Management outcomes of primary pulmonary vein stenosis

Objectives: Primary pulmonary vein stenosis (PPVS) is increasingly diagnosed in children with no prior pulmonary vein intervention history, and management is challenging. We describe characteristics of patients who underwent surgical repair of PPVS at our center, and examine factors associated with treatment failures.

Methods: A retrospective review of all patients who underwent surgical intervention for PPVS (2002-2016) was completed. Patients who had undergone prior cardiac surgery involving the pulmonary veins or atrial switch were excluded. Regression analyses were performed to examine characteristics, PPVS features, including severity score, and surgical details associated with treatment failures.

Results: Thirty-four children underwent initial surgical intervention for PPVS. Median age was 8.9 months (interquartile range, 5.9-18.4 months). Most patients (n = 31; 91%) had unilateral pulmonary vein involvement and the median PPVS severity score was 3.5 (interquartile range, 3-5). On competing risk analysis, 1 year following surgical repair, 9% of patients had died, 14% had undergone reintervention, and 77% were alive without reintervention; at 5 years the numbers were 9%, 30%, and 61%, respectively. Factors associated with mortality included bilateral disease and PPVS severity score >6. Bilateral disease and PPVS severity score >5 were associated with reintervention risk.

Conclusions: Multidisciplinary management strategy is required for PPVS. Despite satisfactory early repair, patients continue to be at risk for recurrence and subsequent mortality, especially those with extensive primary involvement. The disappointing results underscore the need for multi-institutional collaborations to better understand this complex disease, establish management and follow-up protocols, and explore investigational treatment modalities that could modify the unfavorable outcome of this uncommon and challenging disease.