David Kalfa1, Emre Belli2, Emile Bacha3, Virginie Lambert2, Duccio di Carlo4, Martin Kostolny5, Jukka Salminen6, Matej Nosal7, Alain Poncelet8, Jurgen Horer9, Hakan Berggren10, Illya Yemets11, Mark Hazekamp12, Bohdan Maruszewski13, George Sarris14, Marco Pozzi15, Tjark Ebels16, François Lacour-Gayet17. 1. Department of Pediatric Cardiac Surgery, Morgan Stanley Children's Hospital of New York-Presbyterian, Columbia University Medical Center, New York, New York and Columbia University, New York, New York. Electronic address: dk2757@cumc.columbia.edu. 2. Department of Pediatric Cardiac Surgery, Marie Lannelongue Hospital, le Plessis Robinson, France. 3. Department of Pediatric Cardiac Surgery, Morgan Stanley Children's Hospital of New York-Presbyterian, Columbia University Medical Center, New York, New York and Columbia University, New York, New York. 4. Department of Pediatric Cardiac Surgery, Ospedale Pediatrico Bambino Gesù, Rome, Italy. 5. Department of Pediatric Cardiac Surgery, Great Ormond Street Hospital, London, United Kingdom. 6. Department of Pediatric Cardiac Surgery, Hospital for Children and Adolescents, University of Helsinki, Helsinki, Finland. 7. Department of Pediatric Cardiac Surgery, National Institute of Cardio-Vascular Diseases - Childrens Heart Center, Bratislava, Slovakia. 8. Department of Pediatric Cardiac Surgery, Saint-Luc Hospital, Brussels, Belgium. 9. Department of Pediatric Cardiac Surgery, German Heart Center, Clinic of Cardiovascular Surgery, Munich, Germany. 10. Department of Pediatric Cardiac Surgery, Children's Heart Center, The Queen Silvia Children's Hospital, Goteborg, Sweden. 11. Department of Pediatric Cardiac Surgery, Ukrainian Childrens Cardiac Center, Kyiv, Ukraine. 12. Department of Pediatric Cardiac Surgery, Leiden University Medical Center, Leiden, Netherlands. 13. Department of Pediatric Cardiac Surgery, Children's Memorial Health Institute, Warsaw, Poland. 14. Department of Pediatric Cardiac Surgery, Athens Heart Surgery Institute, Mitera Pediatric and Hygeia Hospital, Athens, Greece. 15. Department of Pediatric Cardiac Surgery, Ospedali Riuniti, Ancona, Italy. 16. Department of Pediatric Cardiac Surgery, University Medical Center Groningen, Groningen, Netherlands. 17. Department of Pediatric Cardiac Surgery, Royal Hospital Heart Center, Muscat, Oman.
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.
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.
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