David Kalfa, Emre Belli, Emile Bacha, 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 for the European Congenital Heart Surgeons Association
Morgan Stanley Children’s Hospital of New York-Presbyterian and Columbia University Medical Center. Marie Lannelongue Hospital. Ospedale Pediatrico Bambino Gesù. Great Ormond Street Hospital. Hospital for Children and Adolescents, University of Helsinki. Childrens Heart Center. Saint-Luc Hospital. The Queen Silvia Children’s Hospital. Ukrainian Childrens Cardiac Center. Leiden University Medical Center. Children’s Memorial Health Institute. Mitera Pediatric and Hygeia Hospital. Ospedali Riuniti. University Medical Center Groningen. Royal Hospital Heart Center.
United States, France, Italy, United Kingdom, Finland, Slovakia, Belgium, Germany, Sweden, Ukraine, Netherlands, Poland, Greece. Oman
Annals of Thoracic Surgery
Ann Thorac Surg 2017; 104: 182-189
DOI: 10.1016/j.athoracsur.2017.03.022
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.
Category
Stenosis or Obstruction of Normal Pulmonary Venous Connections
Pulmonary Hypertension Associated with Stenosis or Atresia of Pulmonary Veins
Patient Factors Influencing the Onset, Severity or Outcome of Disease
Length of Life Associated with Pulmonary Venous Obstruction
Diagnostic Testing. Risk Stratification
Surgical Interventions for Pulmonary Venous Obstruction After the Onset of Disease
Year of Publication: 2017
Age Focus: Pediatric
Article Type: Retrospective Observational Cohort Studies (>10 patients)
Article Access: Free PDF File or Full Text Article Available Through PubMed or DOI: No