Here Comes The Science Bit…..

I’m feeling a bit ‘meh’ today so I’m hiding behind the science. I’m going to write this once because in all honesty I still find it a bit hard and a bit weird telling people about my CHD.  I really don’t like the attention and usually fob off people with – oh it’s a dodgy valve, its ok, I’m being monitored, yeah I had a hole in the heart, blah blah blah blah.

So today with my factual head on I shall be boring the pants off you with all the technical medical terms of what my CHD was and what it still is…..

I was born with a hole in my heart.  The technical term is Aortopulmonary Window – AP Window for short. This means (consulting the trusty t’internet) that there is a hole connecting the major artery taking blood from the heart to the body (the Aorta) and the one taking blood from the heart to the lungs (Pulmonary Artery). The condition is congenital, which means I was born with it.  Somewhere along the line in my genetic make up my heart didn’t build itself quite as properly as it should have done when I was in my mother’s womb.  Also reading about this on the internet, it says this condition is quite rare, I’m not so sure (it’s an American site that I’m reading, Medline Plus, and I’m very British about not big-ing anything up 😉 I also can’t seem to find anything on AP Windows on any UK websites)

So, in the ‘Normal’ heart blood flows through the Pulmonary Artery into the lungs, where it picks up oxygen. Then the blood travels back to the heart and is pumped to the Aorta and the rest of the body.  Babies with an Aortopulmonary Window have a hole in between the Aorta and Pulmonary Artery. Because of this hole/window, blood from the Aorta flows into the Pulmonary Artery, and too much blood flows to the lungs. This causes high blood pressure in the lungs (a condition called Pulmonary Hypertension – more on that later) and heart failure. The bigger the defect, the more blood can enter the Pulmonary Artery.  The condition occurs when the Aorta and Pulmonary Artery do not divide normally as the baby develops in the womb.  Aortopulmonary Window is very rare. It accounts for only 0.1% of all congenital heart defects.

Here’s a picture of what it looks like:

Image

Looks tiny doesn’t it?  It marvels me that something so small can cause so much trouble!!

This condition can occur on its own or with other heart defects; 50% of patients usually have no other heart defects…. Oh shit, I fell into the other 50%, had to be awkward didn’t I 😉

So it took 2 attempts to close this ‘hole’. One when I was just over a year old and again when I was 6years old. How they did this was by Open Heart Surgery (OHS). During the operation, the chest is opened and a heart-lung machine takes over for the child’s heart. The surgeon opens the aorta and closes the defect with a patch made either from a piece of the sac that encloses the heart (the Pericardium) or a man-made material. (I think my patch is me, I’m pretty sure I would have been told if I had something Nylon in me…)

My first OHS was performed by Mr. Milstein, I don’t remember him, my second OHS by Sir Terrence English, before he became a Sir for his pioneering heart surgery especially Heart Transplants.

I don’t want to go too much into the ‘problems’ I faced as a child, as I find it quite challenging as it’s tied up (understandably) with emotions and I don’t want to chat about the emotional stuff today (still hiding behind the science, just call me Spock!), but briefly, I never did PE at school, I was blue and cold, didn’t eat well and didn’t grow well, I was quite listless and tired. So a riveting, fun child then.

After the age of 6 I was left alone for 10 years apart from having 6monthly checkups.  As far as I was aware I knew I had another operation to come when I was 18, I vaguely knew I had a blocked Aortic Valve, but I never engaged with it.  I was too busy trying to be ‘normal’ and part of the gang.  Then unexpectedly at 16 I found out they had brought the operation forward.

The 3rd operation in its correct medical term: ‘Sub Aortic Stenosis, resected with Myomectomy’.  Basically means that my Aortic Valve was narrowed (Stenosed) and damaged by a crazy extra bit of heart muscle membrane sitting under the valve, causing the blood to flow through all sorts of congested problems.

Reading on the t’internet it says: Subaortic Stenosis refers to an obstruction or narrowing at the outlet of the left ventricle just below the aortic valve. In typical Subaortic Stenosis, the Aortic valve itself is normal. – Ahhh I’m not your typical girl, my Aortic valve was blocked too.

In the normal heart, there are two sides, and each side has a pumping chamber, called a “Ventricle”.  The un-oxygenated blood comes back from the body to the right side of the heart, where it collects in the right Ventricle.  The right Ventricle pumps this blood through the Pulmonary artery to the lungs, where it collects more oxygen.  The blood, now full of oxygen, returns to the left side of the heart and the left Ventricle, where it is pumped through the left Ventricle’s outflow tract to the Aortic valve and out to the body through the Aorta.  In a heart with Subaortic Stenosis, it is difficult for the left Ventricle to pump the blood out to the body because of this narrowed area in the outflow tract.

