Effectiveness of PAH Specific Treatment Is Comparable Between PAH Associated With Connective Tissue Disease and Idiopathic PAH: Data From the Multicentre National Registry

Anna Smukowska-Gorynia, Sylwia Iwańczyk, Michał Ciurzyński, Marcin Waligóra, Marcin Kurzyna, Piotr Pruszczyk, Grzegorz Kopeć, Katarzyna Mizia-Stec, Ilona Skoczylas, Łukasz Chrzanowski, Piotr Błaszczak, Katarzyna Ptaszyńska, Grzegorz Grześk, Ewa Malinowska, Małgorzata Peregud-Pogorzelska, Michał Tomaszewski, Ewa Mroczek, Miłosz Jaguszewski, Ewa Lewicka, Katarzyna Widejko, Agnieszka Pawlak, Wojciech Jacheć, Zbigniew Gąsior, Katarzyna Betkier-Lipińska, Judyta Winowska-Józwa, Marta Banaszkiewicz, Tatiana Mularek-Kubzdela

https://doi.org/10.1002/pul2.70370 

Abstract

Pulmonary arterial hypertension (PAH) is a rare disease with poor prognosis. The same treatment is recommended in patients with PAH associated with connective tissue disease (CTD) and idiopathic PAH (IPAH). However, the effectiveness of PAH-specific treatment in these both groups is inconsistent. The study incorporated data from the prospective, multicentre polish registry (BNP-PL) by comparison of baseline versus 1-year follow-up clinical, laboratory, echocardiographic and hemodynamic parameters in PAH-CTD versus IPAH group. The all-cause 1-year mortality was also analysed. 624 patients were included in the study: 176 (28%) PAH-CTD and 448 (72%) IPAH. 48 (8% of the total 624) patients died during 1-year follow up: 32 (7%) with IPAH and 16 (9%) with PAH-CTD (p = 0.4). The effectiveness of treatment was examined in 421 patients: 115 (27%) PAH-CTD and 306 (73%) IPAH. The improvement of treatment was effective in both groups in terms of WHO FC (p < 0.001 in both groups), 4-strata risk model (p < 0.001 in both groups), 6 min walking test (6MWT, p < 0.001 in IPAH, p = 0.001 in PAH-CTD), Δ6MWT (p = 0.19 between PAH-CTD and IPAH), tricuspid annular plane systolic excursion (p < 0.001 in both groups), NT- proBNP (p < 0.001 in IPAH and p < 0.01 in PAH-CTD), mean pulmonary arterial pressure (p = 0.043 in IPAH and p = 0.01 in PAH-CTD), pulmonary vascular resistance (p < 0.001 in both groups) and cardiac output (p < 0.001 in IPAH and p = 0.034 in PAH-CTD). PAH specific treatment in PAH-CTD patients is as effective as in IPAH patients regarding improvement in WHO FC, 4-strata risk model, 6MWT, Δ6MWT, TAPSE, NT-proBNP, mPAP, PVR and CO at 1-year follow-up. 1-year all-cause mortality was not significantly different between PAH-CTD and IPAH patients.

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