Serial Balloon Dilation of Pulmonary Veins Improves Outcomes

Background:
Recurrent pulmonary vein stenosis (PVS) in children is a progressive disease with poor outcomes. PVS can either be congenital or acquired following congenital heart surgery. Both surgical & catheter-based procedures (balloon dilation or stent placement) can provide good immediate relief of stenosis, but re-stenosis is common, progressive and can be fatal. We sought to describe the outcomes of serial balloon angioplasty for treatment of recurrent PVS at our center.

Methods:
A retrospective chart review of all children, <18 years, with the diagnosis of PVS admitted to Rady Children’s Hospital San Diego between 2008 - 2017 was performed. Demographic, clinical and outcome data were collected.

Results:
75 stenotic pulmonary veins in 24 patients underwent an average of 6 (2-22) catheterizations per patient with an average of 5(1-22) balloon dilations per vein. 16 patients had 1-6 catheterizations, 4 had 7-12, and 4 had > 12. Median intervals between procedures (in months) were 2.1 (1.2-4.4) for the first 6 catheterizations, 2.7 (1.3-5.8) for the subsequent 6 catheterizations, and 3.9 (3.3-11.8) for catheterizations greater than 12. 14 (58.3%) patients had PVS post-TAPVC repair, and 10 (41.7%) had primary PVS. 4 patients had 1 affected vein, 5 had 2 affected veins, 4 had 3 affected veins, and 11 had ≥ 4 affected veins [8 out of 11 were post TAPVC repair]. The 6-, 12-, 36-, and 60-month survival rates were 95.8%, 87.5%, 79.2%, and 70.8%, respectively.

Conclusions:
We present improved survival of patients with PVS using serial balloon angioplasty. Further prospective studies comparing the outcomes of different treatment strategies are needed.