Rob Jackson

Living with arrhythmogenic right ventricular cardiomyopathy (ARVC)

I am a 31 year old Sergeant in the Army Air Corps and until recently was a Lynx Helicopter pilot and aircraft commander operating in Northern Ireland. I joined the Army at 18 and have spent the past 12 years living an active, healthy lifestyle as a soldier.

I passed the Army Pilots Course in 2001 and have enjoyed the challenge of flying the Lynx MK7 and MK9 Helicopter. I have served in Germany, Bosnia, Canada, Poland, Cyprus and Northern Ireland.

I am a keen sportsman and have represented my Corps at rugby and windsurfing. I cycled approx 10 miles a day to and from work and considered myself as one of the fittest amongst my work colleagues.

It was Saturday 26th September 2006. I’d been out for a run with my dog Amber in the morning and was heading out for a few beers with the boys that evening. On our return from the club in the car, I found myself sweating and out of breath, it was about 2am. I got home and remember my partner giving me grief for the state I was in. I could feel my heart racing and decided to call an ambulance as I was struggling to remain conscious.

The paramedics arrived in what seemed like minutes and diagnosed me as being in VT (ventricular tachycardia), with a sustained heart rate of 240 bpm. I was rushed to Antrim hospital and was defibrillated back into normal sinus rhythm.

I spent a week in hospital connected to an ECG and showed no further symptoms. I then had an ECHO scan on my heart and exercise stress test on a tread mill and still showed no symptoms of VT. My consultant, Dr Tom Trouton, discharged me with suspected arrhythmogenic right ventricular cardiomyopathy (ARVC) and I was to return at a later date for an angiogram and EP studies. These were both carried out and I was still un-diagnosed. The next step was for me to have a cardiac MRI scan which I would have to wait for 4 months. Luckily the Army paid for my MRI privately at St Georges, which confirmed that I had ARVC.

I was shocked, I considered myself as a healthy and fit guy, with a promising future as a pilot and until that night in September, I’d had no symptoms at all.

On 19th January 2007 I was admitted to the Royal Victoria hospital in Belfast to have my implantable cardioverter defibrillator (ICD) fitted, which will protect me from abnormally fast heart rhythms. I was discharged the same day and am currently on 2 weeks sick leave to recover.

I am slowly coming to terms with the fact I’ll never fly again, or do all the things I’ve enjoyed as a soldier. I am lucky though, I very nearly didn’t make it. I’ve had a lot of help from my friends and family and the Army have re-employed me as an instructor.

I’m already planning a fund raising sponsored walk for the summer to help support CRY. I think their national screening programme is invaluable as most people with my condition are diagnosed when its too late.

Barry Hooton

Living with an implantable cardioverter defibrillator (ICD) and an undiagnosed condition

This story comes from two people – me, Anna (aged 17), a.k.a. “the girlfriend” and Barry (aged 23), a.k.a. “the mystery.” Its a long story, but perhaps may give hope to those who are also undiagnosed just like Barry.

This story begins on June 1st, 2007 – it was a weird day. For some reason I (Anna) was staying home from College and my mum was staying home from work.

This didn’t stop my boyfriend Barry from going to work at Pizza Hut. At the time I was playing our new Playstation 3 – Need for Speed Carbon, a racing game – when he said at about 12pm that it was time for him to go to work. We exchanged our usual hug and kiss and told each other that we loved the other. He walked out of the room, I heard his car pull out and he went to work.

The Call
At about 5pm, I looked at my watch for no reason and thought to myself, “He’ll be home in about 3 hours!” (his shift was 12-8pm). This meant I could talk with him for a while and cuddle before it was 10 o’clock, which was usually lights out – but this was a Friday, so it didn’t really matter. About 5-10 minutes after looking at my watch, my mum is running up the stairs with the phone in her hand. She’s looking desperately at me and passes it over saying, “She’s right here.” I casually said, “Hello” thinking that it might be my College tutor calling me.

