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23 Jun

imageOne early morning in February 3 years ago the phone rang and it was the wife of our Best man at our wedding. She was letting us know that she had woken in the night to a strange sound and following attempts at resuscitation by herself and the ambulance crew he had died suddenly. He was a handsome,highly intelligent,charming doctor/dentist/medical director of a pharmoceutical company who had been negotiating with NICE to accept Aricept as a treatment for Dementia.He had also been training for his 7th full marathon which he had taken part in and raised thousands of pounds for leukaemia following the death of his son’s best friend at the age of five.It was unbelievable and a total shock to all such a loss of someone so special. The night before he had been listening to opera with friends singing along with gusto and passion as he was also a musician, a pianist with talent.The funeral was an amazing musical tribute and those who spoke talked about his life with humour and sadness. Afterwards, his friends and colleagues commented that he had seemed the healthiest of everybody and as we chatted agreed it would have been a great party but it lacked one person. He was an ordinary East End lad made good commonly known as ‘Paul boy’! His only fault was that he was a Tottenham supporter which meant many an Saturday afternoon or a Boxing Day was spent in raucous banter whilst ‘the lads’ watched their rivals play – nothing more exciting than a London Derby!
This was an adult sudden cardiac death (SCD)as there was no evidence of disease on examination of his body after death and it was presumed death was due to sudden cardiac arrest,when the heart abruptly and without warning stops pumping.
Ironically, the week after he died the drug was passed!

I was reminded of this when a young attractive 25year old lady attended surgery recently devastated as she had lost her young partner in the same way. This young man had died suddenly without warning and his young partner and family had been left devastated.
She then told me that his mother had become very keen to support the charity CRY was founded in 1995 to raise awareness of conditions that can lead to young sudden cardiac death (YSCD); sudden death syndrome (SDS); SADS. She was also taking part in the CRY Heart of London Bridges Walk 2013 on 7th July. There main reason for supporting this charity is that it funds screening of anyone who could considered at risk as shown on the link. What a brave lady, the love she had for this man was tangible and this family had also lost someone very special whose life had just begun. The family are keen to support this charity as it raises money to screen anyone at risk and support research projects aswell as support bereft families.

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http://www.c-r-y.org.uk/general_information_on_cardiac_s.htm

CRY holds ECG screening clinics for those aged 14 to 35 at a number of established locations around the UK including Belfast, Cardiff, Colchester and London.The CRY mobile screening unit facilitates various screening events at other locations in the UK – often organised by CRY supporters. Details of where these clinics are and how to book are on the website.

What happens when sudden cardiac death(SCD) occurs
When SCD occurs, no blood can be pumped to the rest of the body. It is responsible for half of all heart disease deaths.
Sudden cardiac death occurs when the heart’s electrical system malfunctions. It is not a heart attack (also known as a myocardial infarcation). A heart attack is when a blockage in a blood vessel interrupts the flow of oxygen-rich blood to the heart, causing heart muscle to die. So if the heart can be compared to a house, SCD occurs when there is an electrical problem and a heart attack when there is a plumbing problem.
The most common cause of cardiac arrest is a heart rhythm disorder or arrhythmia called ventricular fibrillation (VF). The heart has a built-in electrical system. In a healthy heart, the sinoatrial node, the heart’s natural “pacemaker” triggers the heartbeat, then electrical impulses run along pathways in the heart, causing it to contract in a regular,rhythmic way. When a contraction happens, blood is pumped.
But in ventricular fibrillation, the electrical signals that control the pumping of the heart suddenly become rapid and chaotic. As a result, the lower chambers of the heart, the ventricles, quiver or fibrillate instead of contracting, and they can no longer pump blood from the heart to the rest of the body. If blood cannot flow to the brain, the brain becomes starved of oxygen, and the person loses consciousness in seconds. Unless an emergency shock is delivered to the heart to restore its regular rhythm using a machine called a defibrillator, death occurs within minutes. It’s estimated that more than 70 percent of ventricular fibrillation victims die before reaching the hospital.
When CPR and an AED (automated external defibrillator) are used together, the chance of survival following a cardiac arrest goes up to 50%, a ten-fold increase over CPR alone.

