Skip to main content
Top
Published in: Lung 1/2020

Open Access 01-02-2020 | Pulmonary Hypertension | PULMONARY HYPERTENSION

The Role of Noninvasive Endpoints in Predicting Long-Term Outcomes in Pulmonary Arterial Hypertension

Authors: Samantha L. Wronski, Margaret Mordin, Kim Kelley, Rebekah H. Anguiano, Peter Classi, Eric Shen, Scott Manaker

Published in: Lung | Issue 1/2020

Login to get access

Abstract

Background

Until recently, many clinical trials in patients with pulmonary arterial hypertension (PAH) evaluated exercise capacity with 6-minute walk distance (6MWD) as the primary endpoint. Common secondary endpoints include PAH functional class (FC), which assesses symptoms, and either brain natriuretic peptide (BNP) or the inactive N-terminal cleavage product of its prohormone (NT-proBNP), which assesses cardiac function.

Objective

Examine the relationships among 6MWD, FC, and BNP/NT-proBNP measured at baseline or follow-up with long-term outcomes in PAH studies.

Methods

Relevant literature from January 1990 to April 2018 were obtained by searching PubMed, Embase, and Cochrane. Articles in English reporting on associations between 6MWD, FC, or BNP/NT-proBNP and outcomes in PAH were identified. Each endpoint was evaluated individually. Prespecified inclusion and exclusion criteria were applied at level 1 (titles/abstracts) and level 2 (full-text review).

Results

The database search yielded 836 unique records; 65 full-text articles were reviewed. Twenty-five studies were eligible for inclusion. Findings supported the importance of measuring PAH noninvasive endpoints in predicting long-term outcomes. Patients with shorter or decreased 6MWD, poor (III/IV) or declining FC (e.g., from II to III), or elevated or increasing BNP/NT-proBNP had a higher risk of death and costly events (e.g., hospitalization, lung transplant). FC also predicted health care resource utilization and costs. Collectively, these endpoints establish risk groups that predict likelihood of complications from PAH or death.

