Résumé
Background: Management of haemodynamically significant pressure-restrictive perimembranous ventricular septal defects (pmVSDs) with left ventricular volume overload, but without pulmonary hypertension, is under debate.Aims: To describe pmVSD characteristics, and factors influencing closure decisions in France.Methods: FRANCISCO is a French cohort of patients aged > 1 year with isolated haemodynamically significant pressure-restrictive pmVSDs. Data collected at inclusion were analysed.Results: From 2018–2020, 212 patients from 38 centres were included: mean age 8.8 ± 11.2 years; 41% aged 1–2 years; 40% aged 3–15 years; 19% aged > 15 years. Mean defect diameter was 6 ± 3 mm; 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 1–2 years, 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 3–15 years, 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 > 15 years, only defect diameter (OR 1.3, 95% CI 1.3–1.8) was predictive of closure.Conclusions: In France, pmVSD closure in patients aged > 1 year lacks standardization, with decisions driven by symptoms, anatomical factors and individual centre protocols