Instead I was greeted with, “Hello, this is so-and-so from Walsgrave Hospital. I’m calling you about Barry Hooton.” I didn’t know what to think, I thought it may just have been a burn or something – what could possibly go wrong at Pizza Hut, right? And it couldn’t have been a crash in his car because he’s been working there since 12. Then I began to become confused as the nurse said to me, “You see, he’s ‘very poorly’. Do you have his parents number?” I said yes, and I gave her their number as I was put as his next of kin. Then she began to say, “Yes, he’s in ‘critical condition’.” This was when I stopped her and said, “Okay, is he ‘very poorly’ or in ‘critical condition’.” To me, there was a massive gap. It was confirmed he was in ‘critical’. So then my mum was frantic, just knowing she had to get me and herself to the hospital – we jumped in the car and drove.

On the Way out the Door
The next mode of action was I called his brother Paul to let him know, because I didn’t know if he had been told yet. His parents were already being called so I knew I didn’t have to worry about that. Still, we had no idea what had happened yet – they can’t tell you anything over the phone. So, on our way to the hospital I call Pizza Hut to try and find out what had happened. To my surprise the ambulance crew told them not to call me. The Area Manager had ironically just popped in to see how things were doing and he picked up the phone. He walked in as all of this just happened with Barry, and I was told that Barry had collapsed in the kitchen for no reason, while cleaning pots for the night shift to help out.

However, he had stopped breathing and his heart had stopped, and Caroline – one of the Managers – had given him CPR before the ambulance took him away. Later, we found out that Caroline was giving him CPR for about 4-5 minutes before the Ambulance came, and after that, it was another 20 minutes before they resuscitated him. In all, he was technically dead for 24-25 minutes. He had to be shocked 6 times before he returned to a semi-stable state.

A&E
Finally, we arrived at the hospital. I went into A&E with my mum, and had to wait in one of those awful off-side cubicles where they put people who are crying so that they can control the atmosphere. It was the longest wait ever; “the doctors are still working on him” they told me. Eventually, they had finished and I was able to go see him. To this day I will never forget what he looked like. I wanted to burst into tears so many times but held it back. He was hooked up to the works – ventilator, all sorts of tubes of different vitamins and sedatives going into him.

He was basically put into a medical coma to stabilise his body. The worst thing was, his eyes weren’t entirely shut, so his eyes were just there with water coming out of them from the air of the hospital – it looked like he was crying. I saw a bit of blood on his arm from I don’t know what and just immediately felt ill. I tried to talk to him like normal, in case he could hear me, but when someone is in that state and can’t respond, you can’t help but talk about them in 3rd person. I still had to wait for his parents, who were going to arrive at about 9pm because they live near Skegness while we were in Coventry Hospital!

Critical Care
Soon he was moved to the much quieter critical care area of Walsgrave Hospital. By now, he was cleaned up and just looked like he was asleep. They kept his body temperature lower than normal because it was therapeutic to the body. However, this made it no easier for myself to touch him because, as most people know, a body goes cold when it dies so every time I felt him it felt like I was touching a corpse. The feeling was awful. I talked to him now, just like he was awake, but the hardest moment came when he began to lurch. If you’ve ever seen the movie ‘The Exorcist’ and have seen the bit where the girl springs to an upright position, this is what happened with him at first. It was terrifying, because you didn’t know if he was in pain or anything and you just hoped that everything inside of him was OK and mentally he was OK – if he was conscious at all. Every lurch I wanted to just burst into tears because it felt like you were getting a reaction from something that technically wasn’t supposed to be able to move at all on its own. Later that night they gave him a full muscle relaxant which stopped this, and although he was away in sleepy bliss, none of the rest of us were. While he was asleep like this, I found it so hard to have much contact with his body. It terrified me. I knew it was him, but it was terrifying. I couldn’t at this point even get the courage to kiss his forehead.

There were two nurses who really stick in my mind, because they were simply brilliant. Their names were Lisa, a brilliant Irish nurse, and Ray, who reminds me of our friend Nicky. They were so brilliant and happy. I remember the first thing that Lisa said to me, “Oh, you must be Anna. I’m Lisa – I’ll be looking after your man today.” Ray also took very good care of Barry, always giving him a thumbs-up and always talking, explaining, and generally looking after him. Barry doesn’t remember meeting Ray, to this day.

“Waking Up”
All of Saturday he was asleep and the doctors on Friday had told us that they would start to wake him up. When someone says “wake up” you imagine yourself in the morning just… waking up! However, with this, they had to first very slowly raise his temperature to normal, then very slowly take him off the sedatives. Also, when he was first waking up, things were very hard. He was looking around, but the doctors had said that he hadn’t talked yet. I went in to see him, he had no energy. He could barely turn his head. The bed wasn’t big enough for him – he’s 6’4″ – and so he was semi-curled up on the bed, looking like a child. His eyes showed that he was terrified.