Conclusion

Assessment of 6MWD, FC, and BNP/NT-proBNP provides low-cost, efficient, and noninvasive means of predicting long-term health and economic outcomes in patients with PAH.
Literature
1.
go back to reference Frost AE, Badesch DB, Barst RJ et al (2011) The changing picture of patients with pulmonary arterial hypertension in the United States: how REVEAL differs from historic and non-US Contemporary Registries. Chest 139(1):128–137PubMedCrossRef Frost AE, Badesch DB, Barst RJ et al (2011) The changing picture of patients with pulmonary arterial hypertension in the United States: how REVEAL differs from historic and non-US Contemporary Registries. Chest 139(1):128–137PubMedCrossRef
2.
go back to reference Ghofrani H, Grimminger F, Grünig E et al (2016) Predictors of long-term outcomes in patients treated with riociguat for pulmonary arterial hypertension: data from the PATENT-2 open-label, randomised, long-term extension trial. Lancet Respir Med 4(5):361–371PubMedCrossRef Ghofrani H, Grimminger F, Grünig E et al (2016) Predictors of long-term outcomes in patients treated with riociguat for pulmonary arterial hypertension: data from the PATENT-2 open-label, randomised, long-term extension trial. Lancet Respir Med 4(5):361–371PubMedCrossRef
3.
go back to reference Lan NSH, Massam BD, Kulkarni SS, Lang CC (2018) Pulmonary arterial hypertension: pathophysiology and treatment. Diseases 6(2):38PubMedCentralCrossRef Lan NSH, Massam BD, Kulkarni SS, Lang CC (2018) Pulmonary arterial hypertension: pathophysiology and treatment. Diseases 6(2):38PubMedCentralCrossRef
4.
go back to reference Burger CD, Ghandour M, Padmanabhan Menon D, Helmi H, Benza RL (2017) Early intervention in the management of pulmonary arterial hypertension: clinical and economic outcomes. Clinicoecon Outcomes Res 9:731–739PubMedPubMedCentralCrossRef Burger CD, Ghandour M, Padmanabhan Menon D, Helmi H, Benza RL (2017) Early intervention in the management of pulmonary arterial hypertension: clinical and economic outcomes. Clinicoecon Outcomes Res 9:731–739PubMedPubMedCentralCrossRef
5.
go back to reference Burke JP, Hunsche E, Regulier E, Nagao M, Buzinec P, Drake Iii W (2015) Characterizing pulmonary hypertension-related hospitalization costs among Medicare Advantage or commercially insured patients with pulmonary arterial hypertension: a retrospective database study. Am J Manag Care 21(3 Suppl):s47–58PubMed Burke JP, Hunsche E, Regulier E, Nagao M, Buzinec P, Drake Iii W (2015) Characterizing pulmonary hypertension-related hospitalization costs among Medicare Advantage or commercially insured patients with pulmonary arterial hypertension: a retrospective database study. Am J Manag Care 21(3 Suppl):s47–58PubMed
6.
go back to reference Dufour R, Pruett J, Hu N et al (2017) Healthcare resource utilization and costs for patients with pulmonary arterial hypertension: real-world documentation of functional class. J Med Econ 20(11):1178–1186PubMedCrossRef Dufour R, Pruett J, Hu N et al (2017) Healthcare resource utilization and costs for patients with pulmonary arterial hypertension: real-world documentation of functional class. J Med Econ 20(11):1178–1186PubMedCrossRef
7.
go back to reference Lajoie AC, Bonnet S, Lacasse Y, Lega J-C, Provencher S (2018) Interpreting risk reduction in clinical trials for pulmonary arterial hypertension. Eur Respir Rev 27(148):180020PubMedCrossRef Lajoie AC, Bonnet S, Lacasse Y, Lega J-C, Provencher S (2018) Interpreting risk reduction in clinical trials for pulmonary arterial hypertension. Eur Respir Rev 27(148):180020PubMedCrossRef
8.
9.
go back to reference Parikh KS, Rajagopal S, Arges K et al (2015) Use of outcome measures in pulmonary hypertension clinical trials. Am Heart J 170(3):419–429.e413PubMedCrossRef Parikh KS, Rajagopal S, Arges K et al (2015) Use of outcome measures in pulmonary hypertension clinical trials. Am Heart J 170(3):419–429.e413PubMedCrossRef
10.
go back to reference Raina A, Humbert M (2016) Risk assessment in pulmonary arterial hypertension. Eur Respir Rev 25(142):390–398PubMedCrossRef Raina A, Humbert M (2016) Risk assessment in pulmonary arterial hypertension. Eur Respir Rev 25(142):390–398PubMedCrossRef
11.
go back to reference Galiè N, Humbert M, Vachiery J-L et al (2015) 2015 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension. Eur Respir J 46(4):903CrossRefPubMed Galiè N, Humbert M, Vachiery J-L et al (2015) 2015 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension. Eur Respir J 46(4):903CrossRefPubMed
