Success rate of primary percutaneous balloon angioplasty in children with Pulmonary Stenosis and Noonan syndrome

Abstract

Background:
Noonan syndrome (NS) is associated with different types of congenital heart defects (CHD), the most common of which is supravalvular pulmonary stenosis ((SV)PS). Possible treatment options are percutaneous balloon pulmonary valvuloplasty (BVP) or surgical intervention. The anatomical location of the PS may help predict BVP failure. We aimed to identify factors predicting treatment outcome and reintervention rate of BVP in these patients. 

Methods:
Medical records of children with a diagnosis of NS and in follow-up at Antwerp- and Ghent University Hospitals from 2000 to 2022 were retrospectively reviewed.

Results:
Thirty-two children were identified with a SVPS, either isolated or in combination with other CHD. Sixty-nine percent of the children with PS had SVPS. Isolated SVPS was identified as a risk factor for intervention.

Surgical or percutaneous intervention was necessary in 17/32 patients with PS (53%). All but 2 children with pulmonary valve stenosis had SVPS. Fifteen (13 with SVPS) underwent percutaneous balloon dilatation, of which 10 (67%) needed a second intervention, but all of them ultimately needed surgical repair due to persistent stenosis. The global success rate of percutaneous intervention in children with Noonan and SVPS was (31,1%).

Conclusion:
SVPS is a frequently encountered CHD in children with NS. The prevalence of SVPS was similar for all NS associated genes. Isolated SVPS is a risk factor for intervention. The success rate of BVP in patients with NS and SVPS is low. BVP might still be useful in selected cases.