Surgical repair for primary pulmonary vein stenosis: Single-institution, midterm follow-up

Background: Primary pulmonary vein stenosis (PVS) is a rare congenital heart condition and carries a poor prognosis.

Methods: A retrospective review of 18 patients who underwent surgical therapy for primary PVS (2006-2014) was conducted. According to the degree of stenosis severity, the involved pulmonary veins (PVs) were divided into 3 categories: mild (34 veins), moderate (8), and severe (3). Pericardial patch venoplasty was used in 10 involved veins, endarterectomy in 11, and sutureless pericardial marsupialization in 19.

Results: Median surgical age and weight were 19.8 (range: 7-100) months and 7.7 (range: 5.3-20.3) kg, respectively. Bilateral PVS was found in 10 patients (56%), and unilateral in 8 (44%). Moderate or severe stenosis was found more frequently in PVs on the left side (P = .035). Multivein involvement was more common in patients age ≤18 months than in older patients (75% vs 20%, P = .054). No early operative death occurred. Median length of hospital stay was 16 (range: 8-60) days. One subsequent death occurred (6%), at the 2-month follow up after discharge. Median follow-up time for the remaining patients was 29 (range: 2-91) months. Three of the 6 PVs treated for moderate stenosis, compared with 7 of the 34 treated for mild stenosis, developed restenosis, irrespective of the surgical strategy (P = .153). Most surviving patients remained in relatively good condition, in New York Heart Association functional class I or II.

Conclusions: Detailed morphologic evaluation of each PV involved is a consideration for surgery, and is closely related to the prognosis. Moderate or severe primary PVS is worse than mild PVS, and no differences were found in effectiveness among endarterectomy, pericardial patch venoplasty, and sutureless pericardial marsupialization in treating primary PVS.