Introduction: Reports suggest 25% mortality within 30 years from repair of coarctation of the aorta (CoA). We aimed to examine the long-term outcome after CoA repair in a contemporary cohort.
Methods: Retrospective study of 842 patients aged ≥18 years who underwent previous biventricular CoA repair with follow-up at one institution. Median age at repair was 3 years (IQR: 1 month-15 years) and was surgical in 82% (693/842) and percutaneous (balloon/stent) in 18% (149/842). Bicuspid aortic valve (BAV) was present in 58% (485/842).
Results: During a mean follow-up of 27 ± 14 years, there were 38 late deaths (5%, 38/842), and 29% of patients (246/842) required ≥1 arch reintervention. Aortic valve or ascending aortic intervention was required from birth in 13% (111/842) and 5% (43/842), respectively. Overall survival was 99.6% at 30 years, 94% at 50 years, and 75% at 70 years of age. Late survival after CoA repair was 97% at 30 years and 72% at 60 years. Multivariable risk factors for late mortality were older age at repair (HR 1.1, p<0.0001), associated cardiac procedure (HR 4.2, p=0.003), arch reoperation (HR 3.0, p=0.007), and ascending aortic intervention (HR 4.1, p=0.008). Freedom from arch reintervention was 71% at 30 years and 49% at 60 years. Multivariable risk factors for arch reintervention were LVOT obstruction (HR 1.9, p=0.009), age <1 year at repair (HR 2.5, p<0.0001), percutaneous repair (HR 5.0, p<0.0001), and patch repair (HR 2.5, p<0.0001). Freedom from aortic valve and ascending aortic intervention was 91% and 97% at 30 years, 83% and 92% at 50 years, and 53% and 81% at 70 years of age, respectively. BAV was a multivariable risk factor for aortic valve and ascending aortic intervention (HR>4.0, p<0.001). Arch reobstruction on echo and resting hypertension at last follow-up was present in 32% (170/534) and 55% (366/664), respectively.
Conclusions: Survival after CoA repair is excellent into old age. Over time, however, 50% of patients require arch reintervention, aortic valve surgery, and/or become hypertensive, and these risks accelerate after the fifth decade. More extensive surgical arch repair at first operation may prevent reintervention and associated risks. Risk stratification data may enable personalized follow-up strategies for patients with CoA.