12.
go back to reference Hoeper MM, Kramer T, Pan Z et al (2017) Mortality in pulmonary arterial hypertension: prediction by the 2015 European pulmonary hypertension guidelines risk stratification model. Eur Respir J 50(2):1700740CrossRefPubMed Hoeper MM, Kramer T, Pan Z et al (2017) Mortality in pulmonary arterial hypertension: prediction by the 2015 European pulmonary hypertension guidelines risk stratification model. Eur Respir J 50(2):1700740CrossRefPubMed
13.
go back to reference Benza RL, Gomberg-Maitland M, Elliott CG et al (2019) 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 156:323–337PubMedCrossRef Benza RL, Gomberg-Maitland M, Elliott CG et al (2019) 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 156:323–337PubMedCrossRef
14.
15.
go back to reference Zamanian RT, Kudelko KT, Sung YK, Perez VdJ, Liu J, Spiekerkoetter E (2014) Current clinical management of pulmonary arterial hypertension. Circ Res 115(1):131–147PubMedPubMedCentralCrossRef Zamanian RT, Kudelko KT, Sung YK, Perez VdJ, Liu J, Spiekerkoetter E (2014) Current clinical management of pulmonary arterial hypertension. Circ Res 115(1):131–147PubMedPubMedCentralCrossRef
16.
go back to reference Divers C, Platt D, Wang E, Lin J, Lingohr-Smith M, Mathai SC (2017) A review of clinical trial endpoints of patients with pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension and how they relate to patient outcomes in the United States. J Manag Care Spec Pharm 23(1):92–104PubMed Divers C, Platt D, Wang E, Lin J, Lingohr-Smith M, Mathai SC (2017) A review of clinical trial endpoints of patients with pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension and how they relate to patient outcomes in the United States. J Manag Care Spec Pharm 23(1):92–104PubMed
17.
go back to reference Farber HW, Miller DP, McGoon MD, Frost AE, Benton WW, Benza RL (2015) Predicting outcomes in pulmonary arterial hypertension based on the 6-minute walk distance. J Heart Lung Transplant 34(3):362–368PubMedCrossRef Farber HW, Miller DP, McGoon MD, Frost AE, Benton WW, Benza RL (2015) Predicting outcomes in pulmonary arterial hypertension based on the 6-minute walk distance. J Heart Lung Transplant 34(3):362–368PubMedCrossRef
18.
go back to reference Gabler NB, French B, Strom BL, Palevsky HI, Taichman DB, Kawut SM, Halpern SD (2012) Validation of 6-minute walk distance as a surrogate end point in pulmonary arterial hypertension trials. Circulation 126(3):349–356PubMedPubMedCentralCrossRef Gabler NB, French B, Strom BL, Palevsky HI, Taichman DB, Kawut SM, Halpern SD (2012) Validation of 6-minute walk distance as a surrogate end point in pulmonary arterial hypertension trials. Circulation 126(3):349–356PubMedPubMedCentralCrossRef
19.
go back to reference Gomberg-Maitland M, Bull TM, Saggar R et al (2013) New trial designs and potential therapies for pulmonary artery hypertension. J Am Coll Cardiol 62(25 Suppl):D82–D91PubMedPubMedCentralCrossRef Gomberg-Maitland M, Bull TM, Saggar R et al (2013) New trial designs and potential therapies for pulmonary artery hypertension. J Am Coll Cardiol 62(25 Suppl):D82–D91PubMedPubMedCentralCrossRef
20.
go back to reference Hill NS, Cawley MJ, Heggen-Peay CL (2016) New therapeutic paradigms and guidelines in the management of pulmonary arterial hypertension. J Manag Care Spec Pharm 22(3-a Suppl):s3-s21.CrossRef Hill NS, Cawley MJ, Heggen-Peay CL (2016) New therapeutic paradigms and guidelines in the management of pulmonary arterial hypertension. J Manag Care Spec Pharm 22(3-a Suppl):s3-s21.CrossRef
21.
go back to reference Macchia A, Marchioli R, Marfisi R, Scarano M, Levantesi G, Tavazzi L, Tognoni G (2007) A meta-analysis of trials of pulmonary hypertension: A clinical condition looking for drugs and research methodology. Am Heart J 153(6):1037–1047PubMedCrossRef Macchia A, Marchioli R, Marfisi R, Scarano M, Levantesi G, Tavazzi L, Tognoni G (2007) A meta-analysis of trials of pulmonary hypertension: A clinical condition looking for drugs and research methodology. Am Heart J 153(6):1037–1047PubMedCrossRef
22.
go back to reference Savarese G, Paolillo S, Costanzo P et al (2012) Do changes of 6-minute walk distance predict clinical events in patients with pulmonary arterial hypertension? A meta-analysis of 22 randomized trials. J Am Coll Cardiol 60(13):1192–1201PubMedCrossRef Savarese G, Paolillo S, Costanzo P et al (2012) Do changes of 6-minute walk distance predict clinical events in patients with pulmonary arterial hypertension? A meta-analysis of 22 randomized trials. J Am Coll Cardiol 60(13):1192–1201PubMedCrossRef
