Delayed Success of Balloon Dilation for Coexisting Pulmonary Valve Stenosis and Sinotubular Narrowing

Abstract

We evaluated the outcomes of children at a single institution who underwent balloon pulmonary valvuloplasty (BPV) for coexisting pulmonary valve stenosis (PVS) and sinotubular narrowing (STN). BPV is the treatment of choice for PVS in children. Current practice favors surgical repair of moderate, severe, and symptomatic pulmonary stenosis when STN exists. This practice arose from lack of reduction in total pulmonary gradient (TPG) and frequent adverse events from BPV. A retrospective analysis of outcomes in children with coexisting PVS and STN following BPV at a single institution was performed. Twenty-three patients were identified. Median age at BPV was 0.5 years (interquartile range (IQR) 0.3-2). Surgery was avoided in 15/23 (65%) (Group 1) and required in 8/23 (35%) (Group 2) following BPV. Group 1 had a mean baseline peak echo TPG of 60 mm Hg (±12) that decreased to 44 mm Hg (±10) following BPV (P < 0.01) and further to 21 mm Hg (±13) at 3.6 years (±2.2) following BPV (P < 0.01). Group 2 had a mean baseline peak echo TPG of 68 mm Hg (±17). TPG was unchanged by first echo after BPV at 56 mm Hg (±13) and just prior to surgery at 63 mm Hg (±15) (P > 0.10). BPV has minimal acute effect on PVS when STN exists; however; long-term benefits are achieved in most. BPV should be considered first-line therapy given its safety and effectiveness. Only those with worsening clinical signs and symptoms should be referred for surgical repair following BPV.