Vol 79, No 7-8 (2021)
Expert opinion
Published online: 2021-07-01

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An expert opinion of the Polish Cardiac Society Working Group on Pulmonary Circulation and the Polish Society for Rheumatology on the diagnosis and treatment of pulmonary hypertension in patients with connective tissue disease

Tatiana Mularek-Kubzdela1, Michał Ciurzyński2, Otylia Kowal Bielecka3, Jarosław D Kasprzak4, Grzegorz Kopeć5, Katarzyna Mizia-Stec6, Ewa Mroczek7, Ewa Lewicka8, Ilona Skoczylas9, Marek Grabka10, Michał Furdal11, Michał Florczyk12, Marek Brzosko13, Bogdan Batko14, Hanna Przepiera-Będzak13, Włodzimierz Samborski15, Eugeniusz J Kucharz16
Pubmed: 34227677
Kardiol Pol 2021;79(7-8):917-929.

Abstract

Systemic connective tissue diseases (CTDs) comprise a large group of diseases that are auto-immune in nature and characterized by the involvement of multiple systems and organs. Pul-monary hypertension (PH) of various etiologies may develop in the course of CTD, including pulmonary arterial hypertension (PAH), PH secondary to the lung disease, postcapillary PH in the course of left heart disease, and chronic thromboembolic pulmonary hypertension (CTEPH). In addition, the different forms of PH may coexist with each other. Among patients with CTD, PAH occurs most commonly in those with systemic sclerosis, where it affects ap-proximately 8%–12% of patients. The prognosis in patients with untreated PAH is very poor. It is particularly important to identify the high-risk CTD-PAH population and to perform effi-cient and accurate diagnostics so that targeted therapy of the pulmonary arteries can be intro-duced. Echocardiography is used to screen for PH, but clinical and echocardiographic suspicion of PH always requires confirmation by right heart catheterization. Confirmation of PAH ena-bles the initiation of life-prolonging pharmacological treatment in this group of patients, which should be administered in referral centers. Drugs available for pharmacological management include endothelin receptor antagonists, phosphodiesterase-5 inhibitors, and prostacyclins.




Polish Heart Journal (Kardiologia Polska)