Skip to main navigation Skip to search Skip to main content

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

  • European Congenital Heart Surgeons Association
  • Columbia University Irving Medical Center
  • Centre Chirurgical Marie Lannelongue
  • Ospedale Pediatrico Bambino Gesù
  • Great Ormond Street Hospital for Children NHS Foundation Trust
  • University of Helsinki
  • National Institute of Cardio-Vascular Diseases
  • Clinique Universitaire St-Luc
  • Queen Silvia Children's Hospital
  • Ukrainian Childrens Cardiac Center
  • Leiden University Medical Centre
  • Memorial Children's Health Institute
  • Mitera Pediatric and Hygeia Hospital
  • University Hospital “Ospedali Riuniti di Ancona”
  • University Medical Center Groningen
  • Royal Hospital

Research output: Contribution to journalArticlepeer-review

79 Scopus citations

Abstract

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 nonsutureless 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
DOIs
StatePublished - Jul 2017

Fingerprint

Dive into the research topics of 'Primary Pulmonary Vein Stenosis: Outcomes, Risk Factors, and Severity Score in a Multicentric Study'. Together they form a unique fingerprint.

Cite this