Late outcomes in adults with coarctation of the aorta
P Choudhary1, C Canniffe2, D J Jackson3
1Department 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.
Insights
Adults with coarctation of the aorta (CoA) have improved long-term survival, reaching 89% by age 60. However, aortic complications and hypertension remain common challenges in this population.
Area of Science:
- Cardiology
- Adult Congenital Heart Disease (ACHD)
- Vascular Surgery
Background:
- Coarctation of the aorta (CoA) historically has poor long-term survival.
- Advances in pediatric cardiac surgery and adult congenital heart disease (ACHD) services have improved outcomes for other congenital heart defects.
- Contemporary outcomes for adult CoA patients require updated evaluation.
Purpose of the Study:
- To investigate long-term survival and morbidity in young adult patients with coarctation of the aorta (CoA) in the contemporary era.
- To assess the impact of surgical repair strategies on long-term outcomes.
Main Methods:
- A cohort of 151 adult patients with simple coarctation of the aorta (CoA) was followed at a tertiary ACHD service.
- Data collected included mortality, re-intervention for re-coarctation, and aneurysm development.
- Surgical repair methods and patient demographics were documented.
Main Results:
- Actuarial survival reached 98% at ages 40 and 50, and 89% by age 60.
- Re-coarctation occurred in 34% of patients, and descending aortic aneurysms in 18%.
- End-to-end repair was associated with lower rates of re-coarctation and aneurysms compared to other methods.
Conclusions:
- Adult patients with coarctation of the aorta (CoA) who survive to adolescence exhibit excellent long-term survival up to age 60.
- Significant long-term morbidity, particularly aortic complications and hypertension, remains prevalent.
- Optimized surgical techniques may reduce the incidence of late aortic complications.
Aims:
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.
Related Concept Videos
Aneurysm III: Interprofessional Care
Aortic Regurgitation II: Clinical Features and Diagnostic Tests
Aortic Regurgitation I: Introduction
Aneurysm II: Clinical Manifestations and Diagnostic Studies
Aortic Regurgitation III: Medical Management
Aortic Regurgitation IV: Nursing Management


