The main priorities with Andrew are to control any arrhythmias (his Loop recording showed episodes of non-sustained runs of ventricular tachycardia) and reduce the risk of sudden cardiac death. Management may involve:
- Optimal dosing with Beta-blockers (Bisoprolol). Beta-blockers improve ventricular relaxation and increase diastolic filling time. This should reduce the likelihood of ventricular arrhythmias occurring.
- Implantation of a subcutaneous Cardioverter Defibrillator (S-ICD). An ICD is a small device that uses electrical shocks or impulses to control potentially life-threatening arrythmias. Evidence suggests that S-ICDs have a number of benefits. They are easy to remove and replace, and require less extensive surgery than ICDs placed in the chest or abdomen. As there are no leads placed in the actual heart itself, the risk of infection in less. Patients should receive fewer unnecessary shocks as subcutaneous devices are more able to discriminate between normal and abnormal heart rhythms.
Andrew’s details are inserted into the European Society of Cardiology (ESC) HMC Risk-SCD Calculator
| Age (years): | 16 |
| Maximum LV wall thickness (mm): | 15 |
| Left atrial size (mm): | 35.5 |
| Max LVOT gradient (mmHg): | 30 |
| Family History of SCD (Yes/no): | No |
| Non-sustained VT (Yes/no): | Yes |
| Unexplained syncope (Yes/no): | No |
The ESC HMC Risk-SCD Calculator recommends that Andrew should be considered for ICD implantation.
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Following further discussion, at home, with his parents and family, Andrew decides to go ahead with the surgery to insert a sub-cutaneous ICD.
Andrew and his father meet with the cardiac arrhythmia nurse.
Scene 1:
Nurse: Hello. I’m Trish, one of the cardiac arrhythmia nurses. How are you both today?
Dad: A bit nervous, to be honest. Everything seems to be happening so quickly – it’s difficult to take it all in.
Scene 2:
Nurse: What about you, Andrew? How are you feeling?
Andrew: Not as bad as my dad! I just want to get everything sorted, so that I can go back to normal.
Scene 3:
Nurse: Has the doctor explained to you that he would like to fit you with what is called an Implantable Cardiac Device?
Andrew: He has. He explained it to me but it was a bit difficult to take everything in.
Dad: It would be really helpful if you could just go over it again with us.
Scene 4:
Nurse: Your ICD will look like this (shows them an ICD). The one you are being fitted is called a subcutaneous ICD and it will lie just underneath your skin, on the left hand side of your chest. The ICD will monitor your heart rhythm all the time. It can detect when your heart is having abnormal heart rhythms and will give your heart a shock if it detects abnormal/dangerous rhythms.
Scene 5:
Andrew: Will I feel when that happens? How often will it happen?
Nurse: You will feel it. It’s difficult to explain until you experience it but some people describe as being like punched in the chest, while others say it’s like having a small electric shock. Some people seem to be able to tell when it is about to happen and feel a bit dizzy or have palpitations before a shock, but not everybody does. We don”t know how often this will happen to you, as everybody is different. It may never happen or it may happen quite often. In a way, it’s a safety mechanism to make sure that your heart doesn’t stop working.
Scene 6:
Andrew: What do I do if I have a shock? Do I have to go to the doctor?
Nurse: Once you have the device implanted, you will be given a S-ICD identity card and instructions on what to do. If you have have a shock and feel ok you should call the device clinic. If you have numerous shocks and feel unwell you should dial 999. We will go over everything with you before you leave hospital.
Scene 7:
Andrew: Will I still be able to play football and do everything I normally do?
Nurse: The guidelines recommend that you don’t play sport at a highly competitive level but it is certainly okay to play football with your friends. You should avoid contact sports such as rugby and judo, incase the device gets damaged, but there is no reason why you shouldn’t do everything else you did before your cardiac event.
Scene 8:
Dad: Does having the device fitted involve a big operation?
Nurse: No, the operation normally takes about two hours. It does mean that Andrew will need a general anaesthetic, though. Afterwards, there will be three small wounds which can be checked at your local health centre. All being well, Andrew will be allowed home the day after his operation.
Scene 9:
Dad: And is that it? Does the device last a lifetime?
Nurse: I’m afraid not. The battery on the device normally lasts around five years and will need changed after that. We’ll keep an eye on battery levels regularly to make sure they don’t run down. As I said, Andrew will be given an S-ICD identity card, emergency information and full instructions. You can also phone me or one of the other arrhythmia nurses at any time, if you have any concerns – I will give you our number.
Scene 10:
Dad: It’s quite a lot to take in.
Nurse: It is, I totally understand that. Why don’t I give you some information leaflets and some useful websites, so that you can both go home and have a further read about everything? You can phone me or email me if you have any further questions once you’ve read more.
Dad: Thank you, you’ve been really helpful.
Pulse point
Hypertrophic cardiomyopathy is an inherited condition, caused by changes or mutations in specific cells in the cardiac muscle. This has implications not only for Andrew but also for his family. With his permission, the cardiologist refers Andrew to the cardiac genetic service.
