Surgical repair of pulmonary venous stenosis: a word of caution.

Background: Pulmonary vein stenosis (PVS), both congenital and acquired, is challenging to treat surgically with uncertain long-term results. We reviewed an 11-year surgical experience in 52 children.

Methods: From 2002 to 2012, 52 children age 0 days to 13 years (mean 1.9 years, median 11.7 months) weighing 2.2 to 32.5 kg (mean 9.3 kg, median 7.6 kg) had surgical relief of PVS. Based on clinical characteristics or complexity, 33 (63%) had a sutureless pericardial well repair and 19 (37%) had a more standard patch repair. There were no significant differences in clinical characteristics between the 2 techniques. Twenty children (38%) had prior anomalous pulmonary vein repair and 8 had primary pulmonary vein stenosis; 26 (50%) had other operations at the time of PVS relief.

Results: There were 2 hospital deaths (10.5%) in the "standard" group and 5 (15.2%) in the sutureless group (p>0.99). Despite postoperative evidence of PVS relief by echocardiogram or cardiac cath in all patients, at 5 years, actuarial freedom from PVS recurrence or death in the hospital survivors was 67% in the standard group and 58% in the sutureless group. Most recurrences or deaths occurred within 6 months of operation. Heterotaxy, single ventricle anatomy, bilateral disease, and previous anomalous pulmonary vein repair were not predictors of failure.

Conclusions: Surgical treatment of pulmonary vein stenosis remains a challenging problem with nontrivial early mortality and ongoing risk for recurrence or death regardless of surgical technique employed. Clearly, development of methods for earlier intervention or detection and improved surgical techniques are warranted.