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Primary Pulmonary Vein Stenosis: Outcomes, Risk Factors, and Severity Score in a Multicentric Study

Kalfa, D., Belli, E., Bacha, E., Lambert, V., di Carlo, D., Kostolny, M., Salminen, J., Nosal, M., Poncelet, A., Horer, J., Berggren, H., Yemets, I., Hazekamp, M., Maruszewski, B., Sarris, G., Pozzi, M., Ebels, T., Lacour-Gayet, F. & European Congenital Heart Surg Ass, Jul-2017, In : Annals of thoracic surgery. 104, 1, p. 182-189 8 p.

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  • Primary Pulmonary Vein Stenosis

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DOI

  • David Kalfa
  • Emre Belli
  • Emile Bacha
  • Virginie Lambert
  • Duccio di Carlo
  • Martin Kostolny
  • Jukka Salminen
  • Matej Nosal
  • Alain Poncelet
  • Jurgen Horer
  • Hakan Berggren
  • Illya Yemets
  • Mark Hazekamp
  • Bohdan Maruszewski
  • George Sarris
  • Marco Pozzi
  • Tjark Ebels
  • Francois Lacour-Gayet
  • European Congenital Heart Surg Ass

Background. Primary pulmonary vein stenosis (PPVS) still carries a poor prognosis, and prognostic factors remain controversial. The aim of this study was to determine outcomes and prognostic factors after PPVS repair in the current era.

Methods. Thirty patients with PPVS and a normal pulmonary vein (PV) connection operated on in 10 European/North American centers (2000-2012) were included retrospectively. A specific PVS severity score was developed based on the assessment of each PV. Studied end points were death, PV reoperation, and restenosis. A univariate and multivariate risk analysis was performed.

Results. The mean number of affected PVs per patient was 2.7 +/- 1.1. Sutureless repair was used in 21 patients (70%), endovenectomy was used in 5 patients, and patch venoplasty was used in 4 patients. Overall PV restenosis, reoperation, and mortality occurred in 50%, 40%, and 30% of patients respectively. Freedom from mortality, reoperation, and restenosis at 8 years of follow-up was 70% +/- 8%, 62% +/- 8%, and 47% +/- 9%, respectively. Restenosis and mortality rates after sutureless repair versus non-sutureless repair were 57% (n = 12 of 21) versus 33% (n[ 3 of 9) (p = 0.42) for restenosis and 38% (n = 8 of 21) versus 11% (n = 1 of 9) (p = 0.21) for mortality. Patients selected for a sutureless technique were younger and smaller and had more severe disease before operation. A postoperative high PVS score and pulmonary hypertension 1 month after the operation were independent risk factors for restenosis (hazard ratio [HR], 1.34; p = 0.002 and HR, 6.81; p = 0.02, respectively), reoperation (HR, 1.24; p = 0.01 and HR, 7.60; p = 0.02), and mortality (HR, 1.39; p = 0.01 and HR, 39.5; p = 0.008).

Conclusions. Primary PVS still has a guarded prognosis in the current era despite adoption of the sutureless technique. Postoperative pulmonary hypertension and severity of disease evaluated by a new severity score are independent prognostic factors regardless of surgical technique.

Original languageEnglish
Pages (from-to)182-189
Number of pages8
JournalAnnals of thoracic surgery
Volume104
Issue number1
Publication statusPublished - Jul-2017

    Keywords

  • SURGICAL REPAIR, SUTURELESS REPAIR, IN-SITU, SURVIVAL, PERICARDIUM, MANAGEMENT, CHILDREN

ID: 48246743