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Publications

Journal Article Epublish

PubMed: 42516678 DOI: 10.1002/pul2.70370

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

Pulm Circ 16(3):e70370

Authors: 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

Affiliations

  • Department of Cardiology Poznan University of Medical Sciences Poznań Poland.
  • Department of Internal Medicine and Cardiology With the Center for Diagnosis and Treatment of Venous Thromboembolism Medical University of Warsaw Warsaw Poland.
  • Department of Cardiac and Vascular Diseases, John Paul II Hospital in KrCakow, 31-202 Krakow, Poland; Pulmonary Circulation Centre, Department of Cardiac and Vascular Diseases, Faculty of Medicine Jagiellonian University Medical College Krakow Poland.
  • Department of Pulmonary Circulation, Thromboembolic Diseases and Cardiology Centre of Postgraduate Medical Education, European Health Centre Otwock Otwock Poland.
  • First Department of Cardiology, I Chair of Cardiology Medical University of Silesia Katowice Poland.
  • Department of Cardiology Faculty of Medical Sciences in Zabrze, Medical University of Silesia Katowice Poland.
  • Cardiology Department Medical University of Lodz Lodz Poland.
  • Department of Cardiology Cardinal Wyszynski Hospital Lublin Poland.
  • Department of Cardiology Medical University of Bialystok Bialystok Poland.
  • Department of Cardiology and Clinical Pharmacology Collegium Medicum in Bydgoszcz, Nicolaus Copernicus University in Toruń Toruń Poland.
  • Pulmonary Department University of Warmia and Mazury Olsztyn Poland.
  • Department of Cardiology Pomeranian Medical University Szczecin Poland.
  • Department of Cardiology Medical University of Lublin Lublin Poland.
  • Department of Cardiology Institute of Heart Diseases, Wroclaw Medical University Wroclaw Poland.
  • Department of Cardiology Medical University of Gdansk Gdansk Poland.
  • Department of Cardiology and Electrotherapy Medical University of Gdansk Gdansk Poland.
  • Department of Cardiology Copper Health Center Lubin Poland.
  • Department of Invasive Cardiology, Polish Academy of Sciences, Mossakowski Medical Research Centre Central Clinical Hospital of the Ministry of Interior Warsaw Poland.
  • Department of Cardiology Faculty of Medical Sciences in Zabrze, Silesian Medical University in Katowice Zabrze Poland.
  • Department of Cardiology School of Health Sciences in Katowice, Medical University of Silesia in Katowice Katowice Poland.
  • Department of Cardiology and Internal Medicine Military Institute of Medicine-National Research Institute Warsaw Poland.
  • Department of Cardiology Samodzielny Publiczny Wojewódzki Szpital Zespolony Szczecin Poland.
  • Department of Vascular and Endovascular Surgery Angiology and Phlebology, Poznan University of Medical Sciences Poznan Poland.

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.

MeSH

Registries, Mortality, Connective Tissue, Connective Tissue Diseases, Therapeutics, Tissues