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BMJ Publishing Group, Heart, p. heartjnl-2018-314257, 2019

DOI: 10.1136/heartjnl-2018-314257

Elsevier, Heart, Lung and Circulation, (27), p. S398

DOI: 10.1016/j.hlc.2018.06.799

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Long-Term Mortality and Cardiovascular Burden for Adult Survivors of Coarctation of the Aorta

This paper was not found in any repository, but could be made available legally by the author.
This paper was not found in any repository, but could be made available legally by the author.

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Data provided by SHERPA/RoMEO

Abstract

ObjectiveTo examine the contemporary long-term outcome after coarctation repair.MethodsThis 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.ResultsAfter 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).ConclusionsLong-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.