Mariana M. Clavé1, Nair Y. Maeda2, Ana M. Thomaz1, Sergio P. Bydlowski3, Antonio A. Lopes1
Congenital Heart Disease, Vol.14, No.2, pp. 246-255, 2019, DOI:10.1111/chd.12688
Abstract Background: Ideally, vasodilator therapies for pulmonary arterial hypertension (PAH) should
have a favorable impact on markers of vascular dysfunction, in addition to their known effects
on hemodynamics, cardiac function, and patient’s physical capacity.
Methods: We analyzed circulating (plasma) markers of endothelial and platelet activation/dys‐
function (enzyme‐linked immunoassays) in the specific setting of advanced PAH associated
with congenital heart disease, during the course of sildenafil and tadalafil therapies. Thirty‐one
patients were enrolled (age 10‐54 years), most of them with chronic hypoxemia and elevated
hematocrit. Drugs were administered orally for 6 months (sildenafil [n = 16], 20 mg t.i.d.; tada‐
lafil [n… More >
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