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

Tariq Abu-Tair ^1,2* Ines Willershausen ³ Melanie Friedmann ² Kai Rubarth ² Annika Weigelt ² Claudia Martin ¹ Sven Dittrich ³ Christoph Kampmann ¹

• ¹ Department of Congenital Heart Diseases, Centre for Diseases in Childhood and Adolescence, University Medical Centre, Johannes Gutenberg University Mainz, Mainz, Rhineland-Palatinate, Germany
• ² Department of Pediatric Cardiology, Friedrich-Alexander-University Erlangen-Nürnberg, University Hospital Erlangen, Erlangen, Bavaria, Germany
• ³ Department of Orthodontics and Orofacial Orthopedics, Friedrich Alexander-University Erlangen-Nürnberg, University Hospital Erlangen, Erlangen, Bavaria, Germany

Introduction: Percutaneous balloon valvuloplasty (PBV) 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 evaluated 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 admitted to the University Medicine Mainz and University Hospital Erlangen between November 1994 and March 2013 who underwent successful PBV as an initial procedure and 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: Overall, 62 neonates (median age at intervention 5 days) were included. Among them, 15 patients (24.2%) reached the endpoint. The mean freedom from surgery time differed according to the number of cusps (P < 0.001), pulmonary valve diameter Z-scores (P = 0.04), and grade of persisting stenosis (P = 0.008), while it 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.