I talked to him, he would slightly nod, or slightly smile, but his body was shivering and shaking all over. I assume this was just the shock that was postponed. It was the sort of shaking your body starts to get when you feel like you’re going to be sick and you can’t help but quiver with the nervousness of it. I told him I was going to get some lunch and that I loved him, leant down and gave him a kiss on the lips. His first words, ever, after this whole thing were, “I love you too.” This made me jump inside. I was so happy. Not only that he remembered me, but that he was showing instant signs of progress – he could talk. I immediately told the doctor.

The next thing that he proceeded to say to me was when he was looking at me weird and I said to him: “Do you remember who I am?” He then pulled me down to his lips and said quite simply, “How can I forget you? You’re Anna, the love of my life.” I could have broken down and cried a million tears at this point. It was like something from a romance movie or novel. There are fairy-tale moments in real life, and this was the proof. However, at this point, he could still barely talk. When he did, it took everything he had in him. He also still could barely move. He was quite “disabled.”

The next thing was the worry. Within the next few days (Monday to Wednesday), every day or even hour just got better and better – more movement, more talking, more awareness, and more insanity! He began to remember things, but was also starting to hallucinate about things as well – this was the effect of the “comedown” of the sedatives. He thought he was living with his parents, going to college, even doing work experience at the hospital! All of the facts were there, but they weren’t being pieced together properly. I think the best instance to explain how someone is coming out of sedatives is this: Barry said to me, “The nurses locked me in the toilet.” I couldn’t believe what I was hearing. However, it was almost true. The nurses had walked him to the toilet because he didn’t like the catheter, and tried to let him do things on his own. When they tried to open the door after he’d finished, the door handle had come off! So he was technically stuck in there but they soon got him out.

However, this was another great step – he hadn’t forgotten how to walk, either. Another thing he seemed to not forget was the ability to become sexually aroused. His father was sat in critical care with me one time and Barry just decided he was going to show me how passionately he cared for me! He suddenly lunged at me from the side of his bed and tried to snog me, right in front of his dad, and the nurses! No matter who was in there, my mum or dad, or his family, he didn’t care. Eventually, it got quite out of hand, and the nurse said to him; “Now, Barry, we think this whole thing might be a problem with your heart so you can’t get frisky!”

It’s Something to do with the Heart
By Thursday, he was moved to the cardiac ward. He was, again, the youngest person just like he was in Critical. It wasn’t long until the doctors moved him into a side room. He was much more like himself now, and starting to remember things even more. When he was in Critical he had the memory of a goldfish. Things were getting better. Soon, they were talking about moving him to Queen Elizabeth Hospital, Birmingham. They had never seen this sort of record recovery and still to this day he has no brain damage of any sort except for when he was in critical care he cannot remember being there, or perhaps the day he was working.

The Taxi Ride
Waiting for a bed in Birmingham took about a week or so, and then we suddenly were told to get packing because the ambulance was here to take us to Birmingham. I rode with Barry, naturally. The “ambulance” was actually a taxi.  However, the driver doubles as a fully qualified paramedic and has all of the tools and kits in the boot if anything went a bit crazy. There are only 15 of them who operate in the area and I say thanks to them because the one we had was brilliant – his name was Bill. We soon got Barry settled into the new ward at Birmingham. Funnily enough, the first nurse we met was called “Rommie” which was funny because ‘Andromeda’ is one of Barry’s favourite shows and the ship in its human form is called “Rommie” for short.

Birmingham
We arrived in Birmingham on Saturday the 16th of June. Birmingham was very different to Walsgrave Hospital. Walsgrave was pristine in condition because it had just been rebuilt and had all new equipment – Birmingham was waiting for this makeover. Things seemed older and less inviting, but the staff were brilliant, and the people on the ward as well. The men on the ward were so nice and just had a laugh as often as possible, with everyone. This was more when Barry remembers things. He doesn’t remember any of critical care and a little of the cardiac ward. Barry says the best thing about this ward was the older men were more than willing to help him through this tough time. Barry was going to have an implantable cardioverter defibrillator (ICD) implanted into his body, and one of the men, Ron, already had one of these, so Barry got to converse with him about what it was like.

