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Early and late outcomes after surgical repair of congenital supravalvular aortic stenosis: a European Congenital Heart Surgeons Association multicentric study. Eur J Cardiothorac Surg 2017 Oct 01;52(4):789-797

Date

11/21/2017

Pubmed ID

29156017

DOI

10.1093/ejcts/ezx245

Scopus ID

2-s2.0-85030666260 (requires institutional sign-in at Scopus site)   18 Citations

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.

Author List

Padalino MA, Frigo AC, Comisso M, Kostolny M, Omeje I, Schreiber C, Pabst von Ohain J, Cleuziou J, Barron DJ, Meyns B, Hraska V, Maruszewski B, Kozlowski M, Vricella LA, Hibino N, Collica S, Berggren H, Synnergren M, Lazarov S, Kalfa D, Bacha E, Pizarro C, Hazekamp M, Sojak V, Jacobs JP, Nosal M, Fragata J, Cicek S, Sarris GE, Zografos P, Vida VL, Stellin G

Author

Viktor Hraska MD Professor in the Surgery department at Medical College of Wisconsin




MESH terms used to index this publication - Major topics in bold

Adolescent
Aortic Stenosis, Supravalvular
Cardiac Surgical Procedures
Cardiology
Child
Child, Preschool
Europe
Female
Follow-Up Studies
Forecasting
Humans
Infant
Infant, Newborn
Male
Postoperative Complications
Prevalence
Retrospective Studies
Societies, Medical
Survival Rate