Long term outcome of repaired pulmonary valve stenosis: role of age at first intervention

Purpose: Despite Pulmonary Valve Stenosis (PVS) being considered a low risk congenital heart disease, a number of events have been described along follow up. The aim of this study is to evaluate long term outcome of repaired PVS and to identify population at risk of complications and need for re-intervention. Methods and results: From 1953 to 2010, 211 patients with PVS received surgical treatment (n=122; 57,8%) or percutaneous balloon valvuloplasty (n=89; 42,1%) in a single tertiary referral centre. The mean age at first intervention was 6.19±8.9 years (median 3 years). Patients with associated congenital heart defects other than atrial septal defect (ASD) or patent ductus arteriosus (PDA) were excluded. 29% (n=59) of the patients were symptomatic before their first intervention and the peak right ventricular pressure was 95±34 mmHg in the cohort with invasive assessment. After a mean follow-up of 22±10.21 years, 90.9% of patients were in NYHA functional class I. However, major complications (death, stroke, embolism, arrhythmia or congestive heart failure) occurred in 28 patients (13.3%) and need for re-intervention in 42 (19.9%). The distribution of major complications followed a bimodal pattern according to age at first intervention. Therefore we considered two groups: group A) 17 years (n=38; 18%), and group B) 1 month to 17 years (n=172; 82%). Patients of group A showed an association with ASD or PDA (44,7% vs. 43%; p=0.01), were more symptomatic (75% vs. 19.2%; p<0.0001), needed more drug therapy (22.2% vs. 2.4%; p=0.0001), and had higher presence of cardiomegaly (51.4% vs. 25.6%; p=0.005) before intervention. A significant difference was observed for major complications (26.3% vs. 10.4%; p=0,009) and need for re-intervention (42.1% vs. 15%; p<0,001) that were more frequent in group A. In the multivariate analysis the independent predictors for major complications were: group A (HR 4.54, CI 1.58-13.07; p=0.005) and the presence of ASD or PDA (HR 4.17, CI 1.31-13.34; p=0,016). Cardiomegaly (HR 5.1, CI 2.33-11.16; p<0.001), percutaneous valvuloplasty (HR 8.98, CI 3.49-23.01; p<0.001) and group A (HR 2.93, CI 1,34 - 6,4; p=0.007) were associated to the need for re-intervention. Conclusions: Although patients with PVS showed in general an excellent long term outcome, intervention at extreme ages identifies a population with a higher risk of major complications and need for re-intervention during follow-up. Patients in this age group and with other risk factors (symptoms and cardiomegaly before first intervention and associated ASD or PDA) should have a more strict control.