Management of perimembranous ventricular septal defect (pmVSD) with left ventricle (LV) volume overload (Zscore of end diastolic LV diameter (LVEDD)>2) without pulmonary arterial hypertension remains controversial.

Our objective was to describe features of patients in the FRANCISCO registry, a prospective nationwide cohort study.

Data were collected at inclusion. Echocardiographic data were cross-checked by a Core-lab.

A total of 212 patients with a median age of 4-year-old [1; 13] have been included in 38 centres. LV was very dilated (Zscore of LVEDD>3) in 38.6% of the study population with a relationship with maximum pmVSD size (P<0.01). Maximum pmVSD size was correlated with Zscore of LVEDD (P<0.01) and left atrial area (P<0.001). In patients aged 1 to 2 yo (n=87; 41%), dyspnoea (26%) was associated with a lower weight (P<0.001), left atrial area (P<0.01), medical therapy (P<0.01), a trend to a higher LVEDD Zscore (P=0.19) but not with pm VSD size.

In 25.5% of cases, pmVSD were closed by catheterization or surgery, with management varying among French regions (P<0.001). PmVSD closure was performed in 19 children aged 1 to 2 yo (22%), in 23 children aged 3 to 15 yo (27%) and in 12 patients over 15 yo (29%), P=0.6 (Fig. 1).

Factors associated with pmVSD closure were maximum pmVSD size in all patients (P<0.001); dyspnoea in children aged 1 to 2 yo (P=0.021); minor to mild aortic regurgitation (P<0.01), inlet or outlet pmVSD extension (P<0.01) and height of the membranous septal aneurysm (P<0.01) in children aged 3 to 15 yo; aortic cusp prolapse in patients over 15 yo (P=0.017).

In France, pmVSD with LV overload are not systematically closed in patients above one year-old. Closure is mainly indicated in case of symptoms or associated anatomical lesions. Long-term outcomes are awaited to refine the therapeutic algorithm.