Surgery
Soon, Barry was in surgery to have an ICD implanted. He was scared about the whole thing, as anyone would be, and so were we – but soon it was in and settled. The surgery had no problems and it was very funny because to this day, if you met Barry you would think that nothing like this had happened to him. He is exactly the same as he was before, with the addition of an ICD.

Laugh or Cry
One of the biggest things about dilemmas like this is whether you laugh or cry. At times, it was both. Here are some things that we look back on now, and can just smile about: we used to joke about Caroline giving Barry CPR – but now, we’re so thankful for it. We even would joke about Barry having a heart attack because of a tingling he gets in his hands sometimes. Now, we realize, its nothing to joke about.

Another thing that was a big laugh / worry was whether or not our love-life would be able to resume. The doctors said it could, and at first it was very very peculiar. At first, because Barry had had an angiogram, we had to put a pillow on his groin/leg area before we started anything. It was quite funny, and we just did it in increments. You can only go as fast as what you’re comfortable with. It was a funny experience, but very weird. We still both feel weird about it today. After not being around each other 24/7 like we normally were for about a month, and then suddenly going back to it, its very weird.

My best advice for people is give yourself time to adjust. A month is when all the heavy stuff comes out like the crying, the emotions, and everything else. After that, things slowly get better. Although you will always know that something is different, don’t let it rule your life. The worst thing for anyone who has had something like this, is not being able to drive. Barry can’t drive for 6 months, and if in that time he gets a shock from his ICD then that timer goes up and up. He hopes not to get a shock.

The Mystery – Still Unsolved
To this day, the doctors have no idea what happened or why Barry collapsed so suddenly. They’ve done an MRI scan, ECGs, an angiogram- everything, and cannot find a single cause or reason as to what happened, why, or what it was that actually happened. He still remains a mystery. The only thing that people can come to the conclusion of, seeing him from literally dead, and now where he’s exactly normal like he was before, is that he’s a walking miracle. I like to think that too.

Joseph Tanner

Living with Brugada syndrome (type 3)

Sunday 16th March 2008, I had a cardiac arrest and died for 7 minutes. I collapsed at the finishing line of the Hastings half marathon.

I had no clue that I had an undiagnosed heart condition, I always considered myself a healthy person.

Before this fateful day, I would run roughly 20+ miles a week and I had completed the London Marathon in 2007.

I’m thankful that this condition showed itself on this day, at an event where defibrillators were at hand. It scares me, to think that this could have happened to me at any time, people with my condition commonly die in their sleep.

On that Sunday, I was picked up in Hove by others runners I had met through my running group. We all arranged to meet after the race for a lift home. I remember the day being very cloudy and wet.

Before the race I texted 5 of my friends to say that I wasn’t feeling right and to wish me luck. I don’t even remember sending that message, I don’t remember feeling queer!

I woke up in hospital on Wednesday, I had been in intensive care for 2 days.

I remember coming out of intensive care, feeling like I was waking up in a horror film. Everything was explained to me, what happened but I don’t think I ever really took it in, maybe it was the shock that I was lucky to be alive.

When I Died!

When I died,

There were no white lights,

No pearly white gates,

Nor did I fall upon the burning pits of hell.

My life didn’t flash upon my eyes,

Nor did I rekindle those moments I desired!

I did not feel my soul leave my body,

To look down upon my lifeless body.

When they brought me back,

I didn’t feel my lungs fill with air,

Nor do I remember my first breath.

For days I laid in intensive care,

Those days were a dreamy haze.

My family sit around my bedside,

All teary eyed, they nearly lost their boy!

Naked as the day I was born

Wired to the elevens,

This is no heaven!

A shock to the system the day I died,

To breathe again,

I cried!

I don’t remember running, my body must have gone into-auto pilot, getting me to the finish line in 1 hour 49 minutes. Once I collapsed St John Ambulance went into action, they realised I was going into cardiac arrest so they shocked me with defibrillators. I was also told that I had another attack while I was in the ambulance on route to hospital. My family was phoned, the police visited my work place and told my manager to expect the worst.

After having an MRI scan at Brompton Road I was diagnosed with Brugada syndrome. It had already been decided that I was going to have an implantable cardioverter defibrillator (ICD) inserted but had to wait on the MRI before getting the go ahead.

