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Original scientific paper

Molecular mechanisms involved in pulmonary arterial hypertension development

DRAGAN MANOJLOVIĆ orcid id orcid.org/0000-0002-8652-5283 ; General Hospital Vukovar, Department for Vascular Surgery, Vukovar, Croatia
INES DRENJANČEVIĆ orcid id orcid.org/0000-0003-4964-7721 ; Department of Physiology and Immunology, Faculty of Medicine, University J.J. Strossmayer Osijek, J. Huttlera 4, 31000 Osijek, Croatia


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Abstract

Pulmonary arterial hypertension (PAH) is an elevation in pulmonary
arterial pressure, characterized by symptoms of dyspnea, chest pain, decrease in exercise tolerance-fatigue, syncope and, if untreated, PAH leads to right heart failure.

In PAH, there is an imbalance between mediators of vasodilation and
vasoconstriction (e.g. nitric oxide and prostacycline – potent vasodilators, platelet inhibitor and antimitogens are decreased in PAH, while thromboxane, vasoconstrictor and platelet activator is increased in PAH, resulting in smooth muscle hypertrophy of small vessels, adventitial and intimal proliferation, and plexiform vascular lesions with vascular thrombosis). Standard diagnostic procedures for PAH include physical examination, pulmonary function testing, radiographic imaging, transthoracic echocardiography, right heart catheterization. Current drugs include synthet c prostanoids (iloprost, epoprostenil, beraprost, treprostinil) – vasodilators and antiplatelet
agents. Phosphodiesterase-5 inhibitors decrease the breakdown of
cGMP, increasing its intracellular levels, leukotriene receptor antagonist, – zafirlukast, decreases pulmonary arterial and venous pressure. Endothelin receptor blockers, bosentan, decrease pulmonary vascular resistance and improve results of functional tests. Other treatments are: anticoagulants, calcium-channel blockers, positive airway pressure therapy for obstructive sleep apnea, or oxygen for hypoxemia, and surgery. In conclusion, although there are some promising drugs in therapy of PAH, there is a need to develop new ones, together with surgical approaches, in order to increase the survival of patients with PAH. Gene and cell therapy could be expected as future perspectives.

Keywords

Hrčak ID:

74095

URI

https://hrcak.srce.hr/74095

Publication date:

31.10.2011.

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