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This is why you may see Automated External Defibrillator (AED) machines are being hung on walls in supermarkets or other public places. I have frequently seen them in French supermarkets and hyper stores.
It is amazing that the nearest limited access AED is in Twickenham in a fitness club and the nearest 24 hr public access AED machine is outside an accountants office. To see the location of AED machines click on the link below:-
http://www.aedlocator.org/AEDLocations.php
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Who should be screened?
It is recommended that screening is requested via your GP if there have been any young sudden deaths in the family. Or if there are symptoms of:

Chest Pain (exercise related)
Severe Breathlessness
Palpitations
Prolonged Dizziness
Fainting/Blackouts

This checklist has been designed to help you determine whether you have a heart rhythm problem. If you have more than one of the symptoms below, see your GP.

    • Have you fainted or passed out during exercise, while emotional or when startled?
    • Have you ever fainted or passed out after exercise?
    • Have you ever had extreme shortness of breath during exercise?
    • Have you ever had extreme fatigue associated with exercise (much more so than others of your age and level of fitness)?
    • Have you ever had discomfort, pain or pressure in your chest during exercise?
    • Has a doctor ever ordered a test for your heart?
    • Have you ever been diagnosed with an unexplained seizure or fit?
    • Have you been diagnosed with epilepsy that fails to respond to medication?
    • Have you ever had exercise-induced asthma that medication didn’t control well?
    • Are there any family members who had a sudden, unexpected, unexplained death before age 50 (including cot death, car accident or drowning)?
    • Are there any family members who died suddenly of heart problems before they were 50?
    • Are there any family members who have had unexplained fainting or seizures?
      Do you have any relatives with the following conditions:
      Hypertrophic cardiomyopathy: thickening of the heart muscles.
      Long QT syndrome: a condition that results in a very fast, abnormal heart rhythm, which can cause fainting.

What is happening by way of research?

VeniceArrhythmias, a biannual meeting started in 1989 and, year after year, has become one of the most important international congresses in the field of arrhythmology with almost 3000 attendees and with more than 400 invited speakers.
As you read many cardiologists and electrophysiologists are preparing their abstracts to be presented at the Venicearrythmia conference next held in October 2013
VeniceArrhythmias, a biannual meeting started in 1989 and, year after year, has become one of the most important international congresses in the field of arrhythmology with almost 3000 attendees and with more than 400 invited speakers.image
At other venues and during the year there will be similar events. It is through these events that there have been major breakthroughs in screening and prevention of sudden death.Research in the channelopathies and cardiomyopathies is progressing rapidly and in the future it is expected that the majority of the genes involved will be discovered.
In the future, it may also be possible to diagnose all carriers easily – even in those people who have a normal ECG reading. It may also be possible to choose the best treatment based on the type of mutations involved, and the treatment may even be designed based on this knowledge.
In the meantime, better understanding of these conditions and improvements in methods for diagnosis should still result in better management. It is crucial that, when a heart disease such as a channelopathy or cardiomyopathy is diagnosed, all immediate blood relatives should be evaluated by a specialist cardiologist to find out if they have an inherited heart disease.

IMPORTANT
It needs to be considered in sudden death in especially younger adults where the post Mortem fails to provide a cause of death, it is important to send the heart to a pathologist who specialises in this field of medicine and this may be a relative, friend or attending doctor who makes that request.
However, to help this research and appreciate the true incidence there must be a change in the Law.
In the UK, unexplained sudden death is frequently recorded as due to death from natural causes. Until the law is changed and coroners have to refer hearts on to specialists we will not know the true figures. CRY’s fast track coroner / pathology service enables the cause of death in a sudden death case to be established more quickly and accurately than might otherwise happen if left to a local coroner lacking expertise in cardiac pathology.

Tests That Predict Risk

There are a number of tests that can be performed to determine if some- one is in a group that is at high risk for cardiac arrest. These include:
Echocardiogram – a painless test in which ultrasound waves are used to create a moving picture of the heart. The test can measure the strength of the heart’s pumping function (ejection fraction) and identify other problems that may increase a person’s risk for SCD.