I took everything in my stride, which seemed to surprise a few people. I looked at it that I was lucky to still be here, so I was like “do what you have to do”.

On the day of my operation, I couldn’t think straight. I had to sign permission for the operation, for the very small possibility that I could die under this procedure. I think that scared me the most, the thought of dying; I showered twice to make sure I was clean. When I was taken down to theatre, I laid down on the operating table, I was so scared that a solitary tear rolled down my cheek. I remember a nurse asking me if I was okay, I lied ‘cause in truth if I had option I would have ran out of that theatre.

Four hours later I woke up in my hospital bed, with a room full of friends, it meant the world to me to be surrounded by so many friends. The next day I was released from hospital, after 3 weeks I was so happy to be out. I was told to take it easy, but on the day of release I was out walking along the beach. A week and half later I was back at work, I wasn’t allowed to do any lifting or climb ladders – among many other things – for about 6 weeks.

Watch Joseph talk about his experience of having an ICD fitted below.

Life is pretty much back to normal for me now, sometimes I even forget that I have an ICD – though it looks like a bar of soap under my skin. I found out about CRY by chance. I wrote many blogs about my experience on Facebook and Myspace and a mother who lost her son to a cardiac arrest contacted me after reading one of my blogs – she told me about CRY and all their work. So I checked out the CRY website, contacted them via email, chatted to Alison a few times, told my story and the rest is history.

I’ll continue to raise awareness through CRY and help give support to people that need it.

It took me over a year to put back on my running shoes, I was finally able to find the courage after attending a couple of myheart meetings.

The only barrier in life is yourself, the moment you say “can’t” is the moment you fail.

Watch Joseph and myheart member, Paula Simmonds, debate with CRY myheart cardiologist, Dr Michael Papadakis on exercising with an ICD below.

  • Joseph cover

David Hillson

Living with suspected arrhythmogenic right ventricular cardiomyopathy (ARVC)

In February 2008 I was out running with a friend. It was bitterly cold and I had not run over the Christmas period so this run was my attempt to ‘get back into it’!

I had always been fairly healthy, played football all my life (and rugby at University) but at the age of 29 I had kind of fallen out of fitness, so a run once a week was my small way of keeping fit.

Anyway, after 20 minutes of the run I felt dizzy and before I knew it, collapsed. After coming round, within minutes I passed out again, so my friend rang for an ambulance. It arrived within 10 minutes and I felt a little embarrassed by what I felt was a bit of a fuss.

On entering the Ambulance they took an ECG and my heart was beating at around 150bpm. They were a little worried as I had stopped exercising about 15 minutes before. Within minutes I was finding it hard to catch my breath and was rushed to hospital.

What I did not know at the time was that my heart had gone into ventricular tachycardia and was accelerating into a life threatening arrhythmia. On arriving at the hospital I was taken through A&E and ‘shocked’ (defibrillated) whilst still awake, to correct the arrhythmia. My heart had apparently almost reached 300bpm.

After the initial overnight observation and resulting stay in the hospital I was transferred to St Thomas’ Hospital in London for further tests. Its funny to reflect on this time, as the night I collapsed was the night before I was due to have a joint birthday party with a friend – all I was preoccupied about was whether I would be OK to go to that!

After a MRI, EP studies and consultation between the cardiologists they advised I have an implantable cardioverter defibrillator (ICD) fitted as they could not rule out the risk that I could be in for further arrhythmias. The doctors feel that I may have the condition known as ARVC and the ICD would act as the ultimate safety net. On receiving the news I was shattered. Though it had been inferred all week it was to be the most likely outcome, it still shocked me as I felt like my life had been altered forever.

So I had my ICD fitted the day after my 30th birthday. Once over my first few weeks of recuperating life pretty much got back to normal. Bike riding and lots of walking were my attempts back to ‘normal’ life. I have since qualified as a teacher and begun teaching in September 2008.

All seemed well until I received my first ‘shock’ from my internal ‘safety net’! I was playing 5 a side football – so was really pushing my heart to its limits perhaps.

It is odd that I have never had a single heart complaint in my life but suddenly this year it seems there is a real problem. However, it is comforting to find out (from sites like this, or being in hospital itself) that there are others out there who have had similar experiences – and like I try to think when reflecting on this: at least I am alive and typing this!

