Massimo A Padalino1, Anna Chiara Frigo2, Marina Comisso1, Martin Kostolny3, Ikenna Omeje3, Christian Schreiber4, Jelena Pabst von Ohain4, Julie Cleuziou4, David J Barron5, Bart Meyns6, Viktor Hraska7, Bohdan Maruszewski8, Michal Kozlowski8, Luca A Vricella9, Narutoshi Hibino9, Sarah Collica9, Hakan Berggren10, Mats Synnergren10, Stojan Lazarov11, David Kalfa12, Emile Bacha12, Christian Pizarro13, Mark Hazekamp14, Vlado Sojak14, Jeffrey P Jacobs15, Matej Nosal16, Jose Fragata17, Sertac Cicek18, George E Sarris19, Panayotis Zografos19, Vladimiro L Vida1, Giovanni Stellin1. 1. Section of Pediatric and Congenital Cardiac Surgery, Department of Cardiac, Thoracic and Vascular Sciences, University of Padova, School of Medicine, Padova, Italy. 2. Section of Biostatistics, Epidemiology and Public Health, Department of Cardiac, Thoracic and Vascular Sciences, University of Padova, School of Medicine, Padova, Italy. 3. Department of Pediatric Cardiac Surgery, Great Ormond Street Hospital for Sick Children, London, UK. 4. Department of Pediatric Cardiac Surgery, Deutsches Herzzentrum, Munich, Germany. 5. Department of Pediatric Cardiac Surgery, Birmingham Children's Hospital, Birmingham, UK. 6. Department of Pediatric Cardiac Surgery, UZ Gasthuisberg, Leuven, Belgium. 7. Department of Pediatric Cardiac Surgery, German Pediatric Heart Center, Sankt Augustin, Germany. 8. Department of Cardiothoracic Surgery, The Children's Memorial Health Institute, Warsaw, Poland. 9. Department of Cardiac Surgery, Johns Hopkins Medical Center, Baltimore, MD, USA. 10. Children's Heart Centre, The Queen Silvia Children's Hospital, Gothenburg, Sweden. 11. Department of Pediatric Cardiac Surgery, National Heart Hospital, Sofia, Bulgaria. 12. Division of Pediatric and Congenital Cardiac Surgery, New York-Presbyterian Morgan Stanley Children's Hospital, Columbia University Medical Center, Columbia University, New York, NY, USA. 13. Department of Pediatric Cardiac Surgery, Nemours Cardiac Center, Alfred I DuPont Hospital for Children, Wilmington, DE, USA. 14. University Medical Center, Leiden, Netherlands. 15. Department of Pediatric Cardiac Surgery, All Children's Hospital, St. Petersburg, FL, USA. 16. Department of Pediatric Cardiac Surgery, Children's Heart Centre, Bratislava, Slovakia. 17. Cardiothoracic Surgery Hospital de Santa Marta Rua de Santa Marta, Lisbon, Portugal. 18. Department of Pediatric Cardiac Surgery, Anadolu Medical Center, Heart and Vascular Care, Istanbul, Turkey. 19. Athens Heart Surgery Institute, Iaso Children's Hospital, Athens, Greece.
Abstract
OBJECTIVES: Our goal was to evaluate the early and late results of the surgical management of congenital supravalvular aortic stenosis (SVAS). METHODS: We performed a retrospective, multicentre study using data from the European Congenital Heart Surgeons Association. Exclusion criteria were age >18 years, operation before 1990 and redo supravalvular aortic stenosis operations. Multivariate Cox regression analysis was performed to detect independent predictors of adverse events. RESULTS: Of a total of 301 patients (male/female = 194/107; median age 3.9 years, range 13 days-17.9 years), 17.6% had a prior surgical or interventional procedure. Pulmonary artery stenosis was present in 41.5% and coronary anomalies in 13.6%. The operation consisted of a single patch repair in 36.7%, a pantaloon-shaped patch in 36.7%, a 3-patch technique in 14.3% and other techniques in 11.7%. Postoperative complications occurred in 14.9%, and the early mortality rate was 5%. At a median follow-up of 13 years (interquartile range 3.5-7.8; follow-up completed 79.1%), there were 10 late deaths (4.2%). A surgical reoperation or an interventional cardiology procedure occurred in 12.6% and 7.2%, respectively. No significant differences in outcomes between the techniques were found. Age at repair <12 months and pulmonary artery stenosis were associated with an increased risk of early (P = 0.0001) and overall mortality (P = 0.025), respectively. Having an operation after 2005 and co-existing pulmonary artery stenosis were significant predictors of late reintervention (P = 0.0110 and P = 0.001, respectively). CONCLUSIONS: Surgical repair of congenital stenosis is an effective procedure with acceptable surgical risk and good late survival, but late morbidity is not negligible, especially in infants and when associated pulmonary artery stenosis is present.
OBJECTIVES: Our goal was to evaluate the early and late results of the surgical management of congenital supravalvular aortic stenosis (SVAS). METHODS: We performed a retrospective, multicentre study using data from the European Congenital Heart Surgeons Association. Exclusion criteria were age >18 years, operation before 1990 and redo supravalvular aortic stenosis operations. Multivariate Cox regression analysis was performed to detect independent predictors of adverse events. RESULTS: Of a total of 301 patients (male/female = 194/107; median age 3.9 years, range 13 days-17.9 years), 17.6% had a prior surgical or interventional procedure. Pulmonary artery stenosis was present in 41.5% and coronary anomalies in 13.6%. The operation consisted of a single patch repair in 36.7%, a pantaloon-shaped patch in 36.7%, a 3-patch technique in 14.3% and other techniques in 11.7%. Postoperative complications occurred in 14.9%, and the early mortality rate was 5%. At a median follow-up of 13 years (interquartile range 3.5-7.8; follow-up completed 79.1%), there were 10 late deaths (4.2%). A surgical reoperation or an interventional cardiology procedure occurred in 12.6% and 7.2%, respectively. No significant differences in outcomes between the techniques were found. Age at repair <12 months and pulmonary artery stenosis were associated with an increased risk of early (P = 0.0001) and overall mortality (P = 0.025), respectively. Having an operation after 2005 and co-existing pulmonary artery stenosis were significant predictors of late reintervention (P = 0.0110 and P = 0.001, respectively). CONCLUSIONS: Surgical repair of congenital stenosis is an effective procedure with acceptable surgical risk and good late survival, but late morbidity is not negligible, especially in infants and when associated pulmonary artery stenosis is present.