23.
go back to reference Studer SM, Gilkin RJ Jr (2014) Clinical trial designs in PAH: shifting from functional measurements to long-term clinical outcomes. Am J Manag Care 20(6 Suppl):S115–122PubMed Studer SM, Gilkin RJ Jr (2014) Clinical trial designs in PAH: shifting from functional measurements to long-term clinical outcomes. Am J Manag Care 20(6 Suppl):S115–122PubMed
24.
go back to reference Vachiéry J-L, Yerly P, Huez S (2012) How to detect disease progression in pulmonary arterial hypertension. Eur Respir Rev 21(123):40–47PubMedCrossRef Vachiéry J-L, Yerly P, Huez S (2012) How to detect disease progression in pulmonary arterial hypertension. Eur Respir Rev 21(123):40–47PubMedCrossRef
25.
go back to reference Waxman AB, Farber HW (2015) Using clinical trial end points to risk stratify patients with pulmonary arterial hypertension. Circulation 132(22):2152–2161PubMedCrossRef Waxman AB, Farber HW (2015) Using clinical trial end points to risk stratify patients with pulmonary arterial hypertension. Circulation 132(22):2152–2161PubMedCrossRef
26.
go back to reference Chakinala MM, Barst R (2013) From short-term benefits to long-term outcomes: the evolution of clinical trials in pulmonary arterial hypertension. Pulm Circ 3(3):507–522PubMedPubMedCentralCrossRef Chakinala MM, Barst R (2013) From short-term benefits to long-term outcomes: the evolution of clinical trials in pulmonary arterial hypertension. Pulm Circ 3(3):507–522PubMedPubMedCentralCrossRef
27.
go back to reference Galiè N, Barberà JA, Frost AE et al (2015) Initial use of ambrisentan plus tadalafil in pulmonary arterial hypertension. N Engl J Med 373(9):834–844PubMedCrossRef Galiè N, Barberà JA, Frost AE et al (2015) Initial use of ambrisentan plus tadalafil in pulmonary arterial hypertension. N Engl J Med 373(9):834–844PubMedCrossRef
28.
go back to reference Sitbon O, Channick R, Chin KM et al (2015) Selexipag for the treatment of pulmonary arterial hypertension. N Engl J Med 373(26):2522–2533PubMedCrossRef Sitbon O, Channick R, Chin KM et al (2015) Selexipag for the treatment of pulmonary arterial hypertension. N Engl J Med 373(26):2522–2533PubMedCrossRef
29.
go back to reference Pulido T, Adzerikho I, Channick RN et al (2013) Macitentan and morbidity and mortality in pulmonary arterial hypertension. N Engl J Med 369(9):809–818PubMedCrossRef Pulido T, Adzerikho I, Channick RN et al (2013) Macitentan and morbidity and mortality in pulmonary arterial hypertension. N Engl J Med 369(9):809–818PubMedCrossRef
30.
go back to reference Tapson VF, Sanchez Diaz CJ, Bohns Meyer GM et al (2019) Treatment with oral treprostinil delays time to clinical worsening in patients with pulmonary arterial hypertension - results from FREEDOM-EV. J Heart Lung Transplant 38(4):S94–S95CrossRef Tapson VF, Sanchez Diaz CJ, Bohns Meyer GM et al (2019) Treatment with oral treprostinil delays time to clinical worsening in patients with pulmonary arterial hypertension - results from FREEDOM-EV. J Heart Lung Transplant 38(4):S94–S95CrossRef
31.
go back to reference White RJ, Sanchez Diaz CJ, Bohns Meyer GM, Pulido T, Sepulveda P, Wang KY, et al (2019) Treatment with oral treprostinil is associated with improved survival in pulmonary arterial hypertension participants from the FREEDOM-EV study. In: 13th Pulmonary Vascular Research Institute (PVRI) Annual World Congress on PVD. Barcelona, Spain White RJ, Sanchez Diaz CJ, Bohns Meyer GM, Pulido T, Sepulveda P, Wang KY, et al (2019) Treatment with oral treprostinil is associated with improved survival in pulmonary arterial hypertension participants from the FREEDOM-EV study. In: 13th Pulmonary Vascular Research Institute (PVRI) Annual World Congress on PVD. Barcelona, Spain
32.
go back to reference White RJ, Jerjes-Sanchez C, Bohns Meyer GM, Pulido T, Sepulveda P, Wang KY et al (2019) Risk scores and risk-based stratification of clinical worsening events in pulmonary arterial hypertension participants treated with oral treprostinil: FREEDOM-EV. Am J Respir Crit Care Med 199(Suppl):A5587–A5587 White RJ, Jerjes-Sanchez C, Bohns Meyer GM, Pulido T, Sepulveda P, Wang KY et al (2019) Risk scores and risk-based stratification of clinical worsening events in pulmonary arterial hypertension participants treated with oral treprostinil: FREEDOM-EV. Am J Respir Crit Care Med 199(Suppl):A5587–A5587
33.
