New recommendations on comprehensive care for patients with aortic disease have been published by Isselbacher et al. in the “2022 ACC/AHA Guideline for the Diagnosis and Management of Aortic Disease”.
These guidelines include specific recommendations on imaging for the diagnosis and follow-up of aortic pathologies. The strengths and weaknesses of the different usable techniques are collected: TTE, TEE, CT, MRI and abdominal ultrasound. Reference is made to the essential elements that a CT and MRI report should include, specifying, among other things, how to make the measurements, the acquisition method, as well as the convenience of comparing with previous studies. Serial CT studies should take into account the risk of cancer, radiation protocols should be optimized and the alternative use of MRI should be considered. Special mention is made of how the measurements should be performed according to the different imaging techniques in each of the aortic segments. Moreover, for each of the techniques the guidelines make a specific review regarding the proper scenario for each one along with their advantages and disadvantages.
With respect to surgical intervention, the new ACC/AHA guidelines state that smaller aneurysms (i.e. in sporadic patients ≥5.0 cm or in Marfan syndrome ≥4.5cm) can be considered to be operated upon with a COR2A recommendation when the patient is taken care of in an experienced center, while watchful waiting should be considered in other circumstances. While the new guidelines do emphasize the importance of experienced teams with a high rate of procedures, there is no explicit advice on referral. In addition, there is no statement yet on the application of newer surgical techniques (e.g. PEARS) or gender-specific considerations. Finally, it is notable that the document reports valve-sparing aortic root replacement as a COR2b indication for Heritable Thoracic Aortic Disease (HTAD) - which is contrary to the ESC guidelines (class 1 in the 2020 ACHD guidelines) (1).
In the publication, there is a fairly extensive section on genetic disorders of the aorta. A table is provided listing genetic and clinical findings of the most common syndromic Heritable Thoracic Aortic Disease (HTAD), non-syndromic HTAD and congenital disorders. Attention is paid to indications for genetic testing (including a flow chart) and imaging screening in family members of patients with HTAD in which the importance of noting the aortic diameter in type A dissection is emphasized, which is obviously necessary for further management of family members. Risk factors are mentioned to identify patients for whom genetic screening should definitely be considered - an age limit of 60 years for aortic dissection is given, which may be relatively low - but later in the text this is modified by stating that there is no upper age limit for dissection in genetic aortic disease. Recommendations are relatively non-committal about the need for genetic counselling, which is recommended only in cases of proven genetic abnormality and otherwise reported as "useful". Guidance is given on both clinical and genetic screening in the section on cascade screening. As already commented on above, one goes on to discuss follow-up (incl. imaging) and treatment (surgical) in Marfan and Loeys-Dietz syndrome with some additional granularity according to the underlying gene defect. Medical treatment is also discussed. In line with the results of the recently published ARB versus beta-blocker meta-analysis in Marfan syndrome (2), ARB administration is now considered a valid alternative (or add-on) for beta-blocker therapy while in previous guidelines ARBs were mostly recommended in those who are intolerant for beta-blockers.
The document also contains dedicated chapters on pregnancy and exercise in patients with aortopathy. Pregnancy recommendations are very similar to the ESC guidelines (2018) (3), emphasizing on the need for pre-pregnancy counseling (about the heritability as well the risk for dissection) and aortic imaging prior to pregnancy. The need for multidisciplinary management and blood pressure control (beta-blocker therapy) is also expressed in the recommendations. There is no wording about options for prenatal/pre-implantation genetic testing. Modes of delivery taking the aortic diameter into account are provided as well as recommendations for pre-pregnancy aortic root surgery. The latter also take the underlying condition (Marfan, Loeys-Dietz, Turner syndrome, nonsyndromic HTAD and bicuspid aortic valve) as well as the specific gene (for Loeys-Dietz syndrome) into account. The exercise recommendations are largely in line with the aortopathy recommendations in the 2020 ESC Guidelines on sports cardiology and exercise in patients with cardiovascular disease (4). They highlight the importance of individual risk assessment and strict blood pressure control. Intense isometric exercise, burst activities and collision sports should be avoided in high-risk individuals, while it is reasonable to encourage mild-to-moderate intensity aerobic training in patients whose blood pressure is adequately controlled. In contrast to the 2020 ESC Guidelines, exercise testing with blood pressure monitoring prior to exercise engagement is not mentioned.
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