{"title":"肺动脉高压和房间隔缺损的四维血流磁共振评价肺动脉重构","authors":"Estibaliz Valdeolmillos MD , Emmanuelle Fournier MD , Hichem Sakhi MD , Paul Vignaud MD , Marion Audié MD, MSc , Marc-Antoine Isorni MD , Bastien Provost MD , Florence Lecerf PhD student , Clément Batteux MD, PhD , Grégoire Albenque MD , Olivier Sitbon MD, PhD , David Montani MD, PhD , Xavier Jais MD, PhD , Laurent Savale MD, PhD , Marc Humbert MD, PhD , Arshid Azarine MD , Sébastien Hascoët MD, PhD, FESC","doi":"10.1016/j.cjcpc.2025.03.004","DOIUrl":null,"url":null,"abstract":"<div><h3>Background</h3><div>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.</div></div><div><h3>Methods</h3><div>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.</div></div><div><h3>Results</h3><div>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] mm<sup>2</sup>/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 (<em>P</em> = 0.01) and a decreased compliance and distensibility (<em>P</em> = 0.02).</div></div><div><h3>Conclusions</h3><div>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.</div></div>","PeriodicalId":100249,"journal":{"name":"CJC Pediatric and Congenital Heart Disease","volume":"4 4","pages":"Pages 189-197"},"PeriodicalIF":0.0000,"publicationDate":"2025-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":"{\"title\":\"Pulmonary Artery Remodelling Assessed by Four-Dimensional Flow Magnetic Resonance Imaging in Pulmonary Arterial Hypertension and Atrial Septal Defect\",\"authors\":\"Estibaliz Valdeolmillos MD , Emmanuelle Fournier MD , Hichem Sakhi MD , Paul Vignaud MD , Marion Audié MD, MSc , Marc-Antoine Isorni MD , Bastien Provost MD , Florence Lecerf PhD student , Clément Batteux MD, PhD , Grégoire Albenque MD , Olivier Sitbon MD, PhD , David Montani MD, PhD , Xavier Jais MD, PhD , Laurent Savale MD, PhD , Marc Humbert MD, PhD , Arshid Azarine MD , Sébastien Hascoët MD, PhD, FESC\",\"doi\":\"10.1016/j.cjcpc.2025.03.004\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"<div><h3>Background</h3><div>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.</div></div><div><h3>Methods</h3><div>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.</div></div><div><h3>Results</h3><div>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] mm<sup>2</sup>/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 (<em>P</em> = 0.01) and a decreased compliance and distensibility (<em>P</em> = 0.02).</div></div><div><h3>Conclusions</h3><div>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.</div></div>\",\"PeriodicalId\":100249,\"journal\":{\"name\":\"CJC Pediatric and Congenital Heart Disease\",\"volume\":\"4 4\",\"pages\":\"Pages 189-197\"},\"PeriodicalIF\":0.0000,\"publicationDate\":\"2025-08-01\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"CJC Pediatric and Congenital Heart Disease\",\"FirstCategoryId\":\"1085\",\"ListUrlMain\":\"https://www.sciencedirect.com/science/article/pii/S2772812925000284\",\"RegionNum\":0,\"RegionCategory\":null,\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"\",\"PubModel\":\"\",\"JCR\":\"\",\"JCRName\":\"\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"CJC Pediatric and Congenital Heart Disease","FirstCategoryId":"1085","ListUrlMain":"https://www.sciencedirect.com/science/article/pii/S2772812925000284","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"","JCRName":"","Score":null,"Total":0}
Pulmonary Artery Remodelling Assessed by Four-Dimensional Flow Magnetic Resonance Imaging in Pulmonary Arterial Hypertension and Atrial Septal Defect
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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