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

作者信息

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

机构信息

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.

出版信息

Arch Cardiovasc Dis. 2025 Jun-Jul;118(6-7):391-399. doi: 10.1016/j.acvd.2025.02.014. Epub 2025 Apr 14.

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.