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

Eric N. Feins, Christina Ireland, Kimberlee Gauvreau, Mariana Ch_avez, Ryan Callahan, Kathy J. Jenkins, and Christopher W. Baird


Take Home Points:

  • Anatomic focused repair strategy provides better survival after pulmonary vein stenosis repair.
  • Characterization of PVS by multi-modalities as well as multidisciplinary team approach are important in the treatment of PVS.

Dr. Yasuhiro Kotani

Commentary from Dr. Yasuhiro Kotani (Okayama, Japan), chief section editor of Congenital Heart Surgery Journal Watch:



Single center retrospective study included 174 patients who underwent pulmonary vein stenosis (PVS) repair between 2007 and 2019. 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 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 or transcatheter intervention but the anatomically focused repair was associated with decreased mortality on univariate (hazard ratio, 0.38, P=0.042) and multivariable analyses (hazard ratio, 0.19, P=0.014). Antiproliferative chemotherapy was also associated with decreased mortality (hazard ratio, 0.47, P=0.026).



This relatively large retrospective study for PVS repair was done at Boston Children’s Hospital. 5-year survival was not satisfactory but acceptable about 60%, indicating the difficulty of the treatment of PVS even in the world renown center in the recent era. Cumulative 2-year incidence of postoperative catheter intervention was 64%.


Boston group has a multidisciplinary team that dedicates diagnosis and treatment of PVS which is outstanding approach as etiology of PVS is multifactorial that needs a tailor made management. They introduced a new technique, i.e., anatomically focused repair to shorten and straighten the course PV course from the lung parenchyma to the left atrium. They showed significantly better survival using this technique compared to conventional repair technique that included sutureless technique. Although etiology of PVS is multifactorial and indication of this new technique is somewhat difficult to understand, they emphasized the importance of characterization of PVS by computed tomography or magnetic resonance imaging. As they used this new technique more recently, it is not clear that improved outcome achieved in the recent era is either by new technique or the result of team approach. Also, because it is not shown how many patients who had this new technique received an adjuvant chemotherapy. Together with the fact of relatively short-term follow up in the current study, long-term follow up will answer the impact of this new technique on the treatment of this unique entity.