Claire Herridge

Living with left ventricular wall abnormality

A year and a half ago, when I was 34, I was out running when I experienced heart palpitations, chest pain and shortness of breath. I was taken by ambulance to Southampton General Hospital.

This was a shock to me as I am a PE teacher and have always lead a reasonably healthy and active life. After a few hours in hospital my symptoms had settled and I was just about to be sent home when a doctor asked for an ECG as he still wasn’t happy with my heart rate.

This was taken and it showed a massive abnormality. After several ECG’s, echocardiograms and then an MRI scan, I was diagnosed with having a large mass in the left ventricular wall of my heart.

This resulted in me having open heart surgery to have the mass removed. The surgeons said it was the size of a tennis ball and if I hadn’t recently seen the photographs of the operation I wouldn’t have believed it myself….. but it really was that big!

The mass was benign and I am now feeling good and am hoping to start running again soon. I take beta-blockers every day and have resumed my job as a PE Teacher.

The doctors still don’t know why this mass occurred and apparently the condition is extremely rare, but they continue to research as to why and how it occurred.

Julia Hubbard

Living with right ventricular outflow tract ventricular tachycardia (RVOT-VT)

I have represented Great Britain in Bobsleigh since 2006 and In October 2007 I crashed in a race and sustained fractured vertebrae and tore all the ligaments in my thoracic spine. I was out of the sport for the rest of the season while I recovered and had rehab.

In April 2008, a month after returning to training, I began to feel dizzy, faint and breathless while I was training.

My heart would feel like it skipped a beat and then race out of control – over and over again while I trained. I went to the doctor who did a basic ECG and told me it was stress and signed me off work for a couple of weeks.

Initially I was only getting symptoms in the gym and track so I was sure it wasn’t stress. I called my team doctor who immediately called me to the Olympic Medical Institute for an exercise ECG. I met Greg Whyte and some of the CRY team and less than two minutes into the test my symptoms started and the test was suspended and I was rushed straight to hospital for an echocardiogram. A week later I was back in London for more tests and an MRI scan. My ECG and symptoms were consistent with two conditions – arrhythmogenic right ventricular cardiomyopathy (a condition that can cause young sudden cardiac death) and right ventricle outflow tract – and they had diagnosed which I had. I was diagnosed with right ventricular outflow tract ventricular tachycardia (RVOT-VT).

The treatment was a lifetime on beta-blockers, which pretty much ended my chances of continuing with competitive sport! As soon as I took the beta-blockers I felt great – my symptoms stopped and I could start to train. I just managed to retain my place on the GB team for the 08/09 season but over the next few months my fitness just got worse and worse. I ended up as a reserve for the season and every time I tested my power, speed and strength, my scores were declining at an alarming rate. In March 2009, I came off the medication as I was so unhappy with the effect it was having and my symptoms returned even more severe than before. I decided to try and live with it, which turned out to be impossible.

I was exhausted all the time – sleeping 10 or 11 hours a day – and at its worst I couldn’t even walk without feeling faint. I couldn’t complete a full training session and was devastated, I just felt ill all the time. I lost my place on the GB team and with it my health insurance, so CRY stepped in and put me in touch with Dr Sharma [now Professor Sharma] who has been monitoring me ever since.

He has explained the condition and the triggers which are not completely understood, but for me appear to be a combination of adrenaline, exercise and stress. RVOT-VT is caused by an extra natural pacemaker that starts firing as well as the main pacemaker and causes the heart rate to shoot up and all the symptoms I experience.

In December 2009, as mysteriously and suddenly as my symptoms started, they virtually stopped. I have been able to start training fully again. I decided to compete in Figure Competitions (a type of women’s bodybuilding) as I needed a goal to aim for and I have placed second in three competitions this year and qualified for next year’s British Finals.

My goal is to compete internationally in 2011 in Figure Competitions. I have also decided to train for the World Master Athletics Championships in 2011. In November this year I was able to return to international Bobsleigh, competing in a European Cup race and achieving a start placing top 10 in the field, which I was really proud of considering I never thought I’d race for Team GB again!

I know my symptoms can return at any time so I am enjoying feeling well and making the most of being able to train again. I also now have the support of CRY and Professor Sharma, so as soon as the symptoms return to the severity I had before, I will have a radiotherapy ablation to cure the problem.

