Data of modern registries on clinical course and prognosis of patients with idiopathic pulmonary arterial hypertension

Shariya A.M.; Martynyuk T.V.

doi:https://doi.org/10.17116/Cardiobulletin20211603123

Shariya A.M.

National Medical Research Center of Cardiology

Martynyuk T.V.

National Medical Research Center of Cardiology

Scopus AuthorID: 6602715051
ORCID: 0000-0002-9022-8097

Data of modern registries on clinical course and prognosis of patients with idiopathic pulmonary arterial hypertension

Authors:

Shariya A.M., Martynyuk T.V.

More about the authors

Journal: Russian Cardiology Bulletin. 2021;16(3): 23‑27

DOI: 10.17116/Cardiobulletin20211603123

Read: 2872 times

Download PDF (RU)

Contact the author

Table of contents

To cite this article:

Shariya AM, Martynyuk TV. Data of modern registries on clinical course and prognosis of patients with idiopathic pulmonary arterial hypertension. Russian Cardiology Bulletin. 2021;16(3):23‑27. (In Russ.)
https://doi.org/10.17116/Cardiobulletin20211603123

Подписка 2026 Кардиологический вестник (Russian Cardiology Bulletin)

Использование инфографики авторами научных работ

Close metadata

Recommended articles:

Prognostic significance of NT-proBNP and sST2 in patients with chronic heart failure and reduced ejection fraction after previous decompensation. Russian Cardiology Bulletin. 2025;(1):49-56

Pulmonary arterial hypertension in disorders of porto-caval hemodynamics. The role of ultrasound diagnostics. Russian Cardiology Bulletin. 2025;(1):90-98

Impact of tumor size on actuarial survival of patients with primary breast sarcoma: a case-control study. Pirogov Russian Journal of Surgery. 2024;(12):38-43

Difficult intubation risk assessment in bariatric surgery. Russian Journal of Anesthesiology and Reanimatology. 2025;(1):62-68

Analysis of dynamics and forecast of morbidity of diseases of the urogenital system in the child population living in the Republic of Sakha (Yakutia). Russian Journal of Preventive Medicine. 2025;(3):60-66

Relationship between stroke-associated pneumonia and long-term outcomes of ischemic stroke. S.S. Korsakov Journal of Neurology and Psychiatry. 2025;(3):57-61

The phenomenon of tumor budding in gastric cancer. Russian Journal of Archive of Pathology. 2025;(2):79-87

Radical cystectomy with fertility preservation for non-muscle invasive bladder cancer. P.A. Herzen Journal of Oncology. 2025;(2):76-82

Diagnostic and prognostic potential of modern biomarkers in community-acquired pneumonia in children. Russian Journal of Preventive Medicine. 2025;(4):130-135

Pathogenetic aspects of autoantibody formation to central nervous system structures in patients with encephalitis. S.S. Korsakov Journal of Neurology and Psychiatry. 2025;(4):53-58

Abstract:

This review is devoted to the largest registries of patients with idiopathic pulmonary arterial hypertension in different countries, such as the NIH (USA), the National French and Swedish Registry, the Spanish REHAP Registry, the REVEAL and COMPERA registries. The authors analyze data from the Chinese, Australian and Russian registries. Significant improvement in clinical characteristics, prognosis and survival, longer period without clinical deterioration in patients with IPAH due to use of new pathogenetic methods of treatment were emphasized. The authors analyze changes of demographic characteristics of patients in recent years, emergence of many parameters used in risk assessment, as well as the tendency of modern studies to searching for more significant prognostic indicators for accurate risk assessment and clinical decision-making.

Keywords:

idiopathic pulmonary hypertension

pulmonary hypertension

prognosis

survival

time to clinical deterioration

Authors:

Shariya A.M.

National Medical Research Center of Cardiology

Martynyuk T.V.

National Medical Research Center of Cardiology

Scopus AuthorID: 6602715051
ORCID: 0000-0002-9022-8097

Received:

17.08.2021

Accepted:

27.08.2021

Close metadata

References:

