Sudaka Annabel, Thambo Jean-Benoit, Vaksmann Guy, Hadeed Khaled, Houeijeh Ali, Khraiche Diala, Hery Eric, Vincenti Marie, Pangaud Nicolas, Benbrik Nadir, Vastel Carine, Legendre Antoine, Jalal Zakaria, Laux Daniela, Harchaoui Samir, Lambert Virginie, Chalard Aurélie, Douchin Stéphanie, Bouzguenda Ivan, Denis Charlotte, Urbina-Hiel Bérangère, Bonnemains Laurent, Mostefa-Kara Meriem, Ansquer Hélène, Léty Sophie, Leborgne Anne-Sophie, Cohen Laurence, Lupoglazoff Jean Marc, Guirgis Maurice, Maragnes Pascale, Moceri Pamela, Bertail Claire, Lefort Bruno, Ovaert Caroline, Haddad Raymond N, Guirgis Lisa, Hascoet Sébastien
Department of Paediatric and Adult Congenital Heart Diseases, M3C network for Complex Congenital Heart Disease, Marie Lannelongue Hospital, Paris-Saclay University, 92350 Le Plessis-Robinson, France; INSERM UMR_S 999 " Pulmonary hypertension: pathophysiology and novel therapies ", Marie Lannelongue hospital and Bicêtre Hospital, 92350, le Plessis-Robinson, France.
Department of Paediatric Cardiology, M3C network for Complex Congenital Heart Disease, Haut-Lévêque University Hospital, 33604 Pessac, France.
Arch Cardiovasc Dis. 2025 Jun-Jul;118(6-7):391-399. doi: 10.1016/j.acvd.2025.02.014. Epub 2025 Apr 14.
Management of haemodynamically significant pressure-restrictive perimembranous ventricular septal defects (pmVSDs) with left ventricular volume overload, but without pulmonary hypertension, is under debate.
To describe pmVSD characteristics, and factors influencing closure decisions in France.
FRANCISCO is a French cohort of patients aged>1year with isolated haemodynamically significant pressure-restrictive pmVSDs. Data collected at inclusion were analysed.
From 2018-2020, 212 patients from 38 centres were included: mean age 8.8±11.2years; 41% aged 1-2years; 40% aged 3-15years; 19% aged>15years. Mean defect diameter was 6±3mm; 77% had membranous aneurysms, 9% inlet/outlet extension, 3% aortic cusp prolapse and 8% aortic regurgitation. Closure (transcatheter or surgical) occurred in 54 patients (26%). Defect closure rates varied across the 10 major regions in France. Closure was associated with larger defect diameter (odds ratio [OR] 1.5, 95% confidence interval [CI] 1.3-1.7), inlet/outlet extension (OR 3.5, 95% CI 1.4-9.1), greater aneurysm height (OR 1.3, 95% CI 1.1-1.5), aortic regurgitation (OR 4.5, 95% CI 1.6-12.8) and prolapse (OR 8.3, 95% CI 1.6-44.4). In those aged 1-2years, closure was driven by dyspnoea (OR 4.9, 95% CI 1.6-15.2) and defect diameter (OR 1.6, 95% CI 1.2-1.6). In those aged 3-15years, key factors included defect diameter (OR 1.5, 95% CI 1.2-1.9), aortic regurgitation (OR 7.4, 95% CI 1.6-33.8), aneurysm height (OR 1.5, 95% CI 1.1-2.0) and inlet/outlet extension (OR 9.5, 95% CI 2.1-42.8). In those aged>15years, only defect diameter (OR 1.3, 95% CI 1.3-1.8) was predictive of closure.
In France, pmVSD closure in patients aged>1year lacks standardization, with decisions driven by symptoms, anatomical factors and individual centre protocols.
对于血流动力学显著的压力限制性膜周部室间隔缺损（pmVSD）伴左心室容量负荷过重但无肺动脉高压的治疗存在争议。
描述法国pmVSD的特征以及影响封堵决策的因素。
FRANCISCO是一项针对年龄大于1岁的孤立性血流动力学显著的压力限制性pmVSD患者的法国队列研究。对纳入时收集的数据进行分析。
2018年至2020年，来自38个中心的212例患者被纳入：平均年龄8.8±11.2岁；41%年龄在1至2岁；40%年龄在3至15岁；19%年龄大于15岁。平均缺损直径为6±3mm；77%有膜部瘤，9%有流入道/流出道延伸，3%有主动脉瓣叶脱垂，8%有主动脉反流。54例患者（26%）进行了封堵（经导管或手术）。法国10个主要地区的缺损封堵率各不相同。封堵与较大的缺损直径（优势比[OR]1.5，95%置信区间[CI]1.3 - 1.7）、流入道/流出道延伸（OR 3.5，95% CI 1.4 - 9.1）、更大的瘤体高度（OR 1.3，95% CI 1.1 - 1.5）、主动脉反流（OR 4.5，95% CI 1.6 - 12.8）和脱垂（OR 8.3，95% CI 1.6 - 44.4）相关。在1至2岁的患者中，封堵的驱动因素是呼吸困难（OR 4.9，95% CI 1.6 - 15.2）和缺损直径（OR 1.6，95% CI 1.2 - 1.6）。在3至15岁的患者中，关键因素包括缺损直径（OR 1.5，95% CI 1.2 - 1.9）、主动脉反流（OR 7.4，95% CI 1.6 - 33.8）、瘤体高度（OR 1.5，95% CI 1.1 - 2.0）和流入道/流出道延伸（OR 9.5，95% CI 2.1 - 42.8）。在年龄大于15岁的患者中，只有缺损直径（OR 1.3，95% CI 1.3 - 1.8）可预测封堵。
在法国，年龄大于1岁的pmVSD患者的封堵缺乏标准化，决策由症状、解剖因素和各中心方案驱动。
