Assesment of factors for late diagnosis of idiopathic pulmonary arterial hypertension in real clinical practice

Idiopathic (primary) pulmonary arterial hypertension – a rare progressive disease that shortens life, usually diagnosed at a late stage. Therefore, it is critical to have a low threshold for suspicion of pulmonary hypertension (PH) and to refer patients to specialized centers for diagnostic evaluation and treatment as early as possible. Echocardiography is the method of choice for pulmonary hypertension assessing.
Aim of the study. To determine the reason for the late diagnosis of idiopathic pulmonary arterial hypertension.
Material and methods. Using the developed questionnaire, 41 patients, men and women aged 18 years and older, who were reliably diagnosed with idiopathic pulmonary arterial hypertension, were interviewed.
Results and discussion. The diagnosis of idiopathic pulmonary arterial hypertension in clinical practice is made on average 7.1±6.0 years from the onset of the first symptoms of the disease. In 95% of cases, the onset of idiopathic pulmonary arterial hypertension begins with shortness of breath on exertion. Most often, the development of the disease was preceded by an acute respiratory viral infection. All patients underwent diagnostic echocardiography at the time of their first visit, but the diagnosis was established in only 56% of patients.
Conclusions. Reasons for late diagnosis of idiopathic pulmonary arterial hypertension: the nature of the disease at its onset and errors in diagnosis.

1. Chazova I. E. Legochnaja arterial'naja gipertenzija v Rossii: analiz shestiletnego nabljudenija po dannym Nacional'nogo registra /I. E. Chazova, O. A. Arhipova, T. V. Martynjuk //Terapevt. arh. – 2019. – №1. – S. 24-31.

2. A global view of pulmonary hypertension /M. M. Hoeper, M. Humbert, R. Souza et al. //Lancet Respir. Med. – 2016. – V. 4 (4). – Pp. 306-322.

3. Evidence-based detection of pulmonary arterial hypertension in systemic sclerosis: the DETECT study /J. G. Coghlan, C. P. Denton, E. Grunig et al. //Ann. Rheum. Dis. – 2014. – V. 73 (7). – Pp. 1340-1349.

4. Haddad R. N. An evidence-based approach to screening and diagnosis of pulmonary hypertension /R. N. Haddad, L. M. Mielniczuk //J. Cardiol. – 2015. – V. 31 (4). – Pp. 382-390.

5. Levine D. J. Pulmonary arterial hypertension: updates in epidemiology and evaluation of patients // Am. J. Manag. Care. – 2021. – V. 27 (3). – Pp. 35-41.

6. Molecular Analysis of BMPR2, TBX4, and KCNK3 and Genotype-Phenotype Correlations in Spanish Patients and Families With Idiopathic and Hereditary Pulmonary Arterial Hypertension / P. Navas, J. Tenorio, C. A. Quezada et al. //Rev. Esp. Cardiol. – 2016. – V. 69 (11). – Pp. 1011-1019.

7. Pulmonary arterial hypertension in France: results from a national registry /M. Humbert, O. Sitbon, A. Chaouat et al. //Am. J. Respir. Crit. Care. – 2006. – V. 173 (9). – Pp. 1023-1030.

8. Ruopp N. F. Diagnosis and Treatment of Pulmonary Arterial Hypertension: A Review / N. F. Ruopp, B. A. Cockrill //JAMA. – 2022. – V. 327 (14). – Pp. 1379-1391.