If you have a referral to a clinic at Royal Brompton Hospital, this could be because:
- a family member has an inherited heart condition
- a family member has died suddenly from a suspected or confirmed heart condition that may be inherited
- you have experienced symptoms which need further investigation.
What are ion channelopathies?
Ion channelopathies are a group of rare genetic conditions. This is caused by a genetic alteration (variant), which affects ion channels in the heart.
These channels are on each individual cell wall within the heart muscle. They provide a route into and out of cells, via the cell wall, of chemical substances (ions). This enables electrical impulses to pass from cell to cell, resulting in a normal heartbeat.
Sodium and potassium channels are particularly important in the heart. Disruption to the movement of ions can cause an abnormal heartbeat.
Changes in the function of these channels result in too few or too many ions entering the cell. This has the ability to change the way the electrical current moves through the heart.
Changes in the electrical current within the heart can cause an irregularity in the way the heart beats causing:
- palpitations
- dizziness
- loss of consciousness (fainting)
- in some cases causes sudden death.
Ion channelopathies can affect children and adults. People affected by an ion channelopathy usually have a structurally and functionally normal heart. But for a small number of affected people, they find structural abnormalities.
Ion channelopathy at the Trust
At the Trust, we investigate the most common ion channelopathies. But there are other rare ion channelopathies which may need investigations. We investigate:
- long QT syndrome
- Brugada syndrome
- catecholaminergic polymorphic ventricular tachycardia (CPVT)
- short QT syndrome.
If a family member has a sudden and unexplained cardiac death, we will investigate and look for both ion channelopathies and cardiomyopathies.
The clinics
On the day of your appointment, come to the main hospital reception and we will direct you to your clinic.
Our children's clinics have toys, colouring pencils and a TV, but you might also want to bring a toy or game for your child.
Royal Brompton Hospital
ICC family follow up clinic (paediatric and adult patients)
Time: Every Wednesday Morning
Consultant: Dr Jan Till
Location: Outpatients West
Nurse-led transition clinic
Time: Every Wednesday - 11am to 3pm
With: Clinical nurse specialists
Location: Fulham Rd outpatients on mezzanine level
ICC family day-case service (paediatric and adult new patients)
Time: Every Thursday
Consultant: Dr Jan Till
Location: Fulham Rd outpatients on mezzanine level
Before your appointment
One of our team of clinical nurse specialists (CNSs) will aim to contact you before your appointment. They will want to know more about your family history to draw a family tree for your appointment.
Your family tree will include information about the health of your immediate family (children, parents, brothers and sisters) and extended family (nieces, nephews, grandparents and as many other family members you can give us information about).
This information helps us identify any previous symptoms or deaths which may be linked, and which could be the result of an inherited condition.
We will look for information about adult sudden deaths, miscarriages and sudden infant or childhood deaths in your family history. We will ask about the cause of death, if you know it, and a copy of the post-mortem if you have one.
You should also include information about any symptoms such as palpitations (rapid or irregular heart beat), seizures (epilepsy) and fainting.
What will happen at the clinic?
At your initial appointment at the day case clinic, we check for inherited conditions that can affect the electrical activity in your heart. If we find a heart condition, it is likely that we can give you treatment to reduce or take away the risk to you.
We can also reassure members of your family who are not at immediate risk. We will try to answer all your questions, offer advice and possible treatment, but this is not always the case.
Getting a positive or unclear test result can be very worrying. A positive test result may confirm that you have inherited a heart condition, in which case we will discuss treatment options with you. An unclear test may show that you need more tests. If this is the case, we discuss this with you and arrange them for you as well.
At your appointment, you will have a series of tests, followed by meetings with members of our team. The way we plan the appointment means we can do everything we need in the one day. We will try to do this as quickly as we can, but you may be with us us until 5.30pm.
What should I bring?
When you come to your appointment you should bring:
- an up-to-date list of all your medication
- any medication that you may need during the day
- a pair of trainers if you are having an exercise test
- any extra information you have learned about your family history
- something to keep you occupied, like a book or tablet to watch TV on as there will be time between tests and meetings.
We have a limited amount of toys, so please bring some fun things to do for any children attending the clinic.
Tests
You will have several cardiac tests during your appointment. We try to arrange these for the morning, but you may have some of them in the early afternoon. Your consultants will give you the results of your tests in the afternoon during your consultation.
The tests you may have include:
- electrocardiogram (ECG)
- echocardiogram (echo)
- exercise stress test
- 24-hour holter monitoring
- blood tests.
Results
Once you have had all the tests you will meet with a consultant.
If we diagnose an inherited heart condition, we will agree your treatment plan. We base this on the results of your tests, your medical history and the effect the condition is having on your daily life.
This could include medication or lifestyle changes, such as doing gentle rather than strenuous exercise. If we find out that you have an inherited condition, we can screen other relatives, such as children, brothers and sisters.
If we don’t find a condition, we may keep a check on you in our outpatient clinic until we are sure there is no inherited condition affecting you.
About the channelopathy team
Consultants
Jan Till, consultant paediatric electrophysiologist
Dr James Ware, senior lecturer and honorary consultant
Dr Leonie Wong, paediatric electrophysiology consultant
Specialist nurses (Royal Brompton Hospital)
Catherine Renwick, lead nurse for cardiomyopathy, aortopathy and channelopathy
Bethan Cowley, adult ICC clinical nurse specialist
Fortunate Rusike, adult ICC clinical nurse specialist (aortopathy and Marfan)
Charlene Casey, paediatric clinical nurse specialist
Lucy Green, paediatric clinical nurse specialist
Emma Ashby, ICC nurse
Louise Parker, ICC nurse
Rachel Mackay, adult ICC clinical nurse specialist (on maternity leave)
Suad Warsama, adult ICC clinical nurse specialist (high risk pregnancy)
Contact
If you have any questions or concerns before or after your appointment you should contact the clinical nurse specialist (CNS) team.
020 7351 8121, ext 2205 (paediatric)
Further information
Credible Meds - details of drugs that should be avoided in Long QT syndrome
Arrhythmia Alliance - promoting effective diagnosis and treatment of arrhythmias
Brugada Drugs - details of drugs that should be avoided in people with Brugada
SADs UK - providing information, funding research and medical equipment to prevent premature sudden cardiac death
British Heart Foundation - supporting those suffering from heart conditions
Heart Research - information and advice for young people growing up with a heart condition
Children’s Heart Federation - a children’s heart charity dedicated to helping children with congenital or acquired heart disease and their families in Great Britain and Northern Ireland
How to refer a patient to the cardiomyopathy and channelopathy clinics
The Trust runs a comprehensive inherited cardiovascular conditions (ICC) service. These services cover adults, children and even fetal life. Find out more.