Features of the clinical course and prognosis of idiopathic pulmonary hypertension in patients depending of comorbidities

Idiopathic pulmonary hypertension (IPAH) nowadays frequently diagnosed in older patients. Consequently, comorbidity increased, complicating patient risk assessment and diagnosis, and influencing therapeutic decisions. This article focuses on comparing clinical, instrumental and demographic parameters, survival rates, and prognosis in patients with “classical” IPAH and those with comorbidities.

Materials and Methods. The study included patients (n=56) with a verified diagnosis of IPAH between 2014 and 2023. All participants were divided into two groups: patients with IPAH without comorbidities (n=26) — Group 1; and patients with IPAH and comorbidities (n=30) — Group 2. The following examinations were performed: 6 minute walk test, echocardiography, right heart catheterization, NTproBNP level assessment, and clinical and biochemical blood tests. Mortality risk assessment was conducted at the time of diagnosis, after 1 year, and after 3 years of therapy using risk stratification scales.

Results. Comorbid patients were older: 46,5 [35,2; 53,0] years vs. 33,0 [28,0; 38,0] years, p=0,001. At time of diagnosis, they had higher body weight and systolic blood pressure values, reflecting the presence of comorbidities. After 36 months, statistically significant differences between the groups were observed in: 6MWT distance (p=0,04); NT-proBNP levels (p=0,014); pulmonary artery systolic pressure ( p=0,014), mean pulmonary artery pressure (p=0,01), mixed venous oxygen saturation (p=0,009), right atrial pressure (p=0,033), pulmonary vascular resistance (p=0,012). Survival rates at 1, 3, and 5 years were 96,1%, 92,3%, and 92,3% respectively in Group 1, . In Group 2, survival rates were 96,7% at 1 year, 86,7% at 3 years, and 73,3% at 5 years (p=0,022).

Conclusion. The increasing proportion of comorbidity patients with IPAH in the population affects the diagnosis and treatment of this patient group, their survival rates, and response to therapy. For a more accurate risk assessment, it is already necessary to include the comorbidity profile in the list of parameters evaluated during risk stratification.

1. Humbert M., Kovacs G., Hooper M. M., et al. 2022 ESC/ ERS Guidelines for the diagnosis and treatment of pulmonary hypertension. European Heart Journal. 2022;43 (38):3618–3731. https://doi.org/10.1093/eurheartj/ehac237

2. Galiè N., Barberà J. A., Frost A. E., et al. Initial Use of Ambrisentan plus Tadalafil in Pulmonary Arterial Hypertension. The New England Journal of Medicine. 2015;373(9):834–844. https://doi.org/10.1056/NEJMoa1413687

3. Opitz C. F., Hoeper M. M., Gibbs J. S. R. et al. Pre-capillary, combined, and post-capillary pulmonary hypertension: a pathophysiological continuum. Journal of the American College of Cardiology. 2016;68(4):368-378. https://doi.org/10.1016/j.jacc.2016.05.047

4. Paramonov V.M., Martynyuk T.V., Dadacheva Z.Kh., Danilov N.M., Masenko V.P., Chazova I.E. Clinical and hemodynamic profile and neurohumoral parameters in patients with idiopathic pulmonary hypertension depending on the results of an acute pharmacological test. Eurasian Heart Journal. 2016;(1):12–20. (In Russ.) https://doi.org/10.38109/2225-1685-2016-1-12-20

5. Pulmonary hypertension, including chronic thromboembolic pulmonary hypertension: Russian Federation Clinical Guidelines 2024 (Russia). ID: 159. URL: (accessed: 25.09.2025) (In Russ.)

6. Taran I.N., Belevskaya A.A., Valieva Z.S., Saidova M.A., Martynyuk T.V. “Portrait” of patients with idiopathic pulmonary hypertension and chronic thromboembolic pulmonary hypertension depending on comorbidity status: disease course and prognosis features. Pulmonologiya (Russian Pulmonology Journal). 2020;30(4):427- 436. (In Russ.) https://doi.org/10.18093/0869-0189-2020-30-4-427-436

7. 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

8. Chazova I.E., Arkhipova O.A., Valieva Z.S., Nakonechnikov S.N., Martyniuk T.V. Pulmonary hypertension in Russia: The first results of the national register. Terapevticheskii arkhiv. 2014;86(9):56-64. (In Russ.)

9. Rosenkranz S, Ghofrani H. A, Grunig E, et al. Cologne consensus ̈ conference on pulmonary hypertension – Update 2018. Int J Cardiol. 2018;272S:1-3. https://doi.org/10.1016/j.ijcard.2018.09.064

10. Hjalmarsson C, Radegran G, Kylhammar D, et al. Impact of age ̊ and comorbidity on risk stratification in idiopathic pulmonary arterial hypertension. Eur Respir J 2018; 51:1702310. https://doi.org/10.1183/13993003.02310-2017

11. Bravo-Marques R, Rivas-Ruiz F., Tenes J A., et.al, Investigators of the Spanish Pulmonary Arterial Hypertension Registry (REHAP), The importance of comorbidities in idiopathic and associated with connective tissue disease pulmonary arterial hypertension: from the Spanish Registry of Pulmonary Hypertension (REHAP Registry). European Heart Journal. October 2024;45(1):ehae666.2170. https://doi.org/10.1093/eurheartj/ehae666.2170

12. Kianzad A, van Wezenbeek J, Celant L.R, et al. Idiopathic pulmonary arterial hypertension patients with a high H2FPEF-score: insights from the Amsterdam UMC PAH-cohort. J Heart Lung Transplant 2022;41:1075-1085. https://doi.org/10.1016/j.healun.2022.05.007

13. McLaughlin V.V., Vachiery J.L, Oudiz R.J et.al. Patients with pulmonary arterial hypertension with and without cardiovascular risk factors: Results from the AMBITION trial&. The Journal of Heart and Lung Transplantation. 2019 Dec;38(12):1286-1295. ISSN 1053-2498. https://doi.org/10.1016/j.healun.2019.09.010

14. Shmalts A.A., Chernogrivov I.E. Post capillary pulmonary hypertension: diagnosis and treatment prospects. Creative Cardiology. 2025;19(2):131-145. (In Russ.) https://doi.org/10.24022/1997-3187-2025-19-2-131-145