Pulmonary vein rehabilitation

Pulmonary vein stenosis (PVS) in children is a progressive disease with poor outcomes. Surgical and catheter-based interventions can provide immediate relief of stenosis, but re-stenosis is common, often progressive, and can be fatal. We pursued serial balloon angioplasty (BA) to treat patients with recurrent PVS at our center. We performed a retrospective chart review and data analysis of all children (age < 18 years) with PVS treated with BA at a single center over ten years (2008–2017). Balloon angioplasties (n = 278) were performed on 69 stenotic pulmonary veins in 23 patients during 150 catheterization procedures. The median number of BA was 3 per pulmonary vein (IQR 2–7, range 1–20) and the median number of catheterization procedures was 5 per patient (IQR 2–9, range 2–23). The median age of the first BA was 4.7 months (IQR 2.9–13.8, range 2–65.3). The median follow-up duration was 2.6 years (IQR 1.4–7.1, range 0.42–9.6). 17 veins were stented with 18 stents (15 bare-metal and 3 drug-eluting). There were no procedural deaths or adverse outcomes requiring surgical intervention. 26 veins underwent subsequent planned surgical revision. The 1-, 3-, and 5-year survival rates (from first BA) were 78%, 70%, and 70%, respectively. In conclusion, for children with PVS, serial balloon angioplasty as a bridge to resolution, stenting, or surgical repair resulted in favorable outcomes. Further prospective studies comparing the outcomes of different treatment strategies are needed.