Inherited Cardiovascular Conditions

You have been redirected to this website because of the coronavirus pandemic.

This is affecting clinical services and we ask you to read the guidance below in the first instance.

We are trying to keep any disruption to your clinical care to a minimum, but please understand that services across the NHS are under extreme pressure.

Everyone is advised to wash their hands regularly with soap and warm water for 20 seconds.

General advice:

If you are concerned about coronavirus please look carefully at the NHS website: https://www.nhs.uk/conditions/coronavirus-covid-19/

For information regarding plans for the coronavirus vaccine, please look at the following NHS link: https://www.nhs.uk/conditions/coronavirus-covid-19/coronavirus-vaccination/coronavirus-vaccine/

If you were previously shielding due to having a condition that places you in a vulnerable group (e.g. cardiomyopathy with left ventricular impairment, obstruction, heart failure or symptoms thereof), the current NHS England guidance is available on their website: https://www.gov.uk/government/publications/guidance-on-shielding-and-protecting-extremely-vulnerable-persons-from-covid-19/guidance-on-shielding-and-protecting-extremely-vulnerable-persons-from-covid-19

If you need advice about a cardiomyopathy or how coronavirus affects it, most queries can be answered using the Cardiomyopathy UK website: https://www.cardiomyopathy.org/cardiomyopathy-information/covid-19-and-cardiomyopathy- . The charity also has a variety of online and telephone support services.

If you have urgent queries about your condition, unrelated to coronavirus, please email leedsth-tr.iccservice@nhs.net. Please bear in mind that we will be extremely busy over the coming months and it may take some time to respond. Emails will be triaged depending on clinical urgency.

Health services are under extreme pressure at the moment. Please follow national guidance first, and only seek specialist advice if absolutely necessary. We cannot advise on travel recommendations, general coronavirus related queries or specific vaccine related questions.

A lot of planned non-urgent care over the next few months may be postponed depending on government advice. You will be contacted if this affects you.

If you have a routine follow up appointment in the next few months, and your symptoms are stable you can contact us and change your appointment to a telephone consultation. Please call our admin team on 0113 392 6476.

Please read the advice below regarding your specific condition. If you feel your question hasn’t been answered, and it is genuinely urgent, please contact us via the following:

• During office hours: 07584 184602.
• If your query is non-urgent or is related to your inherited cardiac condition in general, please email leedsth-tr.iccservice@nhs.net

If you have Brugada syndrome or sodium-channel disease:

• If you feel well you don’t need to do anything differently.
• If you are concerned that you may have coronavirus, please follow the national guidance, available on https://www.nhs.uk/conditions/coronavirus-covid-19/ and/or https://111.nhs.uk/service/covid-19
• If you develop a fever, please follow our usual advice to get your temperature down quickly (cooling measures, paracetamol, and ibuprofen if the above do not help). We are aware of debate about the safety of ibuprofen in viral lung infections but there is no strong evidence of harm and we feel you would be at higher risk of harm if you continue to run a temperature. 
• If you do not have an ICD and your temperature is over 38.5°C despite paracetamol, contact NHS 111 by telephone and state/describe your condition. You may need to attend A&E for an ECG and heart rhythm monitoring, but your local A&E must be advised by NHS 111 of your attendance so that staff can wear appropriate protective equipment.
• If you have an ICD, you can isolate at home and follow NHS 111 guidance even if you have a high fever.
• If your temperature isn’t settling and you are struggling to reach NHS 111, call the ICC specialist nurses during working hours on 07584 184602, or the on-call Cardiology Registrar out of hours via LGI’s switchboard - 0113 243 2799. 

If you have Long QT syndrome:

• If you feel well you don’t need to do anything differently.
• If you are concerned that you may have coronavirus, please follow the national guidance, available on https://www.nhs.uk/conditions/coronavirus-covid-19/ and/or https://111.nhs.uk/service/covid-19
• If you are unwell and requiring medical attention, please remind doctors and nurses to avoid drugs that prolong the QT interval. It is also important to keep hydrated and replace salt levels as necessary especially if you are vomiting or have diarrhoea. If you cannot keep yourself well hydrated, please call 111.
• In time, certain antiviral therapies may become utilised and some of these can prolong the QT interval. Please remind healthcare workers that, if these are essential for your care, they arrange ECG monitoring during therapy.

