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Congenital heart disease
Original research article
Long-term mortality and cardiovascular burden for adult survivors of coarctation of the aorta
  1. Melissa G Y Lee1,2,3,
  2. Sonya V Babu-Narayan4,5,
  3. Aleksander Kempny4,5,
  4. Anselm Uebing4,5,
  5. Claudia Montanaro4,5,
  6. Darryl F Shore5,6,
  7. Yves d’Udekem1,2,3,
  8. Michael A Gatzoulis4,5
  1. 1 Department of Cardiac Surgery, The Royal Children’s Hospital, Melbourne, Victoria, Australia
  2. 2 Department of Paediatrics, University of Melbourne, Melbourne, Victoria, Australia
  3. 3 Heart Research, Murdoch Children’s Research Institute, Melbourne, Victoria, Australia
  4. 4 Adult Congenital Heart Centre and National Centre for Pulmonary Hypertension, Royal Brompton Hospital, London, UK
  5. 5 National Heart and Lung Institute, Imperial College London, UK
  6. 6 Department of Cardiothoracic Surgery and Transplantation, Royal Brompton Hospital, London, UK
  1. Correspondence to Dr Sonya V Babu-Narayan, National Heart and Lung Institute, Imperial College London, London, UK. Adult Congenital Heart Centre, Royal Brompton Hospital, Sydney St, Chelsea, London SW3 6NP, UK.; s.babu-narayan{at}rbht.nhs.uk

Abstract

Objective To examine the contemporary long-term outcome after coarctation repair.

Methods This is a retrospective cohort study of 834 patients aged ≥16 years who underwent coarctation repair under single-centre follow-up. Repair was performed at a median age of 3 years (lower-upper quartile: 1 month to 15 years) by surgery in 83% (690/834) and angioplasty/stenting in 17% (144/834). Survival was compared with an age- and gender matched normal population. Other outcomes included arch reintervention, aortic valve intervention, ascending aortic intervention, and residual/re-coarctation and resting hypertension at latest follow-up.

Results After a median follow-up of 27 years (lower-upper quartile: 18–36), there were 38 late deaths (5%, 38/834). Overall survival was 99%, 88% and 65% at 30, 50 and 70 years of age, respectively, significantly reduced compared with a matched normal population (standardised mortality ratio: 3.20, log-rank: p<0.001). Thirty per cent (246/834) required ≥1 arch reintervention, 13% (111/834) an aortic valve intervention and 5% (43/834) an ascending aortic intervention. Freedom from aortic valve and ascending aortic intervention was 83% and 92% at 50 years and 53% and 81% at 70 years of age, respectively. Residual/re-coarctation (gradient ≥25 mm Hg or repair site/diaphragm ratio ≤70%) at latest follow-up was present in 60% (282/474) and resting hypertension in 57% (379/661).

Conclusions Long-term survival in contemporary adult survivors of coarctation repair is significantly lower than a matched normal population with accelerated decline after the third decade. Nearly 60% of patients eventually develop hypertension, whereas approximately 50% require further invasive cardiovascular treatment by 50 years of age. Our risk-stratifying data may enable personalised follow-up strategies for this common congenital heart condition.

  • aortic coarctation
  • congenital heart disease surgery
  • bicuspid aortic valve

https://doi.org/10.1136/heartjnl-2018-314257

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    Footnotes

    • Yd and MAG contributed equally.

    • Contributors MGYL contributed to the planning, data collection, data analysis, manuscript writing and manuscript revision. SVB-N contributed to the planning, data analysis, manuscript writing, manuscript revision and supervision. AK contributed to the data analysis and manuscript writing. AU and DS contributed to the manuscript writing. CM contributed to the data collection and manuscript writing. Yd’U and MAG contributed equally to as senior authorship to this paper in planning, data analysis, manuscript writing, manuscript revision, provision of resources, and supervision.

    • Funding MGYL is supported by a National Health and Medical Research Council (NHMRC) Medical Research Postgraduate Scholarship (1134274), a National Heart Foundation Health Professional Scholarship supported by The Noel and Imelda Foster Research Award (100681) and Collaboration and Exchange Award (101591), and an Australian Government Research Training Program Scholarship. SVB-N is supported by the British Heart Foundation (FS/11/38/28864). Yves d’Udekem is an NHMRC Clinician Practitioner Fellow (1082186).

    • Competing interests Yd’Ud is a consultant for Actelion and MSD.

    • Provenance and peer review Not commissioned; externally peer reviewed.

    • Patient consent for publication Not required.