Long-term mortality and cardiovascular burden for adult survivors of coarctation of the aorta. Issue 15 (28th March 2019)

• Record Type: Journal Article
• Authors: Lee, Melissa G Y; Babu-Narayan, Sonya V; Kempny, Aleksander; Uebing, Anselm; Montanaro, Claudia; Shore, Darryl F; d'Udekem, Yves; Gatzoulis, Michael A
• Is Part Of: Heart. Volume 105:Issue 15(2019)
• Page Start: 1190
• Page End: 1196
• Publication Date: 2019-03-28
• Subjects: aortic coarctation -- congenital heart disease surgery -- bicuspid aortic valve; Heart -- Diseases -- Treatment -- Periodicals; Cardiology -- Periodicals; 616.12
• DOI: 10.1136/heartjnl-2018-314257
• Languages: English
• ISSNs: 1355-6037

Abstract

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.