Predictors for long-term outcome of pulmonary valve perforation and balloon valvuloplasty in neonates with critical pulmonary valve stenosis or pulmonary valve atresia with intact ventricular septum

Introduction Percutaneous balloon valvuloplasty is the treatment of choice for critical pulmonary valve stenosis (CPS) and pulmonary valve atresia with intact ventricular septum (PA/IVS) if the ventricle has a suitable size. This study aimed to evaluate the long-term outcomes and predictors for surgical intervention and pulmonary valve replacement in patients with CPS or with PA/IVS after PBV, considering different morphological and hemodynamic parameters. Methods Neonates with PA/IVS or CPS who were admitted to the University Medicine Mainz and University Hospital Erlangen between November 1994 and March 2013 and underwent successful PBV as an initial procedure, with a follow-up of at least 5 years (median 13.1 years), were included. The Z-scores of pulmonary valve diameter, balloon/annulus ratio, number of cusps, and persisting stenosis were analyzed. The endpoint was the need for surgical procedures or valve replacement. Results A total of 62 neonates (median age at intervention 5 days) were included. Among them, 15 patients (24.2%) reached the endpoint. The mean time of freedom from surgery differed according to the number of cusps (P < 0.001), pulmonary valve diameter Z-scores (P = 0.04), and degree of persisting stenosis (P = 0.008), but did not differ according to the balloon/annulus ratio (<= 1.2 vs. >1.2). Conclusion Pulmonary valve perforation and PBV achieved favorable long-term outcomes in neonates with PA/IVS and CPS. A small pulmonary valve diameter, reduced number of cusps, and persisting gradient of >40 mmHg increased the risk for reduced time of freedom from surgical intervention and/or pulmonary valve replacement.