Electrocardiogram – A painless test in which electrodes are attached to the patient’s chest to record the electrical activity of the heart in order to identify abnormal heart rhythms. Certain arrhythmias could point to an increased risk of SCD.imageHolter monitor – A cell phone-size recorder that patients attach to their chest for one to two days, recording a longer sampling of their heart rhythm. After the recorder is removed, the tape is analyzed for signs of arrhythmia.
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Event recorder – a pager-sized de- vice that also records the electrical activity of the heart over a longer period of time. Unlike a holter moni- tor, it does not operate continuously. Instead, patients activate the de- vice whenever they feel their heart beating too quickly or chaotically.
Electrophysiology study (EPS) – This test is performed in a hospital. A local anesthetic is used to numb areas in the groin or neck and thin flexible wires called catheters are
advanced through veins into the heart under x-ray to record its electrical signals. During the study, the electrophysiologist studies the speed and flow of electrical sig- nals through the heart and paces the heart to see if arrhythmias can be induced. The physician can also determine if a patient has had a prior heart attack or evidence of prior heart damage without know- ing it. All of this information can help determine whether the patient is in a group at higher risk for SCD.There is another interesting device
The AliveCor Heart Monitor app is designed for medical professionals, patients and health conscious individuals to record, display, store and transfer accurate single-channel electrocardiogram (ECG) rhythms. These recordings could be saved on the mobile phone or shared with others (such as your doctor) via email. The app is CE-mark approved.
imageThe Heart Monitor snaps onto your iPhone 4 or 4S like a case and wirelessly communicates with the app on your phone. No pairing between your iPhone and the Heart Monitor is required. The free AliveECG app will be available for download from the Apple App Store when you receive your Heart Monitor. Once in the app, create a free account and you’re ready to begin recording ECGs. It’s that easy to get started. http://www.AliveCor.com provides you with anytime, anywhere, fully secure, online access to all of your ECGs. Once you have the Heart Monitor and the AliveECG app you can create an account that gives you access to your ECG data.

Prevention

There are a number of things people can do to decrease the likelihood of becoming a victim of sudden cardiac death. To begin with, living a “heart healthy” life can help reduce the chances of dying of cardiac arrest or other heart conditions. This includes ex- ercising regularly, eating healthful foods, maintaining a reasonable weight, and avoiding smoking.
Treating and monitoring diseases and conditions that can contribute to heart problems, including high blood pressure, high cholesterol, and diabetes, is also important.
Finally, for some patients, preventing sudden cardiac death means controlling or stopping the abnormal heart rhythms that may trigger ventricular fibrillation.

Treating arrhythmias is done in three ways:

Medications – Medications, includ- ing ACE inhibitors, beta blockers, calcium channel blockers, and antiarrhythmics, can control abnormal heart rhythms or treat other conditions that may contribute to heart disease or SCD. But taking medication alone has not proved to be very effective in reducing cardiac arrest. These medications are sometimes taken by patients who also have an ICD, in order to reduce how often it fires.

Implantable cardioverter defibrillators (ICDs) – These devices have been very successful in preventing sudden cardiac death in high-risk patients. Like a pacemaker, ICDs are implanted under the skin. Wires called leads run from the ICD to the heart, and the device monitors the heart to detect any abnormal rhythms. If a dangerous arrhythmia is detected, the ICD delivers an electrical shock to re- store the heart’s normal rhythm and prevent sudden cardiac death. The ICD can also act like a pacemaker if the heart is beating too slowly.

Catheter Ablation – In this technique, radiofrequency energy (heat), cryotherapy (freezing), or other energy forms are used to destroy small areas of heart muscle that cause the dangerous, rapid heart rhythms. The energy is delivered through catheters that are positioned through the veins or arteries to the heart. Catheter ablation is sometimes done in patients who have an ICD to decrease the frequency arrhythmias the number of ICD shocks.
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If anyone has any particular concerns Dr Livingston has a special interest in this condition and is very knowledgable and would be pleased to discuss any related problem.

Posted by Dr Bayer

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