Mortality rate and prognostic factors in group I pulmonary arterial hypertension patients
Main Article Content
Abstract
Pulmonary arterial hypertension (PAH) is a pathophysiological disorder that can be seen in many clinical conditions and is associated with various cardiovascular and respiratory diseases. The first registry about pulmonary hypertension in the United States, established in 1981, announced that the 1-year and 5-year mortality rates of the disease were 32% and 66%, respectively. Our study aimed to assess the mortality rate and investigate predictive factors for mortality in PAH patients hospitalized at a cardiovascular center. The results indicated that the mortality rates at 1 year and 5 years were 4.2% and 11.2%, respectively. Various factors, such as functional class III and IV, NT-proBNP value ≥ 1100 pg/ml at the time of admission, have been shown to predict survival in patients with PAH. Conclusion: The mortality rate for hospitalized patients with pulmonary arterial hypertension (group I) remains high. Predicting survival tools have many implications in monitoring treatment for this patient population.
Article Details
Keywords
Pulmonary arterial hypertension, mortality rate, prognostic factors
References
1. Swinnen K, Quarck R, Godinas L, et al. Learning from registries in pulmonary arterial hypertension: pitfalls and recommendations. Eur Respir Rev. 2019;28(154). doi:10.1183/16000617.0050-2019
2. Humbert M, Kovacs G, Hoeper MM, et al. 2022 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension: Developed by the 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 the International Society for Heart and Lung Transplantation (ISHLT) and the European Reference Network on rare respiratory diseases (ERN-LUNG). Eur Heart J. 2022;43(38):3618-3731. doi:10.1093/eurheartj/ehac237
3. 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):343-349. doi:10.7326/0003-4819-115-5-343
4. Benza RL, Miller DP, Gomberg-Maitland M, et al. Predicting Survival in Pulmonary Arterial Hypertension. Circulation. 2010;122(2):164-172. doi:10.1161/CIRCULAT IONAHA.109.898122
5. Gaine S, McLaughlin V. Pulmonary arterial hypertension: tailoring treatment to risk in the current era. Eur Respir Rev Off J Eur Respir Soc. 2017;26(146):170095. doi:10.1183/16000617.0095-2017
6. Campo A, Mathai SC, Pavec JL, et al. Outcomes of hospitalisation for right heart failure in pulmonary arterial hypertension. Eur Respir J. 2011;38(2):359-367. doi:10.1183/09031936.00148310
7. Classification and WHO functional class. PHA Europe. September 16, 2024. Accessed October 6, 2024. https://www.phaeurope.org/about-ph/classification-and-who-functional-cl ass/
8. Bossone E, D’Andrea A, D’Alto M, et al. Echocardiography in Pulmonary Arterial Hypertension: from Diagnosis to Prognosis. J Am Soc Echocardiogr. 2013;26(1):1-14. doi:10.1016/j.echo.2012.10.009
9. Lê Đức Tài. Tìm hiểu khả năng gắng sức bằng test đi bộ 6 phút ở những bệnh nhân tim bẩm sinh có hội chứng Eisenmenger. Trường Đại học Y Hà Nội. 2015.
10. Đỗ Thị Thu Trang. Tìm hiểu giá trị tiên lượng của chỉ số E/Ea ở bệnh nhân tăng áp động mạch phổi nhiều do bệnh tim bẩm sinh có luồng thông trong tim. Trường Đại học Y Hà Nội; 2012.
11. Farber HW, Miller DP, Poms AD, et al. Five-Year Outcomes of Patients Enrolled in the REVEAL Registry. CHEST. 2015;148(4):1043-1054. doi:10.1378/chest.15-0300
12. de-Miguel-Díez J, Lopez-de-Andres A, Hernandez-Barrera V, et al. Retrospective observational analysis of hospital discharge database to characterize primary pulmonary hypertension and its outcomes in Spain from 2004 to 2015. Medicine (Baltimore). 2019;98(18):e15518. doi:10.1097/MD.0000000 000015518
13. Rizvi B, Desai R, Elwing J, et al. Hospitalizations of idiopathic pulmonary arterial hypertension in the united states: a national perspective a decade apart (2007 vs 2017). CHEST. 2021;160(4):A2281-A2282. doi:10.1016/j.chest.2021.07.1996
14. Nguyễn Thị Minh Lý. Nghiên cứu đặc điểm lâm sàng, cận lâm sàng và một số yếu tố liên quan đến tiên lượng ở bệnh nhân tăng áp lực động mạch phổi trung bình đến nặng. Trường Đại học Y Hà Nội. 2020.