If you have Catecholaminergic polymorphic VT (CPVT):

• If you feel well you don’t need to do anything differently.
• If you are concerned that you may have coronavirus, please follow the national guidance, available on https://www.nhs.uk/conditions/coronavirus-covid-19/ and/or https://111.nhs.uk/service/covid-19 

If you have idiopathic VF or early repolarisation syndrome:

• If you feel well you don’t need to do anything differently.
• If you are concerned that you may have coronavirus, please follow the national guidance, available on https://www.nhs.uk/conditions/coronavirus-covid-19/ and/or https://111.nhs.uk/service/covid-19

If you have dilated cardiomyopathy (DCM):

• If you feel well you don’t need to do anything differently.
• We do not yet know whether your condition places you at higher risk of harm. However, we feel structural heart disease may influence your risk. Regular handwashing with soap is extremely important. We recommend strict adherence to government guidance to reduce your chance of contracting the virus.
• If you are concerned that you may have coronavirus, please follow the national guidance, available on https://www.nhs.uk/conditions/coronavirus-covid-19/ and/or https://111.nhs.uk/service/covid-19
• If you are having new symptoms and aren’t sure if they could be related to your heart, please contact us on 07584 184602.

If you have hypertrophic cardiomyopathy (HCM):

• If you feel well you don’t need to do anything differently.
• We do not yet know whether your condition places you at higher risk of harm. However, we feel structural heart disease may influence your risk. Regular handwashing with soap is extremely important. We recommend strict adherence to government guidance to reduce your chance of contracting the virus.
• If you are concerned that you may have coronavirus, please follow the national guidance, available on https://www.nhs.uk/conditions/coronavirus-covid-19/ and/or https://111.nhs.uk/service/covid-19
• If you are having new symptoms and aren’t sure if they could be related to your heart, please contact us on 07584 184602.

If you have arrhythmogenic cardiomyopathy (or ARVC):

• If you feel well you don’t need to do anything differently.
• We do not yet know whether your condition places you at higher risk of harm. However, we feel structural heart disease may influence your risk. Regular handwashing with soap is extremely important. We recommend strict adherence to government guidance to reduce your chance of contracting the virus.
• If you are concerned that you may have coronavirus, please follow the national guidance, available on https://www.nhs.uk/conditions/coronavirus-covid-19/ and/or https://111.nhs.uk/service/covid-19
• If you are having new symptoms and aren’t sure if they could be related to your heart, please contact us on 07584 184602.

If you have Left Ventricular Noncompaction (LVNC):

• If you feel well you don’t need to do anything differently.
• We do not yet know whether your condition places you at higher risk of harm. However, we feel structural heart disease may influence your risk. Regular handwashing with soap is extremely important. We recommend strict adherence to government guidance to reduce your chance of contracting the virus.
• If you are concerned that you may have coronavirus, please follow the national guidance, available on https://www.nhs.uk/conditions/coronavirus-covid-19/ and/or https://111.nhs.uk/service/covid-19
• If you are having new symptoms and aren’t sure if they could be related to your heart, please contact us on 07584 184602.

If you have Restrictive Cardiomyopathy (RCM):

• If you feel well you don’t need to do anything differently.
• We do not yet know whether your condition places you at higher risk of harm. However, we feel structural heart disease may influence your risk. Regular handwashing with soap is extremely important. We recommend strict adherence to government guidance to reduce your chance of contracting the virus.
• If you are concerned that you may have coronavirus, please follow the national guidance, available on https://www.nhs.uk/conditions/coronavirus-covid-19/ and/or https://111.nhs.uk/service/covid-19
• If you are having new symptoms and aren’t sure if they could be related to your heart, please contact us on 07584 184602.

If you are taking an ACE inhibitor (ramipril or similar), ARB (candesartan or similar) or Entresto (sacubitril-valsartan), either for a cardiomyopathy or a pre-existing condition:

• We are aware of publicised concerns about these medications, but the evidence is unclear and stopping these might worsen your underlying condition.
• Several British and European societies have recommended continuing these medications, and we agree that you should keep taking your usual medication.

Inherited Cardiovascular Conditions (or ICCs) are a group of different genetic conditions that mainly affect the heart or main blood vessels.

