Pulmonary vein stenosis: Anatomic considerations, surgical management, and outcomes.

The study objective was to evaluate outcomes of pulmonary vein stenosis repair in a large single-center cohort. Clinical data from a pulmonary vein stenosis registry were retrospectively reviewed identifying patients who underwent pulmonary vein stenosis repair. The primary/index operation was defined as the patient's first pulmonary vein stenosis operation during the study period. Between January 2007 and August 2019, 174 patients underwent pulmonary vein stenosis repair. Bilateral pulmonary vein stenosis occurred in 111 patients (64%); 71 patients (41%) had 4-vessel disease. Fifty-nine patients (34%) had primary pulmonary vein stenosis. Median age was 9 months (interquartile range, 5-27) and weight was 6.5 kg (4.7-10.2). Surgical techniques evolved and included ostial resection, unroofing, reimplantation, sutureless, modified sutureless, and a newer anatomically focused approach of pulmonary vein stenosis resection with lateralization or patch enlargement of the pulmonary vein–left atrium connection. Twenty-three patients (13%) required reoperation. Cumulative 2-year incidence of postoperative transcatheter intervention (balloon dilation ± stenting) was 64%. One-, 2-, and 5-year survivals were 71.2%, 66.8%, and 60.6%, respectively. There was no association between surgery type and reoperation rate (hazard ratio, 2.38, P =. 25) or transcatheter intervention (hazard ratio, 0.97, P =. 95). The anatomically focused repair was associated with decreased mortality on univariate (hazard ratio, 0.38, P =. 042) and multivariable analyses (hazard ratio, 0.19, P =. 014). Antiproliferative chemotherapy was also associated with decreased mortality (hazard ratio, 0.47, P =. 026). This large single-center surgical pulmonary vein stenosis experience demonstrates encouraging midterm results. A new anatomically focused repair strategy aims to alleviate pulmonary vein angulation to minimize turbulence and shows promising early outcomes. Continued follow-up is required to understand longer-term outcomes for this surgical approach. The PVs can take a long, angulated course as they pass from the lung parenchyma to the LA. This angulated course appears to occur due to the fulcrum effect of structures adjacent to the PVs (ie, descending aorta, airway, pulmonary artery, and pericardial reflection). The anatomically focused repair strategy aims to target this long, angulated PV course, which may be the nidus for turbulent blood flow and subsequent PVS. This repair involves extensive takedown of the pericardial reflection, resection of stenosing/scar tissue, and lateralizing/patch augmenting the PV–LA connection with the goal of creating a shorter and straighter pulmonary venous course. Early follow-up suggests improved survival with the anatomically focused repair strategy compared with conventional repair techniques (ie, sutureless and modified sutureless repair). [Display omitted] [ABSTRACT FROM AUTHOR]