title: Late outcomes in children with Shone's complex: a single-centre, 20-year experience.
abstract: OBJECTIVE
  Shone's syndrome is a complex consisting of mitral valve stenosis in addition to left ventricle outflow obstruction. There are a few studies evaluating the long-term outcomes in this population. We sought to determine the long-term outcomes in our paediatric population with Shone's syndrome and the factors associated with left heart growth.
  
  
  METHODS
  All patients diagnosed with Shone's syndrome with biventricular circulation treated between 1978 and 2010 were reviewed. Baseline echocardiograms and data from catheterisations were also reviewed. Number of interventions (surgical+transcatheter), incidence of mitral valve replacement, and incidence of heart transplantation were tracked. Survival of the population and left heart structural growth were also reviewed.
  
  
  RESULTS
  A total of 121 patients with Shone's syndrome presented at a median age of 28 days (0-17.3 years) and were followed-up for 7.2 years (0.01-35.5 years). These patients underwent 258 interventions during the study period, and the presence of coarctation was associated with repeat left heart interventions. The 10-year, transplant-free survival was 86%. Presence of pulmonary hypertension was associated with mortality. Left heart structural growth was seen for mitral and aortic valve annuli and left ventricular end-diastolic dimension over time.
  
  
  CONCLUSIONS
  Shone's syndrome patients undergo a number of left heart interventions. Coarctation of the aorta is associated with an increased likelihood for repeat interventions. Survival appears to be more favourable than expected. Significant left heart growth will occur in the population. Pulmonary hypertension is associated with an increased risk of mortality.
authors.affiliation: 1Department of Pediatrics,Division of Pediatric Cardiology,Children's Healthcare of Atlanta,Emory University School of Medicine,Atlanta,Georgia,United States of America.
authors.forename: George T
authors.initials: GT
authors.lastname: Nicholson
authors.affiliation: 2Department of Pediatrics,Division of Biostatistics,Children's Healthcare of Atlanta,Atlanta,Georgia,United States of America.
authors.forename: Michael S
authors.initials: MS
authors.lastname: Kelleman
authors.affiliation: 3Department of Pediatrics,Division of Pediatric Cardiology,Texas Children's Hospital,Baylor College of Medicine,Houston,Texas,United States of America.
authors.forename: Caridad M
authors.initials: CM
authors.lastname: De la Uz
authors.affiliation: 3Department of Pediatrics,Division of Pediatric Cardiology,Texas Children's Hospital,Baylor College of Medicine,Houston,Texas,United States of America.
authors.forename: Ricardo H
authors.initials: RH
authors.lastname: Pignatelli
authors.affiliation: 3Department of Pediatrics,Division of Pediatric Cardiology,Texas Children's Hospital,Baylor College of Medicine,Houston,Texas,United States of America.
authors.forename: Nancy A
authors.initials: NA
authors.lastname: Ayres
authors.affiliation: 1Department of Pediatrics,Division of Pediatric Cardiology,Children's Healthcare of Atlanta,Emory University School of Medicine,Atlanta,Georgia,United States of America.
authors.forename: Christopher J
authors.initials: CJ
authors.lastname: Petit
mesh_terms: D000293:Adolescent; D001017:Aortic Coarctation / Q000000981:diagnostic imaging; D001021:Aortic Valve / Q000002:abnormalities* / Q000000981:diagnostic imaging; D001024:Aortic Valve Stenosis / Q000151:congenital / Q000000981:diagnostic imaging / Q000453:epidemiology*; D002648:Child; D002675:Child, Preschool; D004452:Echocardiography; D005260:Female; D005500:Follow-Up Studies; D005845:Georgia / Q000453:epidemiology; D006330:Heart Defects, Congenital / Q000000981:diagnostic imaging / Q000601:surgery; D016027:Heart Transplantation; D006801:Humans; D006976:Hypertension, Pulmonary / Q000150:complications* / Q000453:epidemiology; D007223:Infant; D007231:Infant, Newborn; D016014:Linear Models; D008297:Male; D008943:Mitral Valve / Q000002:abnormalities* / Q000000981:diagnostic imaging; D008946:Mitral Valve Stenosis / Q000151:congenital / Q000000981:diagnostic imaging / Q000453:epidemiology*; D012086:Reoperation; D016019:Survival Analysis; D013577:Syndrome
keywords: CHD; Shone’s complex; complex CHD