go back to reference Newman JH, Rich S, Abman SH et al (2017) Enhancing insights into pulmonary vascular disease through a precision medicine approach A joint NHLBI-Cardiovascular Medical Research and Education Fund Workshop report. Am J Respir Crit Care Med 195(12):1661–1670PubMedPubMedCentralCrossRef Newman JH, Rich S, Abman SH et al (2017) Enhancing insights into pulmonary vascular disease through a precision medicine approach A joint NHLBI-Cardiovascular Medical Research and Education Fund Workshop report. Am J Respir Crit Care Med 195(12):1661–1670PubMedPubMedCentralCrossRef
34.
go back to reference Huang J, Mehta S, Mura M (2015) Early decline in six-minute walk distance from the time of diagnosis predicts clinical worsening in pulmonary arterial hypertension. Respiration 89(5):365–373PubMedCrossRef Huang J, Mehta S, Mura M (2015) Early decline in six-minute walk distance from the time of diagnosis predicts clinical worsening in pulmonary arterial hypertension. Respiration 89(5):365–373PubMedCrossRef
35.
go back to reference Tang Y, Luo Q, Liu Z et al (2017) Oxygen uptake efficiency slope predicts poor outcome in patients with idiopathic pulmonary arterial hypertension. J Am Heart Assoc 6(7):e005037PubMedPubMedCentralCrossRef Tang Y, Luo Q, Liu Z et al (2017) Oxygen uptake efficiency slope predicts poor outcome in patients with idiopathic pulmonary arterial hypertension. J Am Heart Assoc 6(7):e005037PubMedPubMedCentralCrossRef
36.
go back to reference Ozpelit E, Akdeniz B, Ozpelit ME et al (2015) Prognostic value of neutrophil-to-lymphocyte ratio in pulmonary arterial hypertension. J Int Med Res 43(5):661–671PubMedCrossRef Ozpelit E, Akdeniz B, Ozpelit ME et al (2015) Prognostic value of neutrophil-to-lymphocyte ratio in pulmonary arterial hypertension. J Int Med Res 43(5):661–671PubMedCrossRef
37.
go back to reference Souza R, Channick RN, Delcroix M et al (2018) Association between six-minute walk distance and long-term outcomes in patients with pulmonary arterial hypertension: Data from the randomized SERAPHIN trial. PLoS ONE 13(3):e0193226PubMedPubMedCentralCrossRef Souza R, Channick RN, Delcroix M et al (2018) Association between six-minute walk distance and long-term outcomes in patients with pulmonary arterial hypertension: Data from the randomized SERAPHIN trial. PLoS ONE 13(3):e0193226PubMedPubMedCentralCrossRef
38.
go back to reference Zelniker TA, Huscher D, Vonk-Noordegraaf A et al (2018) The 6MWT as a prognostic tool in pulmonary arterial hypertension: results from the COMPERA registry. Clin Res Cardiol 107:460–470PubMedCrossRef Zelniker TA, Huscher D, Vonk-Noordegraaf A et al (2018) The 6MWT as a prognostic tool in pulmonary arterial hypertension: results from the COMPERA registry. Clin Res Cardiol 107:460–470PubMedCrossRef
39.
go back to reference Benza RL, Miller DP, Gomberg-Maitland M et al (2010) Predicting survival in pulmonary arterial hypertension: insights from the Registry to Evaluate Early and Long-Term Pulmonary Arterial Hypertension Disease Management (REVEAL). Circulation 122(2):164–172PubMedCrossRef Benza RL, Miller DP, Gomberg-Maitland M et al (2010) Predicting survival in pulmonary arterial hypertension: insights from the Registry to Evaluate Early and Long-Term Pulmonary Arterial Hypertension Disease Management (REVEAL). Circulation 122(2):164–172PubMedCrossRef
40.
go back to reference Fritz JS, Blair C, Oudiz RJ et al (2013) Baseline and follow-up 6-min walk distance and brain natriuretic peptide predict 2-year mortality in pulmonary arterial hypertension. Chest 143(2):315–323PubMedCrossRef Fritz JS, Blair C, Oudiz RJ et al (2013) Baseline and follow-up 6-min walk distance and brain natriuretic peptide predict 2-year mortality in pulmonary arterial hypertension. Chest 143(2):315–323PubMedCrossRef
41.
go back to reference Batal O, Khatib OF, Dweik RA, Hammel JP, McCarthy K, Minai OA (2012) Comparison of baseline predictors of prognosis in pulmonary arterial hypertension in patients surviving ≤ 2 years and those surviving %3c 5 years after baseline right-sided cardiac catheterization. Am J Cardiol 109(10):1514–1520PubMedCrossRef Batal O, Khatib OF, Dweik RA, Hammel JP, McCarthy K, Minai OA (2012) Comparison of baseline predictors of prognosis in pulmonary arterial hypertension in patients surviving ≤ 2 years and those surviving %3c 5 years after baseline right-sided cardiac catheterization. Am J Cardiol 109(10):1514–1520PubMedCrossRef
42.
