PT  - JOURNAL ARTICLE
AU  - Melissa G Y Lee
AU  - Sonya V Babu-Narayan
AU  - Aleksander Kempny
AU  - Anselm Uebing
AU  - Claudia Montanaro
AU  - Darryl F Shore
AU  - Yves d’Udekem
AU  - Michael A Gatzoulis
TI  - Long-term mortality and cardiovascular burden for adult survivors of coarctation of the aorta
AID  - 10.1136/heartjnl-2018-314257
DP  - 2019 Aug 01
TA  - Heart
PG  - 1190--1196
VI  - 105
IP  - 15
4099  - http://heart.bmj.com/content/105/15/1190.short
4100  - http://heart.bmj.com/content/105/15/1190.full
SO  - Heart2019 Aug 01; 105
AB  - 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.