Anatomical insights and management strategies for haemodynamically significant pressure-restrictive perimembranous ventricular septal defects: Findings from the French nationwide FRANCISCO cohort - 08/05/25

on behalf of
the FRANCISCO investigators
Graphical abstract |
Graphical Abstract Legend: Key anatomical features of pressure-restrictive perimembranous ventricular septal defects (pmVSD) — including membranous septal aneurysm size, defect proximity to the aortic cusp, and aortoseptal angle — were assessed to guide individualized management strategies. Treatment decisions between surgical or percutaneous closure versus monitoring were influenced by these anatomical and clinical factors in the French FRANCISCO cohort.
Graphical Abstract Legend: Key anatomical features of pressure-restrictive perimembranous ventricular septal defects (pmVSD) — including membranous septal aneurysm size, defect proximity to the aortic cusp, and aortoseptal angle — were assessed to guide individualized management strategies. Treatment decisions between surgical or percutaneous closure versus monitoring were influenced by these anatomical and clinical factors in the French FRANCISCO cohort.Le texte complet de cet article est disponible en PDF.
Highlights |
• | Study population: 212 patients aged>1year. |
• | Patients had haemodynamically significant pressure-restrictive pmVSD. |
• | A number of key factors drove closure decisions. |
• | Factors included larger defect size, symptoms and inlet/outlet extension. |
• | Factors also included greater aneurysm height, aortic regurgitation and prolapse. |
• | pmVSD closure rates varied across the 10 major regions in France. |
• | 26% of patients underwent pmVSD closure. |
• | Surgical closure was more common in children aged 1–2years. |
Abstract |
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>1year 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±11.2years; 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.
Conclusions |
In France, pmVSD closure in patients aged>1year lacks standardization, with decisions driven by symptoms, anatomical factors and individual centre protocols.
Le texte complet de cet article est disponible en PDF.Keywords : Congenital heart defect, Device closure, Outcomes, Perimembranous ventricular septal defect, Surgery
Abbreviations : CI, LV, LVEDD, MSA, OR, PAH, pmVSD, VSD
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