Screenings if you have a bicuspid aortic valve

The main guidance

In the US, the 2022 ACC/AHA aortic disease guideline has a section on the aorta in people with a bicuspid aortic valve, including imaging and family screening. (ACC/AHA aortic, 2022) The American Association for Thoracic Surgery (AATS) published consensus guidelines on bicuspid valve aortopathy in 2018, with set scan intervals. (AATS, 2018) In the UK, NICE guideline NG208 on heart valve disease in adults covers specialist referral and how often the valve is checked. (NICE NG208)

Checks the guidance lists

Check	When	Source
Echocardiogram of the valve, aortic root and ascending aorta, and a check for narrowing of the aorta (coarctation)	At diagnosis	(ACC/AHA aortic, 2022)
Referral to a heart specialist	Any bicuspid aortic valve, including mild valve disease	(NICE NG208)
CT or MRI of the chest aorta	When the echocardiogram cannot measure the aorta fully	(ACC/AHA aortic, 2022)(AATS, 2018)
Aorta scan, aorta a normal size	Every 3 to 5 years	(AATS, 2018)
Aorta scan, aorta 40 to 49 mm	At 12 months, then every 2 or 3 years if stable	(AATS, 2018)
Aorta scan, aorta 50 to 54 mm	At least every 12 months	(AATS, 2018)
Echocardiogram for mild aortic stenosis	Every 3 to 5 years	(NICE NG208)
Clinical review with an echocardiogram for severe valve disease without symptoms	Every 6 to 12 months	(NICE NG208)
Echocardiogram for parents, siblings and children	Once the bicuspid valve is found	(ACC/AHA aortic, 2022)(AATS, 2018)

The aorta

The 2022 ACC/AHA guideline says a widened aorta is reported in 20% to 84% of people with a bicuspid aortic valve, depending on the group studied, and that people with a bicuspid valve and a widened aorta are at risk of aortic dissection (a tear in the aortic wall). (ACC/AHA aortic, 2022) It recommends an echocardiogram at diagnosis to look at the valve, the aortic root and ascending aorta and to check for coarctation, and a CT or MRI when the echocardiogram cannot show the aorta fully. It says people with a bicuspid aortic valve need lifelong imaging of the aorta, even after valve replacement, and recommends lifelong imaging by echocardiogram, CT or MRI once the aortic root or ascending aorta measures 4.0 cm or more, at an interval set by the diameter and how fast it is growing. (ACC/AHA aortic, 2022) For any widened chest aorta, it says a repeat scan at 6 to 12 months to measure growth is reasonable, then every 6 to 24 months if stable, depending on the diameter. (ACC/AHA aortic, 2022)

The AATS consensus gives set intervals: every 3 to 5 years if the first scan is normal, at 12 months and then every 2 or 3 years if the root or ascending aorta is 40 to 49 mm, and at least yearly at 50 to 54 mm. It says the first repeat scan may be done at 6 months when there is coarctation or a family history of dissection, that the same type of scan should be used each time, and that these intervals also apply after valve replacement. (AATS, 2018) An abdominal aortic aneurysm is a different check; see abdominal aortic aneurysm screening.

The valve

MedlinePlus says calcium builds up sooner on the valve in people born with a bicuspid valve, which narrows it (aortic stenosis). (MedlinePlus) NICE says mild valve disease is common and rarely becomes significant, but offers referral to a specialist for a bicuspid aortic valve of any severity. It suggests an echocardiogram every 3 to 5 years for mild aortic stenosis, and a clinical review with an echocardiogram every 6 to 12 months for severe valve disease without symptoms when treatment is not yet needed, with the timing based on the echo findings and a shared decision with the patient. (NICE NG208)

Parents, siblings and children

The 2022 ACC/AHA guideline says a bicuspid valve is found in 9% to 20% of relatives of someone who has one, and that relatives can also have a widened aorta with a normal valve. It recommends an echocardiogram for all first-degree relatives when the person has a bicuspid valve and a widened aorta, with a CT or MRI if the echo cannot show the aorta fully, and calls it reasonable for the relatives of everyone with a bicuspid aortic valve. If a relative is found to be affected, it says their own relatives should then be checked. (ACC/AHA aortic, 2022) It recommends a medical genetics evaluation when a bicuspid valve comes with a heritable aortic condition or features of Loeys-Dietz syndrome. (ACC/AHA aortic, 2022) See family history and screening and how to collect your family health history.

