As survival rates improve, adults with congenital heart disease (ACHD) increasingly present with aortopathies ranging from aortic root dilatation and aneurysm formation to life-threatening dissection and rupture. These vascular complications are associated with various congenital cardiac lesions, including bicuspid aortic valve, coarctation of the aorta, Tetralogy of Fallot, and transposition of the great arteries. Understanding the incidence, progression, and outcomes of aortopathy in this population is essential for guiding surveillance and surgical strategies.
Objective
To summarize current evidence on the clinical characteristics, progression, surgical decision-making and outcomes of aortopathies in ACHD.
Methods
A systematic review was conducted in accordance with the Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines. Databases searched included PubMed, SCOPUS, Cochrane Library, and Medline, with articles screened for relevance using predefined criteria.
Results
Of 3382 records identified, 46 studies met the inclusion criteria following a full-text review, and these studies combined included 24 241 patients. Bicuspid aortic valve and repaired coarctation were the most studied conditions, with aortic dilatation commonly observed even in the absence of valve dysfunction. Neo-aortic root enlargement postarterial switch operation and root dilation in Tetralogy of Fallot were also prevalent. The decision for surgical intervention is individualized; it is based on overall balance of procedural versus observation risk (ie, aortic diameter thresholds), patient risk factors, suitability for valve preservation, and the presence of concurrent cardiac lesions. However, there remains heterogeneity in imaging protocols, surgical thresholds, and long-term outcome data.
Conclusions
Aortopathies in ACHD patients represent a significant long-term complication requiring lifelong imaging surveillance and individualized surgical management. Despite growing recognition, evidence remains limited regarding optimal thresholds for surgical intervention and personalized risk stratification.
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