Abstract

Objective.Vascular remodeling in patients with pulmonary artery hypertension (PAH) may be assessed by measurement of contrast magnetic-resonance imaging (CMRI) derived pulmonary artery distensibility (PAD) index. The objective of our study was to investigate whether PAD index could be used as a marker for the evaluation of PAH severity.Design and methods.Forty four patients with PAH (mean age — 42,8 ± 14,5 years, males:females = 11:33) were enrolled: 29 patients with idiopathic pulmonary arterial hypertension, 4 subjects with corrected congenital heart disease, 3 subjects with scleroderma PAH and 8 patients with inoperable chronic thromboembolic pulmonary hypertension. All patients underwent 6-minute walk test, right heart catheterization (RHC), heart ultrasound (ECHO), CMRI, cardiopulmonary exercise testing, and serum N-terminal-pro-brain natriuretic peptide (NT-proBNP) level was defined.Results.PAD index was decreased in PAH patients 11,9 % (9,3-19,7). Patients with decreased PAD index < 20 % had higher NT-proBNP level, lower tricuspid annular systolic velocity and right ventricle/left ventricle ratio by ECHO. Patients with PAD index < 20 % had higher pulmonary artery systolic blood pressure (96,5 ± 22,4 versus 77,9 ± 19,4 mmHg; p < 0,05), and reduced cardiac output which were determined by RHC (3,98 ± 1,1 versus 4,95 ± 1,21 l/min; p < 0,05). Decreased VO2peak was found in patients with PAD index < 20 % (14,8 versus 18,8 ml/min/kg; p = 0,05).Conclusions.PA distensibility index may be used for noninvasive assessment of PAH severity and progression.

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