Valve Clinic

▶ Aortopathy
▶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:
Absolute diameter <45mm:
Repeat CT/MRI every 2 years

Absolute diameter >45mm:
Repeat 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
Loeys-Dietz:
In all cases:
- Repeat head-to-pelvis 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

▶ Valve Disease
▶Aortic Valve

▶Sclerotic Aortic Valve

In all cases:
No routine follow-up
This describes an aortic valve with thickened and/or slightly restricted leaflets, but without any significant restriction to blood flow.

▶Aortic Stenosis

Mild aortic stenosis (with trileaflet valve):
Repeat echo in 3 years
Generally, no cardiology input required, unless associated aortopathy >40mm (see section below)
Mild aortic stenosis (with bicuspid valve):
Routine cardiology referral
Known cases should ideally already be under cardiology follow-up
Moderate aortic stenosis:
Routine cardiology referral

But if any of the following features: Urgent cardiology referral
- New left ventricular systolic function (EF <55%) with no other cause and/or reducing ejection fraction on subsequent scans
- Symptoms (syncope, chest pain, breathlessness, paroxysmal nocturnal dyspnoea, oedema)
Severe aortic stenosis:
Urgent cardiology referral
Low-flow/Low-gradient aortic stenosis:
Urgent cardiology referral
This describes a valve which has either Moderate or Severe aortic stenosis, but not possible to differentiate between grades due to low stroke volume and flow rates. Further testing may be required (either stress echo or CT calcium score), if clinically indicated.

▶Aortic Regurgitation

Mild aortic regurgitation (with trileaflet valve):
No routine follow-up
Mild aortic regurgitation (with bicuspid valve):
Routine cardiology referral
Moderate aortic regurgitation:
Routine cardiology referral

But if any of the following features: Urgent cardiology referral
- Aortic root and/or proximal ascending aorta >40mm (by absolute values)
- Dilated LV and/or reducing ejection fraction on subsequent scans
- Symptoms (syncope, chest pain, breathlessness, paroxysmal nocturnal dyspnoea, oedema)
Severe aortic regurgitation:
Urgent cardiology referral

▶Bicuspid Aortic Valve

All first degree relatives should be offered transthoracic echo for screening.

Bicuspid aortic valve (new finding), mild stenosis or regurgitation:
Routine cardiology referral

Bicuspid aortic valve (new finding), moderate stenosis or regurgitation:
Routine cardiology referral

But if any of the following features: Urgent cardiology referral
- Aortic root and/or proximal ascending aorta >40mm (by absolute values)
- Dilated LV and/or reducing ejection fraction on subsequent scans
- Symptoms (syncope, chest pain, breathlessness, paroxysmal nocturnal dyspnoea, oedema)
Bicuspid aortic valve (new finding), severe stenosis or regurgitation:
Urgent cardiology referral

▶Mitral Valve

▶Mitral Stenosis

Mild mitral stenosis (normal valve anatomy - degenerative):
No routine follow-up
Mild mitral stenosis (abnormal valve anatomy – rheumatic/parachute/congenital):
Repeat echo in 3-5 years
Moderate mitral stenosis:
Routine cardiology referral

But if any of the following features: Urgent cardiology referral
- Estimated pulmonary artery systolic pressure >50mmHg and/or right ventricular impairment
- New atrial fibrillation
- New TIA/stroke
- Symptoms (syncope, chest pain, breathlessness, paroxysmal nocturnal dyspnoea, oedema)
Severe mitral stenosis:
Urgent cardiology referral

▶Mitral Regurgitation

Mild mitral regurgitation (normal valve):
No routine follow-up
Mild mitral regurgitation (abnormal valve – MV prolapse, rheumatic, etc)
Routine cardiology referral
Moderate mitral regurgitation:
Routine cardiology referral

But if any of the following features: Urgent cardiology referral
- Dilated LV and/or ejection fraction <60%
- Estimated pulmonary artery systolic pressure >50mmHg and/or right ventricular impairment
- New atrial fibrillation
- Indexed LA volume >60ml/m2 (by BSA)
- Symptoms (syncope, chest pain, breathlessness, paroxysmal nocturnal dyspnoea, oedema)
Severe mitral regurgitation:
Urgent cardiology referral

▶Mitral Annular Dysjunction

TBC

▶Tricuspid Valve

▶Tricuspid Stenosis

Vanishingly rare!
Routine cardiology referral

▶Tricuspid Regurgitation

Mild tricuspid regurgitation (normal valve anatomy and normal RV size/function):
No routine follow-up
Mild tricuspid regurgitation (with abnormal valve and/or impaired/dilated RV):
Routine cardiology referral
Moderate tricuspid regurgitation:
Routine cardiology referral

But if any of the following features: Urgent cardiology referral
- Estimated pulmonary artery systolic pressure >50mmHg and/or right ventricular impairment
- Symptoms (syncope, chest pain, breathlessness, paroxysmal nocturnal dyspnoea, oedema)
Severe tricuspid regurgitation:
Urgent cardiology referral

▶Pulmonary Valve

▶Pulmonary Stenosis

Generally due to known congenital abnormality - in all cases:
Cardiologist led follow-up

But if any of the following features: Urgent cardiology referral
- Estimated pulmonary artery systolic pressure >50mmHg and/or right ventricular impairment
- Symptoms (syncope, chest pain, breathlessness, paroxysmal nocturnal dyspnoea, oedema)

▶Pulmonary Regurgitation

Generally due to known congenital abnormality - in all cases:
Cardiologist led follow-up

But if any of the following features: Urgent cardiology referral
- Estimated pulmonary artery systolic pressure >50mmHg and/or right ventricular impairment
- Symptoms (syncope, chest pain, breathlessness, paroxysmal nocturnal dyspnoea, oedema)