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

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.

Keywords: Congenital heart defect; Device closure; Outcomes; Perimembranous ventricular septal defect; Surgery.