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Noninvasively Assessed Pulmonary Artery Stiffness Predicts Mortality in Pulmonary Arterial Hypertension^*

^* From the Departments of Pulmonary Diseases (Mr. Gan and Drs. Becker, Boonstra, Vonk-Noordegraaf, and Postmus), Physiology (Dr. Westerhof), Physics and Medical Technology (Mr. Lankhaar and Dr. Marcus), and Clinical Epidemiology and Biostatistics (Dr. Twisk), Institute for Cardiovascular Research, VU University Medical Center, Amsterdam, the Netherlands.

Correspondence to: Anton Vonk-Noordegraaf, MD, PhD, FCCP, Department of Pulmonary Diseases, VU University Medical Center, De Boelelaan 1117, PO Box 7057, 1007 MB Amsterdam, the Netherlands; e-mail: a.vonk{at}vumc.nl

Abstract

Aims: Decreased total compliance of the pulmonary vascular bed is associated with increased mortality in patients with pulmonary arterial hypertension (PAH). We investigated whether proximal pulmonary artery stiffness, in terms of area distensibility and noninvasively assessed relative area change (RAC), calculated as relative cross-sectional area change, predicts mortality in patients with PAH.

Methods and results: Eighty-six subjects underwent right-heart catheterization and MRI to assess area distensibility and RAC. Patients were followed up to 48 months. Kaplan-Meier plot and Cox proportional hazards regression analyses assessed the predictive value of area distensibility and RAC. In 70 patients, the diagnosis PAH was confirmed, and 16 subjects served as control subjects. In comparison with control subjects, proximal pulmonary arteries of patients were distended (685 ± 214 mm² vs 411 ± 153 mm², p < 0.001), less distensible (area distensibility = 0.46 ± 0.38·10^–2 mm Hg^–1 vs 3.69 ± 1.96·10^–2 mm Hg^–1, p < 0.0001), and RAC was smaller (20 ± 10% vs 58 ± 21%, p < 0.0001) [mean ± SD]. RAC showed an inverse curvilinear relation with mean pulmonary artery pressure (R² = 0.47). Eighteen patients (26%) died because of cardiopulmonary causes. Patients with a pulmonary artery RAC 16% had a worse prognosis than those with a value > 16% (log-rank p < 0.001). RAC predicted mortality better than area distensibility.

Conclusion: Noninvasively measured pulmonary artery RAC predicts mortality in patients with PAH.

Key Words: MRI • prognosis • pulmonary arterial hypertension • pulmonary artery stiffness