15. Nguyễn Thị Hoa. Đánh giá sự thay đổi về lâm sàng và áp lực động mạch phổi ở bệnh nhân tim bẩm sinh người lớn có tăng áp lực động mạch phổi nặng sau điều trị đóng lỗ thông. Trường Đại học Y Hà Nội. 2017.
16. Ling Y, Johnson MK, Kiely DG, et al. Changing demographics, epidemiology, and survival of incident pulmonary arterial hypertension: results from the pulmonary hypertension registry of the United Kingdom and Ireland. Am J Respir Crit Care Med. 2012;186(8):790-796. doi:10.1164/rccm.20120 3-0383OC
17. Humbert M, Sitbon O, Chaouat A, et al. Pulmonary arterial hypertension in France: results from a national registry. Am J Respir Crit Care Med. 2006;173(9):1023-1030. doi:10.1164/rccm.200510-1668OC
18. Harder EM, Small AM, Fares WH. Primary cardiac hospitalizations in pulmonary arterial hypertension: Trends and outcomes from 2001 to 2014. Respir Med. 2020;161. doi:10.1016/j.rmed.2019.105850
19. Nickel N, Golpon H, Greer M, et al. The prognostic impact of follow-up assessments in patients with idiopathic pulmonary arterial hypertension. Eur Respir J. 2012;39(3):589-596. doi:10.1183/09031936.00092311
20. Barst RJ, Chung L, Zamanian RT, et al. Functional Class Improvement and 3-Year Survival Outcomes in Patients With Pulmonary Arterial Hypertension in the REVEAL Registry. CHEST. 2013;144(1):160-168. doi:10.1378/chest.12-2417
21. Diller GP, Kempny A, Inuzuka R, et al. Survival prospects of treatment naïve patients with Eisenmenger: a systematic review of the literature and report of own experience. Heart. 2014;100(17):1366-1372. doi:10.1136/heartjnl-2014-305690
22. Marques-Alves P, Baptista R, Marinho da Silva A, et al. Real-world, long-term survival of incident patients with pulmonary arterial hypertension. Rev Port Pneumol Engl Ed. 2017;23(3):124-131. doi:10.1016/j.rppnen.2017.01.006
23. Stepnowska E, Lewicka E, Dąbrowska-Kugacka A, et al. Predictors of poor outcome in patients with pulmonary arterial hypertension: A single center study. PLoS ONE. 2018;13(4):e0193245. doi:10.1371/journal.pon e.0193245
24. Raymond RJ, Hinderliter AL, Willis PW, et al. Echocardiographic predictors of adverse outcomes in primary pulmonary hypertension. J Am Coll Cardiol. 2002;39(7):1214-1219. doi:10.1016/S0735-1097(02)01744-8
25. Austin C, Alassas K, Burger C, et al. Echocardiographic Assessment of Estimated Right Atrial Pressure and Size Predicts Mortality in Pulmonary Arterial Hypertension. CHEST. 2015;147(1):198-208. doi:10.1378/chest.13-3035
2. Humbert M, Kovacs G, Hoeper MM, et al. 2022 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension: Developed by the 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 the International Society for Heart and Lung Transplantation (ISHLT) and the European Reference Network on rare respiratory diseases (ERN-LUNG). Eur Heart J. 2022;43(38):3618-3731. doi:10.1093/eurheartj/ehac237
3. 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):343-349. doi:10.7326/0003-4819-115-5-343
4. Benza RL, Miller DP, Gomberg-Maitland M, et al. Predicting Survival in Pulmonary Arterial Hypertension. Circulation. 2010;122(2):164-172. doi:10.1161/CIRCULAT IONAHA.109.898122
5. Gaine S, McLaughlin V. Pulmonary arterial hypertension: tailoring treatment to risk in the current era. Eur Respir Rev Off J Eur Respir Soc. 2017;26(146):170095. doi:10.1183/16000617.0095-2017
6. Campo A, Mathai SC, Pavec JL, et al. Outcomes of hospitalisation for right heart failure in pulmonary arterial hypertension. Eur Respir J. 2011;38(2):359-367. doi:10.1183/09031936.00148310
7. Classification and WHO functional class. PHA Europe. September 16, 2024. Accessed October 6, 2024. https://www.phaeurope.org/about-ph/classification-and-who-functional-cl ass/
8. Bossone E, D’Andrea A, D’Alto M, et al. Echocardiography in Pulmonary Arterial Hypertension: from Diagnosis to Prognosis. J Am Soc Echocardiogr. 2013;26(1):1-14. doi:10.1016/j.echo.2012.10.009
9. Lê Đức Tài. Tìm hiểu khả năng gắng sức bằng test đi bộ 6 phút ở những bệnh nhân tim bẩm sinh có hội chứng Eisenmenger. Trường Đại học Y Hà Nội. 2015.
