Cardiac Amyloidosis Service

We are a group of medical professionals providing a specialist service for patients and their families with suspected or confirmed cardiac amyloidosis within Leeds, as well as across West Yorkshire, York and Harrogate.

Overview

Leeds Teaching Hospitals NHS Trust (LTHT) provides a specialist service for the assessment and diagnosis of cardiac amyloidosis, including transthyretin (ATTR) cardiac amyloidosis. This is provided by the heart failure department, both within LTHT heart failure clinics in addition to the LTHT heart muscle clinic.

We accept referrals from within LTHT, across West Yorkshire, York and Harrogate, offering access to specialist clinical review and advanced diagnostic testing.

There is a growing availability of treatments designed specifically to treat cardiac amyloidosis. Because of this, it is now more important than ever for timely diagnosis and referral for these new therapies where appropriate.

What is Cardiac Amyloidosis?

Cardiac amyloidosis is caused by abnormal proteins (amyloid) building up in the heart muscle. This makes the heart stiff and can lead to heart failure and heart rhythm problems.

The two main types affecting the heart are:

ATTR (transthyretin) amyloidosis
AL (light-chain) amyloidosis

It is important to identify the correct type, as the treatment is very different.

When to Consider Cardiac Amyloidosis

Cardiac amyloidosis should be considered in people with unexplained thickening of the heart muscle or symptoms of heart failure, particularly when this is not explained by common conditions such as high blood pressure.

Features that may raise suspicion include:

Thickened heart muscle thickness on echocardiography, especially with other risk factors
Breathlessness, ankle swelling or fatigue due to heart failure
Heart rhythm or conduction problems (for example atrial fibrillation or slow heart rates)
Thickened heart muscle pattern with low voltages on ECG
Poor tolerance of standard heart failure medications
A history of carpal tunnel syndrome, spinal stenosis or tendon problems
A family history of amyloidosis or unexplained heart disease

If the heart is thickened on imaging investigations and one or more of these features are present, cardiac amyloidosis should be considered and further investigation may be advised.

At LTHT we can provide the full diagnostic pathway for patients with suspected ATTR cardiac amyloidosis, including:

Specialist clinical assessment by an experienced heart failure clinician
Cardiac imaging, including echocardiography and cardiac MRI where indicated
Nuclear scintigraphy (99mTc-DPD scintigraphy) to support the diagnosis of ATTR cardiac amyloidosis
Genetic investigations for individuals with possible amyloidosis and their relatives, where required
Multidisciplinary results review and management with other specialist teams
Nuanced management of heart failure and arrhythmia in patients with confirmed amyloidosis
Onward referral for disease modifying therapies where appropriate
Determining future care plans for primary and secondary care

For patients referred from outside of LTHT, some parts of the diagnostic pathway will be completed locally before referral. This includes echocardiography, cardiac MRI where indicated, and blood and urine tests to exclude AL cardiac amyloidosis.

There are several ways in which patients are referred to the LTHT amyloidosis service.

GP referral – any patient suspected of having heart failure with an elevated NT-proBNP should be referred to secondary care (to the heart failure service) for further assessment. If there is a suspicion of cardiac amyloidosis following initial investigations, patients can be referred on to the heart muscle clinic.

Hospital referrals – any patient who has a suspected or confirmed diagnosis can be referred. Reasons for referral might include diagnostic confirmation, genetic testing, management advice, long term follow up, and family screening.

If referring from secondary care (both within and outside of LTHT), we ask that the following preliminary investigations are completed locally where possible:

12-lead ECG
Echocardiography
Blood tests
- Serum free light chains
- Serum protein electrophoresis and immunofixation
Urine tests:
- Urine protein electrophoresis and immunofixation

If a cardiac MRI has been performed, please include the report with the referral. For referrals from outside LTHT, image transfer of relevant cardiac imaging is also required.

These tests are important to look for or exclude AL amyloidosis before specialist assessment, allowing for appropriate referrals and management to be commenced on the first visit.

GP referrals should be made using standard procedures to secondary care services.

Referrals from within secondary care should be made by contacting one of the Heart Muscle Consultants at LTHT:

Dr Alex Simms
Dr Kate Gatenby
Dr Anshu Sengupta

Please include relevant clinical information, investigation results and imaging reports to support triage.

Following referral, patients will be reviewed by the cardiac amyloidosis team. This may include:

Specialist clinic review
Review of previous investigations
Further tests where required
Discussion at the multidisciplinary team (MDT) meeting:
- Clinicians referring from within secondary care may be invited to our Heart Failure MDT (on Tuesday morning) to discuss cases, or to discuss any specific management queries about patients with cardiac amyloidosis
Advice on diagnosis, treatment and ongoing care
Onward referral to the National Amyloid Centre (NAC) at the Royal Free hospital in London where appropriate
- Patient can either be seen in person in London, or remotely with a telephone clinic

For clinical questions: Contact the Heart Failure specialist nurses on 0113 3926420.
For appointments: Please contact 0113 3926476.

Meet the team

Consultant Cardiologist (Heart muscle, ICC and Device Therapy)
Dr Alex Simms

Consultant Cardiologist (Heart muscle, Valve Disease and Imaging)
Dr Kate Gatenby

Consultant Cardiologist (Heart muscle, ICC and Imaging)
Dr Anshu Sengupta

Clinical Nurse Specialist
Emma Goodman

Clinical scientist / Specialist echocardiographer
Gemma Bassindale

Specialist Echocardiographer
Charlotte Knowles

Secretary
Karen Sherburn

Consultant Haematologist
Dr Christopher Parrish

Consultant Haematologist
Dr Frances Seymour

Consultant Renal physician
Dr Richard Hoefield

Consultant Neurologist
Dr Shaun Foo

Consultant Neurologist
Dr Agam Jung

Consultant Neurologist
Dr Priya Shanmugharajah

Consultant Pathologist
Dr Kathryn Griffin

Consultant Radiologist & Nuclear Medicine Physician
Dr Fahmid Chowdhury