Galiè N, Humbert M, Vachiery JL, Gibbs S, Lang I, Torbicki A, Simonneau G, Peacock A, Vonk Noordegraaf A, Beghetti M, Ghofrani A, Gomez Sanchez MA, Hansmann G, Klepetko W, Lancellotti P, Matucci M, McDonagh T, Pierard LA, Trindade PT, Zompatori M, Hoeper M; ESC Scientific Document Group. 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). European Heart Journal. 2016;37(1):67-119. https://doi.org/10.1093/eurheartj/ehv317
Rich S, Dantzker DR, Ayres SM, Bergofsky EH, Brundage BH, Detre KM, Fishman AP, Goldring RM, Groves BM, Koerner SK, et al. Primary pulmonary hypertension. A national prospective study. Annals of Internal Medicine. 1987;107(2):216-223. https://doi.org/10.7326/0003-4819-107-2-216
D’Alonzo GE, Barst RJ, Ayres SM, Bergofsky EH, Brundage BH, Detre KM, Fishman AP, Goldring RM, Groves BM, Kernis JT, et al. Survival in patients with primary pulmonary hypertension. Results from a national prospective registry. Annals of Internal Medicine. 1991;115(5):343-349. https://doi.org/10.7326/0003-4819-115-5-343
McEvoy CA, Sardana M, Moll M, Farber H, Chakinala M. Patient Registries in Pulmonary Arterial Hypertension: The Role of Survival Equations and Risk Calculators. In: Pulmonary Hypertension. Springer, Cham. 2016;307-325. https://doi.org/10.1007/978-3-319-23594-3_20
Humbert M, Sitbon O, Yaïci A, Montani D, O’Callaghan DS, Jaïs X, Parent F, Savale L, Natali D, Günther S, Chaouat A, Chabot F, Cordier JF, Habib G, Gressin V, Jing ZC, Souza R, Simonneau G; French Pulmonary Arterial Hypertension Network. Survival in incident and prevalent cohorts of patients with pulmonary arterial hypertension. The European Respiratory Journal. 2010;36(3):549-555. https://doi.org/10.1183/09031936.00057010
Benza RL, Miller DP, Barst RJ, Badesch DB, Frost AE, McGoon MD. An evaluation of long-term survival from time of diagnosis in pulmonary arterial hypertension from the REVEAL Registry. Chest. 2012;142(2):448-456. https://doi.org/10.1378/chest.11-1460
Sitbon O, Benza RL, Badesch DB, Barst RJ, Elliott CG, Gressin V, Lemarié JC, Miller DP, Muros-Le Rouzic E, Simonneau G, Frost AE, Farber HW, Humbert M, McGoon MD. Validation of two predictive models for survival in pulmonary arterial hypertension. The European Respiratory Journal. 2015;46(1):152-164. https://doi.org/10.1183/09031936.00004414
Rådegran G, Kjellström B, Ekmehag B, Larsen F, Rundqvist B, Blomquist SB, Gustafsson C, Hesselstrand R, Karlsson M, Kornhall B, Nisell M, Persson L, Ryftenius H, Selin M, Ullman B, Wall K, Wikström G, Willehadson M, Jansson K; Söderberg S, on behalf of SveFPH and SPAHR. Characteristics and survival of adult Swedish PAH and CTEPH patients 2000-2014. Scandinavian Cardiovascular Journal: SCJ. 2016;50(4):243-250. https://doi.org/10.1080/14017431.2016.1185532
Escribano-Subias P, Blanco I, López-Meseguer M, Lopez-Guarch CJ, Roman A, Morales P, Castillo-Palma MJ, Segovia J, Gómez-Sanchez MA, Barberà JA; REHAP investigators. Survival in pulmonary hypertension in Spain: insights from the Spanish registry. The European Respiratory Journal. 2012;40(3):596-603. https://doi.org/10.1183/09031936.00101211
Hoeper MM, Huscher D, Ghofrani HA, Delcroix M, Distler O, Schweiger C, Grunig E, Staehler G, Rosenkranz S, Halank M, Held M, Grohé C, Lange TJ, Behr J, Klose H, Wilkens H, Filusch A, Germann M, Ewert R, Seyfarth HJ, Olsson KM, Opitz CF, Gaine SP, Vizza CD, Vonk-Noordegraaf A, Kaemmerer H, Gibbs JS, Pittrow D. Elderly patients diagnosed with idiopathic pulmonary arterial hypertension: results from the COMPERA registry. International Journal of Cardiology. 2013;168(2):871-880. https://doi.org/10.1016/j.ijcard.2012.10.026
Hoeper MM, Kramer T, Pan Z, Eichstaedt CA, Spiesshoefer J, Benjamin N, Olsson KM, Meyer K, Vizza CD, Vonk-Noordegraaf A, Distler O, Opitz C, Gibbs JSR, Delcroix M, Ghofrani HA, Huscher D, Pittrow D, Rosenkranz S, Grünig E. Mortality in pulmonary arterial hypertension: prediction by the 2015 European pulmonary hypertension guidelines risk stratification model. The European Respiratory Journal. 2017;50(2):1700740. https://doi.org/10.1183/13993003.00740-2017
Zhang R, Dai LZ, Xie WP, Yu ZX, Wu BX, Pan L, Yuan P, Jiang X, He J, Humbert M, Jing ZC. Survival of Chinese patients with pulmonary arterial hypertension in the modern treatment era. Chest. 2011;140(2):301-309. https://doi.org/10.1378/chest.10-2327
Chazova IE, Arhipova OA, Valieva ZS, Nakonechnikov SN, Martynyuk TV. Pulmonary hypertension on Russia: The first results of national register. Terapevticheskii arkhiv. 2014;86(9):56-64. (In Russ.). https://doi.org/10.26442/00403660.2019.01.000024
Taran IN, Belevskaya AA, Valieva ZS, Saidova MA, Martnyuk TV. “Portrait” of patients with idiopathic pulmonary hypertension and chronic thromboembolic pulmonary hypertension depending on comorbid status: current and prognosis features. Pulmonologia. 2020;30(4):427-436. (In Russ.). https://doi.org/10.18093/0869-0189-2020-30-4-427-436
Strange G, Lau EM, Giannoulatou E, Corrigan C, Kotlyar E, Kermeen F, Williams T, Celermajer DS, Dwyer N, Whitford H, Wrobel JP, Feenstra J, Lavender M, Whyte K, Collins N, Steele P, Proudman S, Thakkar V, Keating D, Keogh A; PHSANZ Registry. Survival of Idiopathic Pulmonary Arterial Hypertension Patients in the Modern Era in Australia and New Zealand. Heart, Lung and Circulation. 2018;27(11):1368-1375. https://doi.org/10.1016/j.hlc.2017.08.018