title: Late outcomes in adults with coarctation of the aorta.
abstract: OBJECTIVE
  Previous cohort studies of patients with coarctation of the aorta (CoA) demonstrate reduced long-term survival. Improved surgical outcomes in children and evolution of adult congenital heart disease (ACHD) services have resulted in improved survival in patients with other CHDs. We hypothesise that for young adult patients with CoA long-term outcomes have improved in the contemporary era.
  
  
  METHODS
  151 patients (58% men) with simple CoA followed up at a tertiary ACHD service in Sydney, Australia, from 1993 to 2013 were included. We documented mortality and major morbidity such as the need for re-intervention for re-coarctation or aneurysms.
  
  
  RESULTS
  140 patients (mean age 35±15 years) underwent CoA repair at median age of 5 (IQR 0-10) years. Initial surgical strategy included end-to-end repair in 43, subclavian flap aortoplasty in 28 and patch aortoplasty in 31 patients (and was not documented in 28 cases). 6 patients had endovascular repair, 4 had interposition tube grafts and 11 were unrepaired. There were a total of seven deaths at a median age of 60 years. Actuarial survival was 98% at 40, 98% at 50 and 89% at 60 years of age. Re-coarctation occurred in 34% and descending aortic aneurysms were noted in 18%. Patients with end-to-end repair had lower rates of significant re-coarctation or descending aortic aneurysms (p=0.026 and <0.001, respectively). 66% had bicuspid aortic valve and 44% were hypertensive.
  
  
  CONCLUSIONS
  Patients with CoA who reach adolescence demonstrate very good long-term survival up to age 60 years. Long-term morbidity is common, however, related largely to aortic complications and late hypertension.
authors: Department of Cardiology, Royal Prince Alfred Hospital, Sydney, New South Wales, Australia Discipline of Medicine, Central Clinical School, University of Sydney, Sydney, New South Wales, Australia Department of Cardiology, Westmead Hospital, Sydney, New South Wales, Australia.
authors: P
authors: P
authors: Choudhary
authors: Department of Cardiology, Royal Prince Alfred Hospital, Sydney, New South Wales, Australia Department of Cardiology, Mater Misericordiae Hospital, Dublin, Ireland.
authors: C
authors: C
authors: Canniffe
authors: Department of Cardiology, Royal Prince Alfred Hospital, Sydney, New South Wales, Australia Discipline of Medicine, Central Clinical School, University of Sydney, Sydney, New South Wales, Australia.
authors: D J
authors: DJ
authors: Jackson
authors: Department of Cardiology, Royal Prince Alfred Hospital, Sydney, New South Wales, Australia Department of Cardiology, Westmead Hospital, Sydney, New South Wales, Australia.
authors: D
authors: D
authors: Tanous
authors: Department of Cardiology, Mater Misericordiae Hospital, Dublin, Ireland.
authors: K
authors: K
authors: Walsh
authors: Department of Cardiology, Royal Prince Alfred Hospital, Sydney, New South Wales, Australia Discipline of Medicine, Central Clinical School, University of Sydney, Sydney, New South Wales, Australia.
authors: D S
authors: DS
authors: Celermajer
mesh_terms: D000328:Adult; D000367:Age Factors; D001014:Aortic Aneurysm / Q000209:etiology / Q000601:surgery; D001017:Aortic Coarctation / Q000175:diagnosis / Q000401:mortality / Q000601:surgery*; D018572:Disease-Free Survival; D005260:Female; D006801:Humans; D006973:Hypertension / Q000209:etiology; D053208:Kaplan-Meier Estimate; D008297:Male; D008875:Middle Aged; D009517:New South Wales; D016016:Proportional Hazards Models; D012008:Recurrence; D012086:Reoperation; D012189:Retrospective Studies; D012307:Risk Factors; D013997:Time Factors; D016896:Treatment Outcome; D014656:Vascular Surgical Procedures / Q000009:adverse effects / Q000401:mortality; D055815:Young Adult