10. Đỗ Thị Thu Trang. Tìm hiểu giá trị tiên lượng của chỉ số E/Ea ở bệnh nhân tăng áp động mạch phổi nhiều do bệnh tim bẩm sinh có luồng thông trong tim. Trường Đại học Y Hà Nội; 2012.
11. Farber HW, Miller DP, Poms AD, et al. Five-Year Outcomes of Patients Enrolled in the REVEAL Registry. CHEST. 2015;148(4):1043-1054. doi:10.1378/chest.15-0300
12. de-Miguel-Díez J, Lopez-de-Andres A, Hernandez-Barrera V, et al. Retrospective observational analysis of hospital discharge database to characterize primary pulmonary hypertension and its outcomes in Spain from 2004 to 2015. Medicine (Baltimore). 2019;98(18):e15518. doi:10.1097/MD.0000000 000015518
13. Rizvi B, Desai R, Elwing J, et al. Hospitalizations of idiopathic pulmonary arterial hypertension in the united states: a national perspective a decade apart (2007 vs 2017). CHEST. 2021;160(4):A2281-A2282. doi:10.1016/j.chest.2021.07.1996
14. Nguyễn Thị Minh Lý. Nghiên cứu đặc điểm lâm sàng, cận lâm sàng và một số yếu tố liên quan đến tiên lượng ở bệnh nhân tăng áp lực động mạch phổi trung bình đến nặng. Trường Đại học Y Hà Nội. 2020.
15. Nguyễn Thị Hoa. Đánh giá sự thay đổi về lâm sàng và áp lực động mạch phổi ở bệnh nhân tim bẩm sinh người lớn có tăng áp lực động mạch phổi nặng sau điều trị đóng lỗ thông. Trường Đại học Y Hà Nội. 2017.
16. Ling Y, Johnson MK, Kiely DG, et al. Changing demographics, epidemiology, and survival of incident pulmonary arterial hypertension: results from the pulmonary hypertension registry of the United Kingdom and Ireland. Am J Respir Crit Care Med. 2012;186(8):790-796. doi:10.1164/rccm.20120 3-0383OC
17. Humbert M, Sitbon O, Chaouat A, et al. Pulmonary arterial hypertension in France: results from a national registry. Am J Respir Crit Care Med. 2006;173(9):1023-1030. doi:10.1164/rccm.200510-1668OC
18. Harder EM, Small AM, Fares WH. Primary cardiac hospitalizations in pulmonary arterial hypertension: Trends and outcomes from 2001 to 2014. Respir Med. 2020;161. doi:10.1016/j.rmed.2019.105850
19. Nickel N, Golpon H, Greer M, et al. The prognostic impact of follow-up assessments in patients with idiopathic pulmonary arterial hypertension. Eur Respir J. 2012;39(3):589-596. doi:10.1183/09031936.00092311
20. Barst RJ, Chung L, Zamanian RT, et al. Functional Class Improvement and 3-Year Survival Outcomes in Patients With Pulmonary Arterial Hypertension in the REVEAL Registry. CHEST. 2013;144(1):160-168. doi:10.1378/chest.12-2417
21. Diller GP, Kempny A, Inuzuka R, et al. Survival prospects of treatment naïve patients with Eisenmenger: a systematic review of the literature and report of own experience. Heart. 2014;100(17):1366-1372. doi:10.1136/heartjnl-2014-305690
22. Marques-Alves P, Baptista R, Marinho da Silva A, et al. Real-world, long-term survival of incident patients with pulmonary arterial hypertension. Rev Port Pneumol Engl Ed. 2017;23(3):124-131. doi:10.1016/j.rppnen.2017.01.006
23. Stepnowska E, Lewicka E, Dąbrowska-Kugacka A, et al. Predictors of poor outcome in patients with pulmonary arterial hypertension: A single center study. PLoS ONE. 2018;13(4):e0193245. doi:10.1371/journal.pon e.0193245
24. Raymond RJ, Hinderliter AL, Willis PW, et al. Echocardiographic predictors of adverse outcomes in primary pulmonary hypertension. J Am Coll Cardiol. 2002;39(7):1214-1219. doi:10.1016/S0735-1097(02)01744-8
25. Austin C, Alassas K, Burger C, et al. Echocardiographic Assessment of Estimated Right Atrial Pressure and Size Predicts Mortality in Pulmonary Arterial Hypertension. CHEST. 2015;147(1):198-208. doi:10.1378/chest.13-3035