Intracranial Aneurysms in Patients With Coarctation of the Aorta

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I n this issue of JACC: Advances, Buckley et al 1 examine the link between intracranial aneurysms (IAs) and coarctation of the aorta (CoA), and consider the implications for asymptomatic screening.CoA is one of the commonest forms of congenital heart disease, with an estimated prevalence around 4:10,000 live births. 2 Surgical repair of CoA was first described in 1944, 3 significantly improving the natural history of the disease.Nonetheless, historical studies reported reduced survival rates even after surgical repair, with a mean age at death of 38 years. 4Age at the time of surgical repair emerged as the most important predictor for the development of future hypertension and long-term survival.It is not surprising that with the development and implementation of new diagnostic modalities, and advances in surgical, interventional, and pharmacological strategies, the life expectancy of patients with CoA has been transformed, with an actuarial survival rate of 89% at 60 years. 5e association between CoA and IA was first described in 1871 and was followed by several case reports 6  with some earlier studies reporting a prevalence of 10% 7,8 and other contemporary cohorts reporting a 0% prevalence. 9Further evidence suggesting a link between CoA and IA was investigated in a populationbased study of patients diagnosed with stroke.It found that stroke patients with CoA had higher rates of subarachnoid hemorrhage when compared to those without coarctation (11.8% vs 4.8%, P ¼ 0.039), and that among patients with subarachnoid hemorrhage, the prevalence of unruptured IAs was higher in patients with CoA compared with those without (23% vs 2.5%, P ¼ 0.002). 10Hypertension has been recognized as a risk factor for the development and rupture of IA in the general population 11 ; however, the impact of the age at hypertension diagnosis or antihypertensive medical treatment has been inconsistently studied in these cohorts.
In this issue of JACC: Advances, Buckley et al 1 present the findings of their meta-analysis investigating the prevalence and risk factors for IA in patients with CoA.The authors included five studies published over a 17-year span.They included 442 patients, of whom 27 were diagnosed with an IA, estimating a pooled prevalence of 3.8% (95% CI: 0.1%-12.3%),similar to the 3.2% prevalence in the general population. 11Buckley et al 1  The authors attest they are in compliance with human studies committees and animal welfare regulations of the authors' institutions and Food and Drug Administration guidelines, including patient consent where appropriate.For more information, visit the Author Center.A more recent study that followed 1,960 aneurysms for 7,388 aneurysm years found that aneurysms $5 mm were associated with increased risk of rupture when compared to aneurysms of 2 to 4 mm. 12Surgical or percutaneous treatment strategies have high morbidity and mortality risks and are only recommended in patients with high-risk features. 11nce this meta-analysis by Buckley et al 1  In this vein, a cost analysis study suggested that screening for IA in patients with repaired CoA was cost-effective at 10, 20, and 30 years of age in a simulated cohort. 13However, the authors used an estimated IA prevalence of 10%, which likely reflects an overestimate in a contemporary population of patients with repaired CoA.Furthermore, previous studies have failed to detect IAs in younger patients with CoA (mean age at brain imaging: 16 years), 14 which also conflicts with this cost analysis study.
Current guidelines are also discrepant with regard to IA screening: the American Heart Association suggests that screening of IA in patients with CoA may be reasonable, 15 whereas the European Society of Cardiology suggests that routine screening is not recommended. 16 answer our question, "To screen or not to screen?", we conclude that the prevalence of IAs in patients with CoA who have received the current standard of care is similar to the prevalence of IAs in the general population (Figure 1).A universal screening strategy is therefore likely to detect mostly also highlight the role of modifiable risk factors in the development of IAs in patients with CoA.Among the available risk factors, the presence of systemic arterial hypertension was the only univariate predictor of IA, with an odds ratio of 3.1 (95% CI: 1.1-8.2;P ¼ 0.03).The authors emphasize the down trending incidence of IAs between earlier and modern studies, ISSN 2772-963X https://doi.org/10.1016/j.jacadv.2023.100430*Editorials published in JACC: Advances reflect the views of the authors and do not necessarily represent the views of JACC: Advances or the American College of Cardiology.From the Division of Cardiology, University of Toronto, University Health Network and Mount Sinai Health System, Toronto, Ontario, Canada.
N D A T I O N .T H I S I S A N O P E N A C C E S S A R T I C L E U N D E R T H E C C B Y L I C E N S E ( h t t p : / / c r e a t i v e c o m m o n s .o r g / l i c e n s e s / b y / 4 .0 / ) .with the latter reporting a lower IA rate.We agree with Buckley et al in that CoA is frequently diagnosed even before birth, and interventions are performed earlier in life; moreover, the increasing numbers of specialized adult congenital heart disease centers, the known relationship of CoA with the development of systemic arterial hypertension, and the deleterious effects of the latter, have prompted better surveillance and treatment strategies in this population.An earlier age at CoA diagnosis and treatment could also reflect lower coarctation-years and hypertensiveyears exposure, likely reducing the risk of the development of future IAs.The American Heart Association 2015 stroke guidelines suggest that an aneurysm measuring <7 mm is associated with a very low risk of rupture.11 mm, it brings into question the true benefit of asymptomatic screening in this population.

FIGURE 1
FIGURE 1 Intracranial Aneurysms in Patients With Coarctation of the Aorta until 2003, when the first prospective study