Specialist Guidance

More detailed specialist guidance for valve clinic use etc.
▶ Aortopathy
UHBW CLinical Guidelines for the Management of Chronic Thoracic Aortic Disease - Click Here
▶New Finding of Dilated Aorta

All patients should have cardiovascular risks managed as appropriate.

▶Absolute diameter <40mm (root or proximal ascending aorta)

Age >60:
No routine follow-up
Age <60 with no features of underlying disease (see below):
No routine follow-up
Age <60 with strong suspicion of underlying disease (see below):
Routine cardiology referral
Features of underlying disease:
- 1st degree family history of dissection at young age (<70 years without hypertension, or <60 years with hypertension)
- Bicuspid aortic valve
- Suspected genetic disposition (e.g. Marfan / Loeys-Dietz / Ehlers-Danlos)

▶Absolute diameter 41-49mm (root or proximal ascending aorta)

Tricuspid or Bicuspid aortic valve (with no more than mild stenosis/regurgitation):
Routine cardiology referral
(For baseline CT/MRI)
Genetic disposition (e.g. Marfan / Loeys-Dietz / Ehlers-Danlos):
Urgent cardiology referral
(For baseline CT/MRI and genetic testing)

Advise patient that if they develop chest pain - they should call 999 and attend their local emergency department immediately.

▶Absolute diameter >50mm (root or proximal ascending aorta)

In all cases:
Urgent cardiology referral
(For baseline CT/MRI and consideration for surgery)

Advise patient that if they develop chest pain - they should call 999 and attend their local emergency department immediately.

▶Follow-up for Known Aortopathy

In most cases, follow-up for aortopathy should be cardiologist-led.

A brief/general overview of surveillance for those eligible for surgery (but not requiring immediate intervention) is as follows:

1. Baseline echo and cross-sectional imaging (CT/MRI)

2. After 1 year, either:
- a. Repeat echo (If baseline echo images are adequate and no suspected hereditary cause), or
- b. CT/MRI (If baseline echo images inadequate and/or suspected hereditary cause)

Ongoing surveillance is based on progression over the 1-year period.

▶Tricuspid aortic valve (no suspected/known hereditary cause)

Absolute diameter 41-44mm, stable at 1 year:
Repeat echo every 5 years
Absolute diameter 45-49mm, stable at 1 year:
Repeat echo every 1 year
Repeat CT/MRI every 2 years
Absolute diameter 50-54mm and/or significant growth:
Repeat CT/MRI every 1 year

▶Bicuspid aortic valve (no suspected/known hereditary cause)

Absolute diameter 41-44mm, stable at 1 year:
- Repeat echo every 1 year
- Repeat CT/MRI every 2 years
Absolute diameter 45-52mm and/or significant growth:
- Repeat CT/MRI every 1 year

▶Hereditary thoracic aortic disease (Marfan, Loeys-Dietz, Ehlers-danlos etc)

Marfan Syndrome:
Good echo windows:
Repeat echo every 1 year

Poor echo windows, absolute diameter >45mm:
Repeat CT/MRI every 1 year

Poor echo windows, absolute diameter >45mm:
Repeat CT/MRI every 1 year
Loeys-Dietz:
Good echo windows:
Repeat echo every 1 year
Poor echo windows:
Repeat head-to-pelvis CT/MRI every 1 year
Ehlers-Danlos:
vEDS with completely normal vasculature:
Repeat CT/MRI every 3-5 years

vEDS with any abnormal vasculature:
Repeat CT/MRI every 1 year
Turner Syndrome:
Aorta Size Index (ASI) <20mm/m2:
- Repeat CT/MRI every 5 years

Aorta Size Index (ASI) 20-23mm/m2:
Repeat CT/MRI every 2 years

Aorta Size Index (ASI) >23mm/m2 and/or additional risk factors:
Repeat CT/MRI every 1 year