Background: Idiopathic pulmonary artery (PA) hypertension (IPAH) is associated with severe PA remodelling. Although the resulting increase in pulse wave velocity (PWV) might be of major pathophysiological relevance, little is known about PA-PWV in IPAH. The aim of this study was to characterize PA-PWV and its predictors in patients with IPAH.
Methods: We studied 26 consecutive patients with incident IPAH aged 55.0 (45.0-66.0) years (62% female) and 10 control subjects without pulmonary hypertension. PA-PWV was measured invasively; PA wall thickness and diameter were assessed using intravascular and transthoracic ultrasonography.
Results: PA-PWV was higher in IPAH than in control subjects (10.0 [7.5-14.0] m/s vs 3.5 [1.9-4.0] m/s; P < 0.001) as was also PA diameter and PA wall thickness. In IPAH patients, in univariate analysis PA-PWV was greater in men than in women and in patients with body mass index (BMI) < 25 kg/m(2) than with BMI ≥ 25 kg/m(2) and correlated positively with symptomatic disease duration, mean PA pressure, pulmonary vascular resistance, creatinine level, and negatively with low-density lipoprotein (LDL) cholesterol and triglyceride level but not with PA diameter or PA wall thickness. In multiple regression analysis mean PA pressure, LDL cholesterol level and BMI < 25 kg/m(2) were the main predictors of PA-PWV in IPAH patients (R(2) = 77%; P < 0.001).
Conclusions: PA-PWV is increased in IPAH patients. High PA pressure, low LDL cholesterol level, and BMI < 25 kg/m(2) explain most of its variability in this group.
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