Although the thought of surgery is a bit scary, just knowing that there is a cure if and when I need it, means I don’t have to worry any more. In the meantime I fully intend to make the most of every opportunity I have!

In February 2010 I competed in the British Masters Indoor Athletics Champs and won Gold in both the 60m and 200m for my age group (W35). Now I’m aiming to compete at the World Masters Athletics Champs in the summer – as long as the condition doesn’t get worse I think I could do well! It’s funny, a year ago I didn’t think any of this could be possible and although I know I could get ill again, I know it can be treated so I’m not worrying about it! Just enjoying life and never taking my health for granted any more – just making the most of every day!

Arrhythmogenic right ventricular cardiomyopathy (ARVC)

Read personal stories from myheart members with ARVC here.

The incidence of arrhythmogenic right ventricular cardiomyopathy (ARVC) is now thought to be higher than previously believed (affecting 1 in every 1000 individuals), due to the availability of better diagnostic techniques and general awareness of the disorder amongst the medical profession. ARVC was first recognised in the late 1970’s. It is anticipated that even more information regarding ARVC will be available in the coming years, to help us understand the natural history of the condition.

What causes ARVC?

ARVC is caused by a defect in the ‘glue’ that holds the muscle cells of the heart together, working as a unit. When stretched the ‘glue’ breaks down, the muscle cells separate and an inflammatory process begins to repair the break. In a way the heart muscle sustains an injury that the body attempts to repair. As this process is repeated there is a progressive replacement of the normal heart muscle cells by scar tissue and fat. Initially this may only involve small areas of the right heart but later on it becomes global and may even involve the left side.

ARVC is inherited, passed on in the genes from one generation to the next. The pattern of inheritance is such that the child of an affected parent has 50% chance of inheriting the condition. The disease affects men and women equally and has been recognised in people of diverse ethnic origin.

What are the symptoms?

Clinical presentation is usually with symptoms of palpitations (feeling the heart pounding) because of a fast and irregular heart rhythm. The irregular heart rhythm may be associated with light-headedness or fainting episodes. Unlike most cardiomyopathies, shortness of breath and chest pains are unusual symptoms and tend to occur at the later stages of the disease.

How is it diagnosed?

The diagnosis of ARVC can be extremely difficult and usually requires specialist expertise. Your doctor will usually start by asking you some questions and examining you. Most of the investigations are painless and non-invasive, similar to those performed in the diagnosis of hypertrophic and other cardiomyopathies. Initial investigations include a tracing of the electrical activity of the heart (ECG) and an ultrasound scan (echocardiogram). As with hypertrophic cardiomyopathy (HCM), the ECG is very sensitive in picking-up ARVC since up to 80% of individuals with the disease will have an ECG abnormality. The echocardiographic features, however, can be very subtle in the early stages of the condition, often confined only to the right ventricle and therefore further imaging of the right side of the heart is required in most cases with a magnetic resonance imaging scan (MRI). Further evaluation includes an exercise treadmill test and a 24-hour Holter monitor (tape) in an attempt to capture the irregular heart rhythms. In some specialist centres further invasive investigations are performed in an attempt to identify the electrical faults of the heart muscle associated with ARVC (electrophysiological studies) and to biopsy part of the heart muscle and examine it under the microscope. These investigations, however, are not widely accepted, they are still being developed, can still miss ARVC, which affects some areas of the heart but not others and can be associated with potentially serious complications.

Advances in molecular genetics (DNA) means that in some centres, the condition may be diagnosed using a blood test. This test, however, is not available in every hospital, it is expensive, the results may take up to several months and it is not always positive in ARVC patients (we are able to identify a defective gene in 60% of clinically confirmed ARVC patients). It can be used after the clinical evaluation to confirm the diagnosis or in the context of family screening.

Treatment and advice

The majority of patients with this condition have no symptoms for many years unless irregular heart rhythm develops. Treatment in the majority of cases aims to prevent or at least control the irregular heart rhythms.

If your tests prove positive your specialist will advise you on lifestyle modifications. You will most likely be advised not to participate in competitive, strenuous activities.

Watch CRY’s myheart cardiologist, Dr Michael Papadakis talk about things to avoid with cardiomyopathy below.

Drug treatment may also be used to control the irregular heart rhythms. Drugs may include beta-blockers, amiodarone or sotalol.