go back to reference Humbert M, Sitbon O, Chaouat A et al (2010) Survival in patients with idiopathic, familial, and anorexigen-associated pulmonary arterial hypertension in the modern management era. Circulation 122(2):156–163PubMedCrossRef Humbert M, Sitbon O, Chaouat A et al (2010) Survival in patients with idiopathic, familial, and anorexigen-associated pulmonary arterial hypertension in the modern management era. Circulation 122(2):156–163PubMedCrossRef
43.
44.
go back to reference Benza RL, Seeger W, McLaughlin VV et al (2011) Long-term effects of inhaled treprostinil in patients with pulmonary arterial hypertension: the Treprostinil Sodium Inhalation Used in the Management of Pulmonary Arterial Hypertension (TRIUMPH) study open-label extension. J Heart Lung Transplant 30(12):1327–1333PubMedCrossRef Benza RL, Seeger W, McLaughlin VV et al (2011) Long-term effects of inhaled treprostinil in patients with pulmonary arterial hypertension: the Treprostinil Sodium Inhalation Used in the Management of Pulmonary Arterial Hypertension (TRIUMPH) study open-label extension. J Heart Lung Transplant 30(12):1327–1333PubMedCrossRef
45.
go back to reference Weatherald J, Boucly A, Chemla D et al (2018) Prognostic value of follow-up hemodynamic variables after initial management in pulmonary arterial hypertension. Circulation 137(7):693–704PubMedCrossRef Weatherald J, Boucly A, Chemla D et al (2018) Prognostic value of follow-up hemodynamic variables after initial management in pulmonary arterial hypertension. Circulation 137(7):693–704PubMedCrossRef
46.
go back to reference Nickel N, Golpon H, Greer M et al (2012) The prognostic impact of follow-up assessments in patients with idiopathic pulmonary arterial hypertension. Eur Respir J 39(3):589–596PubMedCrossRef Nickel N, Golpon H, Greer M et al (2012) The prognostic impact of follow-up assessments in patients with idiopathic pulmonary arterial hypertension. Eur Respir J 39(3):589–596PubMedCrossRef
47.
go back to reference Snipelisky D, Jentzer J, Batal O, Dardari Z, Mathier M (2018) Serum albumin concentration as an independent prognostic indicator in patients with pulmonary arterial hypertension. Clin Cardiol 41:782–787PubMedPubMedCentralCrossRef Snipelisky D, Jentzer J, Batal O, Dardari Z, Mathier M (2018) Serum albumin concentration as an independent prognostic indicator in patients with pulmonary arterial hypertension. Clin Cardiol 41:782–787PubMedPubMedCentralCrossRef
48.
go back to reference Kane GC, Maradit-Kremers H, Slusser JP, Scott CG, Frantz RP, McGoon MD (2011) Integration of clinical and hemodynamic parameters in the prediction of long-term survival in patients with pulmonary arterial hypertension. Chest 139(6):1285–1293PubMedCrossRef Kane GC, Maradit-Kremers H, Slusser JP, Scott CG, Frantz RP, McGoon MD (2011) Integration of clinical and hemodynamic parameters in the prediction of long-term survival in patients with pulmonary arterial hypertension. Chest 139(6):1285–1293PubMedCrossRef
49.
go back to reference Barst RJ, Chung L, Zamanian RT, Turner M, McGoon MD (2013) Functional class improvement and 3-year survival outcomes in patients with pulmonary arterial hypertension in the REVEAL Registry. Chest 144(1):160–168PubMedCrossRef Barst RJ, Chung L, Zamanian RT, Turner M, McGoon MD (2013) Functional class improvement and 3-year survival outcomes in patients with pulmonary arterial hypertension in the REVEAL Registry. Chest 144(1):160–168PubMedCrossRef
50.
go back to reference Tiede H, Sommer N, Milger K et al (2013) Short-term improvement in pulmonary hemodynamics is strongly predictive of long-term survival in patients with pulmonary arterial hypertension. Pulm Circ 3(3):523–532PubMedPubMedCentralCrossRef Tiede H, Sommer N, Milger K et al (2013) Short-term improvement in pulmonary hemodynamics is strongly predictive of long-term survival in patients with pulmonary arterial hypertension. Pulm Circ 3(3):523–532PubMedPubMedCentralCrossRef
51.
go back to reference Mauritz GJ, Rizopoulos D, Groepenhoff H et al (2011) Usefulness of serial N-terminal pro-B-type natriuretic peptide measurements for determining prognosis in patients with pulmonary arterial hypertension. Am J Cardiol 108(11):1645–1650PubMedCrossRef Mauritz GJ, Rizopoulos D, Groepenhoff H et al (2011) Usefulness of serial N-terminal pro-B-type natriuretic peptide measurements for determining prognosis in patients with pulmonary arterial hypertension. Am J Cardiol 108(11):1645–1650PubMedCrossRef
52.