There are 4 main groups of conditions that we deal with:

Cardiomyopathies – these conditions mainly affect the muscle of the heart. Examples include:

• Hypertrophic cardiomyopathy - when the heart muscle becomes too thick
• Dilated cardiomyopathy - when the heart enlarges and the muscle becomes weak
• Arrhythmogenic cardiomyopathy - when the muscle of the heart is replaced by fat and scar
• Restrictive cardiomyopathy - a quite rare condition that can cause heart failure
• Non-compaction - an abnormality of muscle development
  
  Ion channel disorders – these conditions mainly affect the electrical system of the heart. Examples include:

• Long QT syndrome – when the heart takes too long to relax
• Short QT syndrome - when the heart relaxes too quickly
• Brugada syndrome – an unusual condition with a characteristic pattern on the heart trace
• Catecholaminergic polymorphic VT – a rare condition that causes heart rhythm problems during times of stress or exercise
  
  Neuromuscular conditions – these conditions affect both the muscles of the body and the muscle or electrical system of the heart. Examples include:

• Muscular dystrophy – conditions such as Duchenne and Becker’s muscular dystrophy can cause significant weakness of the muscles of the body and can also cause heart failure
• Lamin – can cause problems with skeletal muscles, rhythm problems and heart failure
• Myotonic dystrophy – this is a condition in which the muscles of the body get tired easily and it can also affect the heart electrical system
  
  Conditions affecting the major blood vessels – these conditions affect the major blood vessels. Examples include:

• Marfan syndrome – a condition that affects many different aspects of the body including the eyes, the joints, the heart and the main artery called the aorta.
• Ehler’s Danlos syndrome – a condition that affects the skin and joints and the main artery (aorta).
• Loeys-Dietz syndrome

Our “Core Team” includes:

It is a busy clinic and we see hundreds of patients each year, but because it is a specialist clinic we are able to spend a lot longer with you than normal.

Inherited conditions are often difficult to diagnose and can be complicated to assess, so we often need to do a number of tests on your heart. We try to do as many of these tests as we can on the day of your clinic appointment so that we can provide you with the results straight away and to try and minimize the number of visits you need to make. For this reason we have designed the clinic so that when you come for your first appointment we will:

• Spend some time talking to you about your family and make a family tree
• Arrange some tests for you in the morning such as a scan of your heart, a heart trace or an exercise test
• See the consultant who will assess any symptoms you may have, talk to you about the condition that may run in your family and discuss your test results with you
• If you require any further tests, we will explain what they involve and why we are arranging them for you
• If you require any treatment this will be discussed with you and you may be given a prescription on the day
• Some patients may find it useful to see our dedicated counsellor and we can often arrange an appointment on the day of your visit as well
• Provide you with an information pack which will include contact details, patient information about the relevant heart condition, information about charities that support patients and their families, and information sheets for your relatives

To fit all this in takes some time so for your first appointment please expect to be in the hospital for about 3-4 hours. There will be plenty of time to get a coffee or some lunch so don’t panic! We believe that it is much better to spend more time on the first visit and get as many of your tests done as possible.

Sometimes it is helpful to share information about you and your test results with some of your relatives. We would never share any details about your health without your permission and if you wish to keep your results to yourself that is absolutely fine. If on the other hand you are happy to share some basic clinical information then we will ask you to sign a consent form. You can change your mind at any time however.

The role of the Inherited Cardiovascular Conditions Service is changing and expanding. National and international guidelines now recommend that individuals with, or at risk of having, an inherited cardiovascular condition should be seen in a specialist clinic at least once.

This is a new development and is supported by:

• Association for Inherited Cardiovascular Conditions (AICC) – the national body for inherited cardiovascular conditions
• NHS England
• European Heart Rhythm Association and (US) Heart Rhythm Society consensus guidelines

Not all patients will need to keep coming back to the clinic and many with stable or mild disease can be seen in secondary and tertiary care.

There are several ways in which patients are referred to the Inherited Cardiovascular Conditions Service:

• GP referral – any patient with, or at risk of having, an inherited cardiovascular condition who isn’t already being seen should be referred.
• Hospital referrals – any patient who has a suspected or confirmed diagnosis can be referred. Reasons for referral might include diagnostic confirmation, exclusion of phenocopies (conditions that mimic a genetic condition), genetic testing, risk stratification, management advice, long term follow up, family screening.

For clinical questions in patients over 16 years of age:

Contact the Clinical Nurse Specialists on 07584 184602

For appointments

0113 3926476

For questions relating to genetic testing:

Contact Kath Ashcroft on 01133924436 or kathashcroft@nhs.net

Paediatric Liaison Nurses - 0113 3925467

We have produced the following information sheets:

Information Booklet for Patients and their Families 

Information Booklet for Patients and Their Families Transitioning from Children's to Adult Services 

We also work closely with several charitable organizations that provide information, support and advice, support research and raise funds.

Sudden Adult Death Trust - SADS UK

Cardiomyopathy UK - Cardiomyopathy UK

Cardiac Risk in the Young - CRY