Subaortic Stenosis causes two main problems for the heart:  Extra work for the left Ventricle to pump blood through the obstruction and turbulent blood flow created by the narrowing may damage the Aortic valve, causing an otherwise normal valve to leak.

Luckily I had Discreet Subaortic Stenosis, the more common (hurrah!) form of Subaortic Stenosis which occurs when a membrane made up of fibrous tissue forms the obstruction underneath the aortic valve.  The operation for this condition has a high likelihood of success. The operation begins with a vertical incision in the middle of the chest and dividing the breastbone.  While repairing this area, it is necessary to work on a still, quiet heart, so use of the heart-lung bypass machine is required again while the heart is repaired.

Subaortic Stenosis is fixed by opening the aorta just above the Aortic valve. The Subaortic Membrane can be seen through the valve. The membrane can then be cut out, leaving the left Ventricle’s outflow tract open and smooth.  This operation is called Asubaortic Resection.

It is uncommon to have major complications after Asubaortic Resection. It is possible to have a leftover membrane, though it is extremely unusual for that to cause any remaining obstruction. Other very uncommon complications include damage to the Aortic valve itself, heart block, which is a problem with the electrical conduction of the heart, or creating a hole between the two pumping chambers (Ventricular Septal Defect).  It is possible for the Subaortic Membrane to re-grow. There is no good way of predicting re-growth of the membrane from child to child. Thus, patients with Subaortic Membranes need to be followed for a long period of time by a cardiologist to make certain that re-growth of the membrane has not occurred.

Again this OHS was carried out by Sir Terrence English, apparently he tested out a new piece of equipment (a little whizzy thing that is now used in Angina patients for clearing clogged arteries) on the crazy bit of muscle (the Myomectomy bit) and then stuck his little finger in the valve to open it up further (The Aortic Stenosis bit).  I can safely say that Sir Terrence is the only man ever to have poked my heart with his pinkie!

So that’s that.  What I was left with was a leaky valve.  Go away and forget about it I was told, and in truth, I have managed to do that for the last 28years.

So at present the medical terms for what I have now are: Aortic Regurgitation with Pulmonary Hypertension. So breaking that down into English it means; Aortic regurgitation is where blood leaks back through the aortic valve. This is because the valve does not close properly, (Rembember to widen the valve Sir Terrence stuck his little finger in it to stretch it). With each heartbeat, more blood than usual enters the left ventricle and so it needs to work harder. Mild regurgitation may not cause symptoms. More severe regurgitation can cause symptoms and may lead to heart failure. Medication can help to ease symptoms. Surgery to replace the valve may be needed.  If the backflow of blood becomes worse the left ventricle has to work harder with each heartbeat to pump the extra blood back into the aorta. The wall of the ventricle may then enlarge and may also become thickened. Symptoms can then include: Dizziness and or chest pain when you exert yourself, and/or forceful heartbeats which you may feel as palpitations.

If the backflow of blood is severe the left Ventricle may not function properly and you can develop heart failure. This causes shortness of breath, tiredness, and fluid retention in various tissues of the body.  Medication may be advised to help ease symptoms of heart failure if heart failure develops.   The medication I’m on, Diuretics (water tablets), were prescribed as I was feeling breathless and my ankles and feet were swelling up.  What the Diuretics do is to make the Kidneys produce more urine. This gets rid of excess blood and fluid which may build up in the lungs or other parts of the body with heart failure. Hence the pissing like a race horse quote from an earlier blog.  I was prescribed Angiotensin-Converting Enzyme (ACE) Inhibitors, these are medicines which help to reduce the amount of work the heart does and to ease symptoms of heart failure, but they left me with a yucky cough and the feeling of fatigue, which wasn’t helping an already feeling exhausted me, so I came off them.  The effect was immediate the next day I felt like running a Marathon….!

The Pulmonary Hypertension is raised blood pressure within the Pulmonary Arteries, which are the blood vessels that supply the lungs. It is a serious medical condition that can damage the right side of the heart, making the heart less efficient at pumping blood around the body and getting oxygen to the muscles.  Symptoms of Pulmonary Hypertension include: shortness of breath/fatigue/feeling faint or dizzy/chest pain/racing heart beat (Tachycardia)/leg swelling – I had it once, my left leg swelled up so much I couldn’t pull my boot up, it was quite scary!  Luckily, I only have MILD Pulmonary Hypertension.