go back to reference Frantz RP, Farber HW, Badesch DB et al (2018) Baseline and serial brain natriuretic peptide level predicts 5-year overall survival in patients with pulmonary arterial hypertension: data from the REVEAL registry. Chest 154:126–135PubMedPubMedCentralCrossRef Frantz RP, Farber HW, Badesch DB et al (2018) Baseline and serial brain natriuretic peptide level predicts 5-year overall survival in patients with pulmonary arterial hypertension: data from the REVEAL registry. Chest 154:126–135PubMedPubMedCentralCrossRef
53.
go back to reference Ehlken N, Verduyn C, Tiede H et al (2014) Economic evaluation of exercise training in patients with pulmonary hypertension. Lung 192(3):359–366PubMedCrossRef Ehlken N, Verduyn C, Tiede H et al (2014) Economic evaluation of exercise training in patients with pulmonary hypertension. Lung 192(3):359–366PubMedCrossRef
54.
go back to reference Boucly A, Weatherald J, Savale L et al (2017) Risk assessment, prognosis and guideline implementation in pulmonary arterial hypertension. Eur Respir J 50(2):1700889PubMedCrossRef Boucly A, Weatherald J, Savale L et al (2017) Risk assessment, prognosis and guideline implementation in pulmonary arterial hypertension. Eur Respir J 50(2):1700889PubMedCrossRef
55.
go back to reference Kylhammar D, Kjellstrom B, Hjalmarsson C et al (2018) A comprehensive risk stratification at early follow-up determines prognosis in pulmonary arterial hypertension. Eur Heart J 39(47):4175–4181CrossRefPubMed Kylhammar D, Kjellstrom B, Hjalmarsson C et al (2018) A comprehensive risk stratification at early follow-up determines prognosis in pulmonary arterial hypertension. Eur Heart J 39(47):4175–4181CrossRefPubMed
56.
go back to reference Galie N, Humbert M, Vachiery JL et al (2016) 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 37(1):67–119PubMedCrossRef Galie N, Humbert M, Vachiery JL et al (2016) 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 37(1):67–119PubMedCrossRef
57.
go back to reference Iannuzzi GL, D'Alto M, Formisano R, Maniscalco M (2019) Biomarkers in clinical management of pulmonary hypertension: has the emperor no clothes? A call for action. Biomark Med 13(4):235–238PubMedCrossRef Iannuzzi GL, D'Alto M, Formisano R, Maniscalco M (2019) Biomarkers in clinical management of pulmonary hypertension: has the emperor no clothes? A call for action. Biomark Med 13(4):235–238PubMedCrossRef
58.
go back to reference Chin KM, Rubin LJ, Channick R et al (2019) Association of N-terminal pro brain natriuretic peptide and long-term outcome in patients with pulmonary arterial hypertension. Circulation 139(21):2440–2450PubMedPubMedCentralCrossRef Chin KM, Rubin LJ, Channick R et al (2019) Association of N-terminal pro brain natriuretic peptide and long-term outcome in patients with pulmonary arterial hypertension. Circulation 139(21):2440–2450PubMedPubMedCentralCrossRef
59.
go back to reference Weatherald J, Boucly A, Sahay S, Humbert M, Sitbon O (2018) The low-risk profile in pulmonary arterial hypertension Time for a paradigm shift to goal-oriented clinical trial endpoints? Am J Respir Crit Care Med 197(7):860–868PubMedCrossRef Weatherald J, Boucly A, Sahay S, Humbert M, Sitbon O (2018) The low-risk profile in pulmonary arterial hypertension Time for a paradigm shift to goal-oriented clinical trial endpoints? Am J Respir Crit Care Med 197(7):860–868PubMedCrossRef
60.
go back to reference McLaughlin VV, Hoeper MM, Channick RN et al (2018) Pulmonary arterial hypertension-related morbidity is prognostic for mortality. J Am Coll Cardiol 71(7):752–763PubMedCrossRef McLaughlin VV, Hoeper MM, Channick RN et al (2018) Pulmonary arterial hypertension-related morbidity is prognostic for mortality. J Am Coll Cardiol 71(7):752–763PubMedCrossRef
61.
go back to reference Sitbon O, Gomberg-Maitland M, Granton J et al (2019) Clinical trial design and new therapies for pulmonary arterial hypertension. Eur Respir J 53(1):1801908PubMedPubMedCentralCrossRef Sitbon O, Gomberg-Maitland M, Granton J et al (2019) Clinical trial design and new therapies for pulmonary arterial hypertension. Eur Respir J 53(1):1801908PubMedPubMedCentralCrossRef
62.
Metadata
Title
The Role of Noninvasive Endpoints in Predicting Long-Term Outcomes in Pulmonary Arterial Hypertension
Authors
Samantha L. Wronski
Margaret Mordin
Kim Kelley
Rebekah H. Anguiano
Peter Classi
Eric Shen
Scott Manaker
Publication date
01-02-2020
Publisher
Springer US
Published in
Lung / Issue 1/2020
Print ISSN: 0341-2040
Electronic ISSN: 1432-1750
DOI
https://doi.org/10.1007/s00408-019-00289-2

