Intracranial aneurysms (ICAs) occur in patients with Coarctation of the Aorta (CoA), who are thus at risk for both ischemic and hemorrhagic stroke. However, recommendations to screen for their presence in this patient population are variable. The 2018 ACC/AHA guidelines  for the care of the adult with congenital heart disease (CHD) suggest that screening for ICAs by magnetic resonance angiography (MRA) or computed tomography angiography (CTA) may be reasonable in adults with CoA (COR IIb, LOE B-NR).¹ In contrast, routine imaging to assess for ICAs in asymptomatic patients with CoA was not recommended in the 2020 ESC Guidelines.²  In the pediatric age group, screening practices are likewise variable and inconsistent.

This poll evaluated the customary approach to imaging surveillance for ICAs in patients with CoA. The poll garnered opinions from 140 responders from 20 different countries, with the majority being in the United States. Most respondents (41.4%) obtain head imaging only in patients with CoA who are older than 12 years old. Slightly over a tenth of respondents (14.3%) recommend head imaging only in patients who have had long standing hypertension. Interestingly, among our poll respondents, less than 10% screen for aneurysms in young children, with only 2.9% performing head imaging in infants within the first year of life, and 4.3% recommending surveillance in those younger than 12 years of age. Just over 37% do not obtain head imaging routinely in this patient population.

ICAs have a higher incidence in older patients with CoA.³ Compared to the general population, these adult patients suffer from both ischemic and hemorrhagic stroke at significantly younger ages.⁴ On the other hand, children with CoA who are treated early in life appear to not have a higher incidence of ICAs.⁵ This may question the previously held belief of a developmental relationship between CoA and ICAs, as well as the value of screening for cerebral vascular anomalies in infants and young children with isolated disease. This may also suggest that there are perhaps some modifiable risk factors, such as long-standing hypertension, that may be responsible for the development of ICAs in patients with CoA who are diagnosed and treated in later adulthood.⁶

References

1. Stout KK, Daniels CJ, Aboulhosn JA, et al. 2018 AHA/ACC guideline for the management of adults with congenital heart disease: a report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines. J Am Coll Cardiol 2018;73:1494-1563.
2. Baumgartner H, De Backer J, Babu-Narayan SV, et al. 2020 ESC guidelines for the management of adult congenital heart disease: the Task Force for the Management of Adult Congenital Heart Disease of the European Society of Cardiology (ESC). Eur Heart J 2020;Aug 29:[Epub ahead of print].
3. Cook SC, Hickey J, Maul TM, et al. Assessment of the cerebral circulation in adults with coarctation of the aorta. Congenit Heart Dis 2013;8:289-95.
4. Pickard SS, Gauvreau K, Gurvitz M, et al. Stroke in adults with coarctation of the aorta: a national population-based study. J Am Heart Assoc 2018;7:e009072.
5. Donti A, Spinardi L, Brighenti M, et al. Frequency of intracranial aneurysms determined by magnetic resonance angiography in children having operative or endovascular treatment of coarctation of the aorta. Am J Cardiol 2015;116:630-3.
6. Curtis SL, Bradley M, Wilde P, et al. Results of Screening for intracranial aneurysms in patients with coarctation of the aorta. AJNR Am J Neuroradiol 2012;33:1182-86.

Clinical Topics: Congenital Heart Disease and Pediatric Cardiology, Invasive Cardiovascular Angiography and Intervention, Noninvasive Imaging, Prevention, Vascular Medicine, Congenital Heart Disease, CHD and Pediatrics and Imaging, CHD and Pediatrics and Interventions, CHD and Pediatrics and Prevention, CHD and Pediatrics and Quality Improvement, Interventions and Imaging, Interventions and Structural Heart Disease, Interventions and Vascular Medicine, Angiography, Magnetic Resonance Imaging, Hypertension

Keywords: Aortic Coarctation, Magnetic Resonance Angiography, Intracranial Aneurysm, Incidence, Risk Factors, Stroke, Aorta, Intracranial Hemorrhages, Hypertension, Pediatrics, ESC Congress, ESC20

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