Apart from that I am well, I can dance, I can function on an everyday level, I have my bad days (see last week’s blog).  I had a scare last year where it was thought the membrane had grown back under my Aortic Valve and another hole (Atrial Septal Defect mentioned above) had appeared but with lots of tests it turned out ok.  I’m lucky!  I just have in addition to the leaky valve and the mild Pulmonary Hypertension something simply called ‘Mad Muscle’.  This is the word Cardiologist Jane Somerville came up with to describe heart muscle that wasn’t behaving how heart muscle should behave!!  My arrhythmia and palpitations are tiresome to deal with but after researching my 2nd operation it may well be that all the surgery I’ve had has buggered my electrical wiring and I’ve got a bit of heart block with the electrical conduct of my heart.

So there we are a scientific research into my CHD, thanks to the Internet/Wikipedia/MedLine Plus/Patient.co.uk/NHS Website.  What amazes me is that compared to what help/support/medicines/OHS procedures/equipment that was around when I was first born and diagnosed with a CHD to the advances in all these areas in the last 45years.  You can now do a lot of OHS by keyhole surgery, reducing both physical and emotional trauma for the patient, reducing time in hospital and time in recovery.  Advances are being made all the time, I’m hoping I can hold out a few more years before I need any more surgery as I hope it will be keyhole, as I have been told that a 4th OHS is very risky…

But just touching a little on the emotional side that I’m trying to avoid today, These wonderful, Surgeons, Cardiologists etc who have worked so hard on patching us up combined with the pressure of an NHS service crumbling under a strict government regime, that still manages to more often than not, deliver outstanding results, I just feel that more time needs to be taken to look at the emotional impact of what is going on.  Cardiac surgery may have progressed in leaps and bounds over the last 45years but taking care of the emotional self, the spirit, the bit not on paper or recorded on an Echo Cardiogram, I think that bit needs a little look at, a little cuddle maybe.  That’s why I want to become a counsellor, this type of care needs to be addressed and my Cardiac Nurse is with me on this one.

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7 thoughts on “Here Comes The Science Bit…..

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    • Thank you so much for taking the time to leave a comment. I am so amazed that people are enjoying reading my thoughts and taking time out to respond. I too understand feeling humbled and in my case slightly guilty of seeing others with similar conditions worse off than I. I love your Shark story for your scar, my mum who had also had heart surgery as a child, has a scar that goes all the way around her left side, she used to tell people she got caught on barbed wire!! I have no such interesting story, but my scar does look like a large cross on my chest… I had the nick-name ‘Witch’ by one boyfriend, I liked it! Next week I’m dressing my scar up to look worse than it is for a Zombie bellydance performance…. always handy, no stick on fake stuff for me!! 😉 Maybe I’ll write about the scars one day. Anyway. Thank you again much appreciated x

  2. Very well written and informative piece Raychel – really good that you’re taking the time & effort to explain your condition / defect (or did we agree on ‘distinction’ in the end?….). I’m sorry to hear the problems you’ve had in your life and very glad last years scare was a false alarm. I was born with TGA and had my operation at 10 months old (rescheduled after the only fire in the hospitals history happened in my operating theatre at the ‘exact’ time the surgeon was about to make the first incision – make of that what you may….). They never actually swapped anything over as the technology wasn’t quite there yet I have since learned – but I certainly had some tinkering done and my rib-cage opened leaving me with an 11 inch scar and some pretty impressed faces when I now explain it’s the result of a shark attack!
    I was taken off P.E also – a little later than yourself as I was then a teenager and only allowed to ‘gentle swim’ – which with a single-sex pool meant I’d be with the boys one term and then with 30-odd girls the following term and getting changed in the Janitors cupboard! But as I’ve got older it seems I’ve been pretty lucky health-wise also – I drive myself to appointments without any need for anyone to accompany me etc and I’m always utterly humbled when I see people (in many cases ‘younger’ than I) in the waiting room with the exact same condition as myself but in wheelchairs / on oxygen / with brain damage due to their operation either not being totally successful or that they were too ill at birth to be able to fully recover. It breaks my heart.
    My cardiologists have often told me that apparently I’m in an ‘elite’ little group of people who have had what I have – without a switch or subsequent operation – only needing daily Enalapril as medication and, with a few exceptions – no heavy exercise – no smoking / heavy drinking can pretty much lead a life as ‘normal’ as someone without a CHD . However the day will come when my heart will need to be ‘fixed’ and I’ll have to have the operation I’ve long been dreading – but they don’t want to ‘go in’ while I’m doing this well until my heart decides it’s had enough of doing a harder job than it’s paid to do and they absolutely need to.
    In the meantime I really appreciate your blog and for sharing your experiences – it really helps to know that there are other people out there with similar conditions and it is reassuring to be able to discuss and comment on those issues which affect us. Thank you and wish you all the best in your career path – you will obviously make a fantastic counsellor!

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