Bicuspid Aortic Valve-Related Aortopathy

Publication Date: May 15, 2018

Recommendations

Initial Imaging

TTE is the initial imaging modality of choice for assessment of the aortic valve and thoracic aorta in patients with BAV.
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The entire thoracic aorta should be measured by TTE, reporting each aortic segment separately in millimeters: root (sinuses of Valsalva), STJ, tubular ascending aorta (proximal, mid, and distal), arch, and descending thoracic aorta. Maximum diameter, regardless of location, should be reported. Aortic coarctation should be ruled out with Doppler evaluation of the descending thoracic aorta and abdominal aorta.
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If TTE cannot visualize any aortic segment, any segment measures 45 mm, or aortic coarctation cannot be ruled out, recommend assessment of the entire thoracic aorta with ECG-gated cardiac MRA or CTA.
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If a patient is undergoing cardiac surgery and root or tubular ascending aorta measure 40-44 mm by TTE, recommend assessment of the thoracic aorta with MRA or CTA before surgery.
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If aortic coarctation is present, screening for cerebral aneurysms is recommended.
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Interval Monitoring Imaging

Interval imaging should be performed with the same imaging technique and measurement method, and compared side-by-side with previous study by an expert in that imaging technique.
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Interval aorta imaging recommendations apply to patients with native BAV and those who have undergone AVR, given that aorta complications may occur in patients with BAV postsurgery.
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In patients with normal initial aortic diameters by TTE, the thoracic aorta should be reimaged every 3 to 5 y.
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In patients with initial aortic dilatation (root or tubular ascending aorta measure 40-49 mm), the thoracic aorta should be reimaged at 12 mo. If stability is confirmed, then reimaging can be performed every 2 or 3 y.
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In patients with more advanced initial aortic dilatation (root or tubular ascending aorta measure 50-54 mm), the thoracic aorta should be reimaged at least every 12 mo (yearly).
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If thoracic aortic dilation (45 mm) noted by TEE is not reproducible with CTA or MRA (ie, >2-mm difference between modalities), then interval imaging follow-up should be performed with MRA or CTA.
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Aortic Repair

Repair of the ascending aorta/root is recommended when the aortic diameter is 55 mm in patients without risk factors.
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Repair of the ascending aorta/root should be performed when the aortic diameter is 50 mm in patients with risk factors (ie, root phenotype or predominant AI, uncontrolled hypertension, family history of aortic dissection/sudden death, coarctation, aortic growth >3 mm/y).
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Repair of the ascending aorta/root may be performed in patients with an aortic diameter of 50 mm when the patients are at low surgical risk and operated on by an experienced aortic team in a center with established surgical results.
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Concomitant repair of the ascending aorta/root should be performed when the aortic diameter is 45 mm in patients undergoing cardiac surgery.
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Repair of the aortic arch is recommended in patients with an aortic arch diameter of 55 mm.
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Concomitant repair of the aortic arch should be performed in patients undergoing cardiac surgery with an aortic arch diameter of 50 mm.
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Concomitant repair of the aortic arch may be performed in patients undergoing cardiac surgery with an aortic arch diameter of 45 mm, provided the patients are at low surgical risk and operated on by an experienced aortic team with established surgical results.
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It is recommended that patients undergoing elective aortic arch repair be referred to an experienced aortic team with established surgical results.
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Postsurgical Repair, Medical Management, and Watchful Waiting
 

Radiologic imaging (with CTA or MRA) may be performed after aortic surgery to establish a postrepair baseline.
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Ongoing postoperative surveillance intervals should be individualized on the basis of the clinical, anatomic, and surgical features. In the presence of residual aortic dilation/ pathology, it is reasonable to image the entire aorta every 3-5 y by CTor MRI after repair.
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MRI should be considered for repeat examinations in an adolescent or in the adult population aged <50 y.
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Treatment of hypertension is recommended according to country- and region-specific guidelines.
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Beta-blockers and inhibitors of the reninangiotensin system should be considered for blood pressure control based on evidence extrapolated from populations with connective tissue disease. Nonpharmacologic approaches (salt reduction, weight reduction) should be advocated as part of blood pressure control strategies.
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Patients with aortic aneurysms that are at or near surgical thresholds for correction should avoid strenuous lifting, pushing, or straining that would require a Valsalva maneuver.
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It is recommended to avoid heavy weight lifting or competitive athletics involving isometric exercise when the ascending aortic diameter is >45 mm.
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Patients with BAV and dilated aorta should be precluded from private driving if the ascending aorta diameter is >6.0 and restricted from commercial driving if the ascending thoracic aorta diameter is >5.5 cm.
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It is recommended that prepregnancy evaluation and postpregnancy management of women with BAV with or without associated aortopathy be performed by practitioners with expertise in the management of pregnant women with heart disease.
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First-degree relatives of patients with BAV should undergo screening echocardiography.
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Recommendation Grading

Disclaimer

Overview

Title

Bicuspid Aortic Valve-Related Aortopathy

Authoring Organization

Publication Month/Year

May 15, 2018

Supplemental Implementation Tools

Document Type

Guideline

External Publication Status

Published

Country of Publication

US

Document Objectives

 Comprehensive review of BAV-related aortopathy and its management.

Inclusion Criteria

Female, Male, Adult, Older adult

Health Care Settings

Ambulatory, Hospital, Operating and recovery room

Intended Users

Nurse, nurse practitioner, physician, physician assistant

Scope

Diagnosis, Management, Treatment

Diseases/Conditions (MeSH)

D001021 - Aortic Valve, D001024 - Aortic Valve Stenosis, D001022 - Aortic Valve Insufficiency, D001018 - Aortic Diseases

Keywords

BAV, aortopathy, Bicuspid aortic valve, bicuspid valve, aortic repair

Source Citation

Dvir D, Bourguignon T, Otto CM, Hahn RT, Rosenhek R, Webb JG, et al. Standardized Definition of Structural Valve Degeneration for Surgical and Transcatheter Bioprosthetic Aortic Valves. Circulation. 2018;