go back to reference D’Alonzo GE, Barst RJ, Ayres SM, et al. Survival in patients with primary pulmonary hypertension. Results from a national prospective registry. Ann Intern Med. 1991;115(5):343–9.CrossRefPubMed D’Alonzo GE, Barst RJ, Ayres SM, et al. Survival in patients with primary pulmonary hypertension. Results from a national prospective registry. Ann Intern Med. 1991;115(5):343–9.CrossRefPubMed
5.
go back to reference Thenappan T, Ormiston ML, Ryan JJ, Archer SL. Pulmonary arterial hypertension: pathogenesis and clinical management. BMJ. 2018;360:j5492.CrossRefPubMed Thenappan T, Ormiston ML, Ryan JJ, Archer SL. Pulmonary arterial hypertension: pathogenesis and clinical management. BMJ. 2018;360:j5492.CrossRefPubMed
6.
7.
8.
go back to reference Galiè N, Humbert M, Vachiery JL, et al. 2015 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension: the Joint Task Force for the Diagnosis and Treatment of Pulmonary Hypertension of the European Society of Cardiology (ESC) and the European Respiratory Society (ERS): Endorsed by: Association for European Paediatric and Congenital Cardiology (AEPC), International Society for Heart and Lung Transplantation (ISHLT). Eur Respir J. 2015;46(4):903–75.CrossRefPubMed Galiè N, Humbert M, Vachiery JL, et al. 2015 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension: the Joint Task Force for the Diagnosis and Treatment of Pulmonary Hypertension of the European Society of Cardiology (ESC) and the European Respiratory Society (ERS): Endorsed by: Association for European Paediatric and Congenital Cardiology (AEPC), International Society for Heart and Lung Transplantation (ISHLT). Eur Respir J. 2015;46(4):903–75.CrossRefPubMed
9.
go back to reference Galiè N, Humbert M, Vachiery JL, et al. 2015 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension: the Joint Task Force for the Diagnosis and Treatment of Pulmonary Hypertension of the European Society of Cardiology (ESC) and the European Respiratory Society (ERS): Endorsed by: Association for European Paediatric and Congenital Cardiology (AEPC), International Society for Heart and Lung Transplantation (ISHLT). Eur Heart J. 2016;37(1):67–119.CrossRefPubMed Galiè N, Humbert M, Vachiery JL, et al. 2015 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension: the Joint Task Force for the Diagnosis and Treatment of Pulmonary Hypertension of the European Society of Cardiology (ESC) and the European Respiratory Society (ERS): Endorsed by: Association for European Paediatric and Congenital Cardiology (AEPC), International Society for Heart and Lung Transplantation (ISHLT). Eur Heart J. 2016;37(1):67–119.CrossRefPubMed
10.
go back to reference Benza RL, Gomberg-Maitland M, Miller DP, et al. The REVEAL registry risk score calculator in patients newly diagnosed with pulmonary arterial hypertension. Chest. 2012;141(2):354–62.CrossRefPubMed Benza RL, Gomberg-Maitland M, Miller DP, et al. The REVEAL registry risk score calculator in patients newly diagnosed with pulmonary arterial hypertension. Chest. 2012;141(2):354–62.CrossRefPubMed
11.
go back to reference Benza RL, Gomberg-Maitland M, Elliott CG, et al. Predicting Survival in Patients with Pulmonary Arterial Hypertension: The REVEAL Risk Score Calculator 2.0 and Comparison with ESC/ERS-Based Risk Assessment Strategies. Chest. 2019;S0012-3692(19):30152–7. Benza RL, Gomberg-Maitland M, Elliott CG, et al. Predicting Survival in Patients with Pulmonary Arterial Hypertension: The REVEAL Risk Score Calculator 2.0 and Comparison with ESC/ERS-Based Risk Assessment Strategies. Chest. 2019;S0012-3692(19):30152–7.
12.
go back to reference Kylhammar D, Kjellstrom B, Hjalmarsson C, et al. A comprehensive risk stratification at early follow-up determines prognosis in pulmonary arterial hypertension. Eur Heart J. 2018;39(47):4175–81.CrossRefPubMed Kylhammar D, Kjellstrom B, Hjalmarsson C, et al. A comprehensive risk stratification at early follow-up determines prognosis in pulmonary arterial hypertension. Eur Heart J. 2018;39(47):4175–81.CrossRefPubMed
13.
go back to reference Hoeper MM, Kramer T, Pan Z, et al. Mortality in pulmonary arterial hypertension: prediction by the 2015 European pulmonary hypertension guidelines risk stratification model. Eur Respir J. 2017;50(2):1700740.CrossRefPubMed Hoeper MM, Kramer T, Pan Z, et al. Mortality in pulmonary arterial hypertension: prediction by the 2015 European pulmonary hypertension guidelines risk stratification model. Eur Respir J. 2017;50(2):1700740.CrossRefPubMed
14.
go back to reference Boucly A, Weatherald J, Savale L, et al. Risk assessment, prognosis and guideline implementation in pulmonary arterial hypertension. Eur Respir J. 2017;50(2):1700889.CrossRefPubMed Boucly A, Weatherald J, Savale L, et al. Risk assessment, prognosis and guideline implementation in pulmonary arterial hypertension. Eur Respir J. 2017;50(2):1700889.CrossRefPubMed
15.
go back to reference Rosenkranz S, Preston IR. Right heart catheterisation: best practice and pitfalls in pulmonary hypertension. Eur Respir Rev. 2015;24(138):642–52.CrossRefPubMed Rosenkranz S, Preston IR. Right heart catheterisation: best practice and pitfalls in pulmonary hypertension. Eur Respir Rev. 2015;24(138):642–52.CrossRefPubMed
Metadata
Title
Assessment of Risk of Disease Progression in Pulmonary Arterial Hypertension: Insights from an International Survey of Clinical Practice
Authors
Jonathan E. Simons
Elena B. Mann
Adam Pierozynski
Publication date
01-09-2019
Publisher
Springer Healthcare
Published in
Advances in Therapy / Issue 9/2019
Print ISSN: 0741-238X
Electronic ISSN: 1865-8652
DOI
https://doi.org/10.1007/s12325-019-01030-4

Other articles of this Issue 9/2019

Advances in Therapy 9/2019 Go to the issue