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Updated: Jan 30, 2026
Left Atrial Stenosis Induced Pulmonary Venous Arterialization and Group 2 Pulmonary Hypertension in Rat
Published on: November 18, 2018
Pulmonary Artery Remodelling Assessed by Four-Dimensional Flow Magnetic Resonance Imaging in Pulmonary Arterial
Estibaliz Valdeolmillos1,2, Emmanuelle Fournier1,2, Hichem Sakhi1,3,4
1Department of Congenital Heart Diseases, Reference Center for Complex Congenital Disease M3C, Rare Disease Ntework, Hôpital Marie Lannelongue, Le Plessis-Robinson, France.
Background:
Pulmonary artery (PA) stiffness plays a significant role on right ventricular (RV) function, as evidenced in pulmonary arterial hypertension (PAH). In patients with atrial septal defect-related PAH (ASD-PAH), prognosis is dependent on RV function. We aimed to characterize PA remodelling and its relationship with RV function using 4-dimensional flow magnetic resonance imaging (4D flow MRI) in patients with ASD-PAH.
Methods:
We prospectively included 24 adults with ASD-PAH. They underwent 4D flow MRI and right heart catheterization within 24-48 hours. Remodelling of PA was analysed (stiffness, compliance, distensibility, elastance, and pulsatility) and PA vortex was evaluated. RV adaptation was measured by RV ejection fraction and RV-PA coupling.
Results:
The median age was 50 [41-55] years, and the median mean PA pressure and pulmonary vascular resistance were 50 [39-58] mm Hg and 8.0 [6.8-11.3] WU, respectively. Fourteen patients (58.3%) had RV dysfunction (RV ejection fraction <45%). All presented PA dilation (median main PA diameter: 42 [37-48] mm) and an increase in PA stiffness (10 [7.4-15.3]), whereas compliance and distensibility were decreased (2.1 [1.8-4.1] mm2/mm Hg and 0.19 [0.13-0.29] %/mm Hg). No correlation was found between PA dilation and indexes of PA remodelling. PA remodelling parameters were more severely impaired in patients with preserved RV function, with an increased stiffness (P = 0.01) and a decreased compliance and distensibility (P = 0.02).
Conclusions:
PA stiffness parameters characterized by 4D flow MRI were impaired in the population with ASD-PAH, and PA remodelling appears to be influenced by volumetric overload from the left-to-right shunt through the ASD.
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