Blood pressure

The AATS consensus says there are no data to support lower blood pressure thresholds for people with a bicuspid aortic valve and a widened aorta, so the usual national high blood pressure guidelines apply. (AATS, 2018) See blood pressure screening and screenings if you have high blood pressure.

Before or during pregnancy

NICE says to refer women who are pregnant or considering a pregnancy to a cardiologist with expertise in pregnancy if they have a bicuspid aortic valve of any severity with a widened aorta, whether or not they have symptoms. (NICE NG208) The AATS consensus says checks before and after pregnancy should be done by practitioners with expertise in pregnancy and heart disease. (AATS, 2018)

Routine age-based screening

These checks are added to, not instead of, the routine screenings for your age; see screenings by age and gender and heart screenings by age.

When to contact a provider

MedlinePlus, aortic stenosis, when to contact a medical professional: (MedlinePlus)

Contact your provider if you or your child has symptoms of aortic stenosis.
Also contact your provider right away if you have been diagnosed with this condition and your symptoms get worse or new symptoms develop.

Where they differ:

Set intervals. The AATS consensus gives fixed aorta scan intervals by diameter (every 3 to 5 years when normal, every 2 or 3 years at 40 to 49 mm, yearly at 50 to 54 mm); (AATS, 2018) the 2022 ACC/AHA guideline sets no fixed interval for a bicuspid valve and ties it to the diameter and growth rate, with 6 to 24 months for any widened chest aorta. (ACC/AHA aortic, 2022)
Family screening. The ACC/AHA guideline makes relative screening a firm recommendation only when the aorta is widened and calls it reasonable otherwise; (ACC/AHA aortic, 2022) the AATS consensus says first-degree relatives should be screened whatever the aorta size; (AATS, 2018) NICE NG208 does not cover family screening. (NICE NG208)
Specialist care. NICE refers every adult with a bicuspid aortic valve to a specialist, even with mild valve disease, while saying mild valve disease in general rarely progresses. (NICE NG208)
Type of scan. The ACC/AHA guideline leaves the choice between CT and MRI to the person and the center; (ACC/AHA aortic, 2022) the AATS consensus favors MRI for repeat scans under age 50. (AATS, 2018)

Your doctor decides what your result means for you.

Questions people ask

What is a bicuspid aortic valve?

It is an aortic valve with two flaps (cusps) instead of three, present from birth. The 2022 ACC/AHA aortic guideline says it affects about 1% of people, is 2 to 3 times more common in males, and is often linked with a widened aortic root or ascending aorta. (ACC/AHA aortic, 2022)

Do I need checks even if my valve works well?

Yes, according to these guidelines. The 2022 ACC/AHA guideline says people with a bicuspid aortic valve need lifelong imaging of the aorta, even after valve replacement. NICE offers referral to a heart specialist for a bicuspid aortic valve of any severity, including mild valve disease. (ACC/AHA aortic, 2022) (NICE NG208)

How often is the aorta scanned?

The 2018 AATS consensus says every 3 to 5 years if the aorta is a normal size, again at 12 months and then every 2 or 3 years if it measures 40 to 49 mm, and at least every year if it measures 50 to 54 mm. The 2022 ACC/AHA guideline links the interval to the diameter and how fast it is growing. (AATS, 2018) (ACC/AHA aortic, 2022)

Should my children, parents and siblings be checked?

The 2022 ACC/AHA guideline says an echocardiogram for all first-degree relatives (parents, siblings and children) is recommended when the aorta is widened, and reasonable for every person with a bicuspid aortic valve. The AATS consensus says first-degree relatives should have a screening echocardiogram. (ACC/AHA aortic, 2022) (AATS, 2018)

Why are CT or MRI scans sometimes used?

The 2022 ACC/AHA guideline says a CT or MRI of the chest aorta is indicated when an echocardiogram cannot measure the aortic root or ascending aorta accurately or completely. The AATS consensus says people under 50 would benefit from MRI for repeat scans, to avoid CT radiation. (ACC/AHA aortic, 2022) (AATS, 2018)