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

Doi : 10.1016/j.acvd.2025.02.014 
Annabel Sudaka a, af, Jean-Benoit Thambo b, Guy Vaksmann c, Khaled Hadeed d, Ali Houeijeh e, Diala Khraiche f, Eric Hery g, Marie Vincenti h, Nicolas Pangaud i, Nadir Benbrik j, Carine Vastel k, Antoine Legendre l, Zakaria Jalal b, Daniela Laux a, m, Samir Harchaoui n, Virginie Lambert a, o, Aurélie Chalard p, Stéphanie Douchin q, Ivan Bouzguenda c, Charlotte Denis r, Bérangère Urbina-Hiel s, Laurent Bonnemains t, Meriem Mostefa-Kara a, Hélène Ansquer u, Sophie Léty v, Anne-Sophie Leborgne w, Laurence Cohen x, Jean Marc Lupoglazoff y, Maurice Guirgis z, Pascale Maragnes aa, Pamela Moceri ab, Claire Bertail ac, Bruno Lefort ad, Caroline Ovaert ae, Raymond N. Haddad a, af, Lisa Guirgis a, af, Sébastien Hascoet a, af,
on behalf of

the FRANCISCO investigators

a 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 
b Department of Paediatric Cardiology, M3C network for Complex Congenital Heart Disease, Haut-Lévêque University Hospital, 33604 Pessac, France 
c Department of Paediatric Cardiology, La Louvière Hospital, 59800 Lille, France 
d Department of Paediatric Cardiology, M3Cnetwork for Complex Congenital Heart Disease, Children's hospital, Toulouse University Hospital, 31059 Toulouse, France 
e Department of Paediatric Cardiology, M3C network for Complex Congenital Heart Disease, Lille University Hospital, 59000 Lille, France 
f Department of Congenital and Paediatric Cardiology, M3C network for Complex Congenital Heart Disease, Hôpital Universitaire Necker-Enfants Malades, AP–HP, 75015 Paris, France 
g Department of Cardiology, Sainte-Marie Hospital, 95520 Osny, France 
h Department of Paediatric and Congenital Cardiology, M3C network for Complex Congenital Heart Disease, Montpellier University Hospital, PhyMedExp, INSERM, CNRS, 34295 Montpellier, France 
i Department of Paediatric Cardiology, Val d’Ouest Hospital, 69130 Écully, France 
j Department of Paediatric Cardiology and Cardiac Surgery, M3C network for Complex Congenital Heart Disease, Nantes University Hospital, 44000 Nantes, France 
k Department of Paediatric Cardiology, Eastern Paris Paediatric Specialties Centre, 94000 Créteil, France 
l Department of Adult Congenital Heart Disease, M3C network for Complex Congenital Heart Disease, Georges Pompidou European Hospital, AP-HP, 75015 Paris, France 
m Department of Paediatric and Adult Congenital Cardiology, UE3C Lowendal Centre for Cardiac Investigations and Congenital Heart Disease, 75015 Paris, France 
n Department of Paediatric and Adult Congenital Cardiology, Lisieux Hospital, 14100 Lisieux, France 
o Department of Paediatric and Adult Congenital Cardiology, Mutualiste Montsouris Institute, 75014 Paris, France 
p Department of Paediatric Cardiology, M3C network for complex congenital heart diseases, Gabriel Montpied University Hospital, 63000 Clermont Ferrand, France 
q Department of Paediatric Cardiology, M3C network for complex congenital heart diseases, Grenoble Alpes University Hospital, Grenoble Alpes University, 38700 La Tronche, France 
r Department of Paediatric Cardiology, M3C network for complex congenital heart diseases, Dijon University Hospital, 21000 Dijon, France 
s Department of Paediatric Cardiology, M3C network for complex congenital heart diseases, Amiens University Hospital, 80000 Amiens, France 
t Department of Paediatric Cardiology, M3C network for complex congenital heart diseases, Hautepierre University Hospital, 67200 Strasbourg, France 
u Department of Paediatric Cardiology, Paediatric and Fetal Cardiology Centre, 29490 Guipavas, France 
v Department of Paediatric Cardiology, M3C network for complex congenital heart diseases, Angers University Hospital, 49100 Angers, France 
w Department of Paediatric Cardiology, Rennes University Hospital, 35000 Rennes, France 
x Department of Paediatric Cardiology, Jacques Cartier Hospital, 91300 Massy, France 
y North Cardiology Centre, 93200 Saint-Denis, France 
z Department of Paediatric Cardiology, La Roseraie Hospital, 93300 Aubervilliers, France 
aa Department of Paediatric Cardiology, Côte-de-Nacre University Hospital, 14000 Caen, France 
ab Department of Cardiology, M3C network for complex congenital heart diseases, Nice University Hospital, UR2CA, Côte d’Azur University, 06000 Nice, France 
ac Department of Paediatric Cardiology, M3C network for complex congenital heart diseases, Louis Pradel University Hospital, 69500 Bron, France 
ad Department of Paediatric Cardiology, M3C network for complex congenital heart diseases, Tours University Hospital, 37000 Tours, France 
ae Department of Paediatric and Congenital Cardiology, M3C network for complex congenital heart diseases, La Timone University Hospital, AP–HM, 13005 Marseille, France 
af INSERM UMR_S 999 " Pulmonary hypertension: pathophysiology and novel therapies ", Marie Lannelongue hospital and Bicêtre Hospital, 92350, le Plessis-Robinson, France 

Corresponding author. Department of Paediatric and Adult Congenital Heart Diseases, Hôpital Marie Lannelongue, 133, avenue de la Résistance, 92350 Le Plessis-Robinson, France.Department of Paediatric and Adult Congenital Heart Diseases, Hôpital Marie Lannelongue, Groupe Hospitalier Paris Saint-Joseph133, avenue de la RésistanceLe Plessis-Robinson92350France
Sous presse. Épreuves corrigées par l'auteur. Disponible en ligne depuis le Thursday 08 May 2025

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.

Le texte complet de cet article est disponible en PDF.

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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