Electrophysiology & Inherited Cardiovascular Conditions

Electrophysiology & Inherited Cardiovascular Conditions

The service is lead by Dr Michael Bowes. Dr Bowes is a consultant paediatric electrophysiologist. He was trained in general paediatrics in the North West before specialising in paediatric cardiology at Alder Hey Children’s hospital in Liverpool. Subsequently, Dr Bowes acquired further specialist training in Electrophysiology at Liverpool Heart and Chest hospital and the Royal Children’s Hospital, Melbourne.

Dr Bowes' areas of expertise include childhood arrhythmia, invasive electrophysiology, implantable devices, channelopathies and inherited cardiac condition

Dr Bowes works closely with adult colleagues in both Liverpool Heart and Chest Hospital and Manchester Heart Centre to help provide a joined-up, whole family service for those impacted by inherited cardiac conditions such as long QT syndrome, Brugada syndrome and CPVT.

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Cardiac Electrophysiology (EP)

Cardiac electrophysiology deals with the assessment and treatment of heart rhythm problems. Disorders of the heart rhythm can cause:

  • Palpitations
  • Blackouts
  • Symptoms of ‘heart failure’

 

Catheter Ablation

Many cardiac conditions which cause heart rhythm problems can now be diagnosed and treated with a procedure called Catheter Ablation.   We are able to offer a cure to many patients who previously had to take long-term drug therapy, which often caused side effects.

Ablation procedures are usually carried out as day-case procedures.

They are relatively painless and take approximately two to three hours. Following induction of general anaesthetic, fine recording electrodes are passed into the heart from a vein at the top of the leg. The electrical activity of the heart is recorded and the source of the heart rhythm problem identified. A special catheter is then used to gently heat up the inside wall of the heart at that site. Using this technique, up to 90% of patients can be cured of their heart rhythm problem.

 

Pacemakers

Pacemakers are implanted to prevent slow heart rates known as bradycardias. More complex pacemakers are available to treat patients with heart failure who remain symptomatic despite full medical therapy. For those patients thought to be at risk of sudden cardiac death we implant cardiac defibrillators which can return a life-threatening heart rhythm to normal. 

A full range of diagnostic facilities are available at Alder Hey to ensure appropriate individualised prescription of devices and their settings. Follow up clinics run weekly, with access to investigations, expert pacing physiologists and a paediatric electrophysiology consultant all in the same visit.

 

ICC

Inherited Cardiovascular Conditions (ICC) are a group of genetic disorders that primarily affect the heart. They includes conditions such as cardiomyopathy, inherited electrical conditions called channelopathies and problems with the main blood vessel carrying blood from the heart, the aorta.

Some heart conditions can have no symptoms, but could still potentially be serious. For some families, the first sign of a problem is when someone dies suddenly with no obvious cause or explanation.

It is important that patients with ICC disorders and their relatives are identified and seen by a cardiologist (heart doctor) with expertise in these conditions.  

 

What causes an inherited heart condition?

Our DNA makes each of us unique. The genes within DNA are our code and give the body instructions on how to work. We inherit DNA from our parents.

Inherited heart conditions are caused by a fault (or mutation) in one of more of our genes. If a parent has a faulty gene, there’s a 50:50 chance it could pass on to each of their children. Most inherited cardiac conditions follow this pattern; there are rare exceptions when a particular faulty gene can only be passed on by a mother.

It’s possible to have a faulty gene that can lead to a heart condition, yet not have any clear signs of symptoms of the condition itself, and this is especially true in children and young adults, where repeat investigations over the course of childhood are often required. 

 

What inherited heart conditions are there?

The most common inherited heart conditions are:

Cardiomyopathies, for example:

  • Hypertrophic cardiomyopathy
  • Dilated cardiomyopathy
  • Arrhythmogenic (right) ventricular cardiomyopathy
  • Non compaction cardiomyopathy
  • Restrictive cardiomyopathy

Inherited heart rhythm disturbances, for example:

  • Long QT syndrome (LQTS)
  • Brugada syndrome
  • Catecholaminergic polymorphic ventricular tachycardia (CPVT)
  • Progressive cardiac conduction defect (PCCD)

 

What treatments may be required?

Many patients with an ICC will not require specific treatment. Others will be prescribed medications to help treat symptoms and reduce risk.

One of the main reasons for review in the ICC clinics is to look closely at the heart and assess the risk of sudden cardiac death. In most cases this risk is low and our patients can be reassured. In a small proportion of patients an increased risk of sudden cardiac death will be identified. These patients may require a special type of pacemaker that can correct life threatening abnormalities of the electricity in the heart – an Implantable Cardioverter Defibrillator (ICD).

Further information on ICDs 

See web links

 www.qtdrugs.org

www.brugadadrugs.org

 


Alder Hey Children's Charity
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