Characterization of Patients with Pulmonary Arterial Hypertension: Data from the Polish Registry of Pulmonary Hypertension (BNP-PL)

• Main Authors: Grzegorz Kopeć, Marcin Kurzyna, Ewa Mroczek, Łukasz Chrzanowski, Tatiana Mularek-Kubzdela, Ilona Skoczylas, Beata Kuśmierczyk, Piotr Pruszczyk, Piotr Błaszczak, Ewa Lewicka, Danuta Karasek, Katarzyna Mizia-Stec, Michał Tomaszewski, Wojciech Jacheć, Katarzyna Ptaszyńska-Kopczyńska, Małgorzata Peregud-Pogorzelska, Anna Doboszyńska, Agnieszka Pawlak, Zbigniew Gąsior, Wiesława Zabłocka, Robert Ryczek, Katarzyna Widejko-Pietkiewicz, Marcin Waligóra, Szymon Darocha, Michał Furdal, Michał Ciurzyński, Jarosław D. Kasprzak, Marek Grabka, Karol Kamiński, Piotr Hoffman, Piotr Podolec, Adam Torbicki
• Format: Article
• Language: English
• Published: MDPI AG 2020-01-01
• Series: Journal of Clinical Medicine
• Subjects: pulmonary arterial hypertension; registry; epidemiology; prevalence; incidence
• Online Access: https://www.mdpi.com/2077-0383/9/1/173

Current knowledge of pulmonary arterial hypertension (PAH) epidemiology is based mainly on data from Western populations, and therefore we aimed to characterize a large group of Caucasian PAH adults of Central-Eastern European origin. We analyzed data of incident and prevalent PAH adults enrolled in a prospective national registry involving all Polish PAH centers. The estimated prevalence and annual incidence of PAH were 30.8/mln adults and 5.2/mln adults, respectively and they were the highest in females &#8805;65 years old. The most frequent type of PAH was idiopathic (<i>n</i> = 444; 46%) followed by PAH associated with congenital heart diseases (CHD-PAH, <i>n</i> = 356; 36.7%), and PAH associated with connective tissue disease (CTD-PAH, <i>n</i> = 132; 13.6%). At enrollment, most incident cases (71.9%) were at intermediate mortality risk and the prevalent cases had most of their risk factors in the intermediate or high risk range. The use of triple combination therapy was rare (4.7%). A high prevalence of PAH among older population confirms the changing demographics of PAH found in the Western countries. In contrast, we found: a female predominance across all age groups, a high proportion of patients with CHD-PAH as compared to patients with CTD-PAH and a low use of triple combination therapy.