Other articles of this Issue 1/2020

Lung 1/2020 Go to the issue
Live Webinar | 27-06-2024 | 18:00 (CEST)

Keynote webinar | Spotlight on medication adherence

Live: Thursday 27th June 2024, 18:00-19:30 (CEST)

WHO estimates that half of all patients worldwide are non-adherent to their prescribed medication. The consequences of poor adherence can be catastrophic, on both the individual and population level.

Join our expert panel to discover why you need to understand the drivers of non-adherence in your patients, and how you can optimize medication adherence in your clinics to drastically improve patient outcomes.

Prof. Kevin Dolgin
Prof. Florian Limbourg
Prof. Anoop Chauhan
Developed by: Springer Medicine
Obesity Clinical Trial Summary

At a glance: The STEP trials

A round-up of the STEP phase 3 clinical trials evaluating semaglutide for weight loss in people with overweight or obesity.

Developed by: Springer Medicine

Highlights from the ACC 2024 Congress

Year in Review: Pediatric cardiology

Watch Dr. Anne Marie Valente present the last year's highlights in pediatric and congenital heart disease in the official ACC.24 Year in Review session.

Year in Review: Pulmonary vascular disease

The last year's highlights in pulmonary vascular disease are presented by Dr. Jane Leopold in this official video from ACC.24.

Year in Review: Valvular heart disease

Watch Prof. William Zoghbi present the last year's highlights in valvular heart disease from the official ACC.24 Year in Review session.

Year in Review: Heart failure and cardiomyopathies

Watch this official video from ACC.24. Dr. Biykem Bozkurt discusses last year's major advances in heart failure and cardiomyopathies.