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

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.