In cases where drug treatment is unsuccessful in controlling rapid heart beats, an implantable cardioverter defibrillator (ICD) may be fitted. This is similar to a pacemaker where a box is implanted under the skin in the chest. The box has a fine wire which is attached to the heart to record and deliver electrical impulses in the presence of an abnormal heart rhythm.

It will be necessary for you to have at least annual check-ups which usually will include a repeat of the initial investigations. Since the disease runs in families, all first degree relatives of the affected patients have to be screened with ECG and Echocardiogram.

Coronary artery anomalies

The heart is a muscle and requires a regular supply of oxygen and nutrients to function. This is supplied by blood carried by two blood vessels known as the left and right coronary arteries. The arteries arise from the left and right side of the body’s main artery, the aorta. In up to 1% of people both coronary arteries originate on the same side of the aorta. This is called an anomalous coronary origin or coronary artery anomaly. The unusual origin of the artery means that the artery must take a different course to supply the heart muscle. In the majority of cases this causes no problems. However, in a small number of people the abnormal course can cause the anomalous coronary artery to be squashed or kinked during exercise. If the blood supply to the heart is interrupted it can cause a heart attack or the heart to beat in dangerous fast rhythms that can be fatal. The cause of coronary artery anomalies is not known, however they do not appear to be hereditary.

What are the symptoms?

Half of people with dangerous coronary artery anomalies experience warning symptoms including chest discomfort or blackouts that usually happen during physical exercise. In people who have symptoms the origin of the coronary arteries from the aorta should be investigated.

How is it diagnosed?

Screening for coronary artery anomalies remains challenging because electrocardiography (ECG) at rest and even during exercise is usually normal, even in individuals at high risk. This can often be done using echocardiography. If both arteries are not clearly seen during echocardiography, more detailed heart scans such as cardiac magnetic resonance imaging (MRI) or computed tomography (CT) can be used.

Treatment and advice

People with low risk anomalies can be treated with beta-blockers and are advised to avoid high levels of physical exertion. If high-risk anomalies are found surgery to re-implant the anomalous artery on the correct side of the aorta can be lifesaving.

Myocarditis

Read personal stories from myheart members with myocarditis here.

The term myocarditis refers to inflammation of the heart muscle. It is most often due to viral illness. Other causes include drug abuse and autoimmune disorders such as systemic lupus erythematosus. Viral myocarditis is relatively common, but most cases are very mild and never come to the attention of the doctor. However, some cases are severe and can lead to considerable impairment in the capacity of the heart to pump efficiently, resulting in severe heart failure. It is the result of poor contraction or electrical instability of the heart due to the inflammation of the heart muscle.

What are the symptoms?

Most patients feel feverish and have generalised aches and pains as with any other viral illness. When the heart is severely affected the patients feel tired and breathless. Chest pain and palpitations are relatively common under these circumstances.Apart from a rapid heart rate, the doctor may not find any other abnormalities when examining the patients with mild myocarditis. However, in severely affected individuals there is evidence of heart failure.

How is it diagnosed?

Simple blood tests may demonstrate markers of inflammation. The ECG may show changes, which are usually non-specific and occur in many other cardiac diseases. However, the patient’s symptoms and the presence of a fever may raise the suspicion of myocarditis. An echocardiogram (ultrasound of the heart) will reveal an enlarged heart, which is poorly contracting. In very mild cases both the ECG and the echocardiogram may be normal and in these situations one should exercise caution and not participate in any sporting activity when symptoms of a flu-like illness are present. In patients presenting with prominent cardiac symptoms and signs, the diagnosis can be confirmed by a biopsy (small specimen of the heart) taken from the right ventricle. More recently, MRI has emerged as a useful method of diagnosing an inflamed myocardium (the heart’s muscular wall).

Treatment and advice

All patients should rest. Patients with heart failure and complicated electrical disturbances of the heart require hospital admission to manage these complications. Steroids are reserved for patients with auto-immune myocarditis at cardiac biopsy.

Refrain from sporting activity until all symptoms have subsided and the ECG and echocardiogram appearances are normal. Generally competitive athletes are advised to refrain from sporting activities during flu-like illness until the symptoms settle, as the majority of cases of myocarditis produce no cardiovascular symptoms.

Over 75% of the patients improve within two weeks without any complications. Approximately 10% of patients proceed to develop dilated cardiomyopathy and require life long treatment with heart failure medication.