Life is a risky affair. We have to take risks every day in order to lead a meaningful existence. Many of these risks have a very little probability of turning into a real danger and yet this probability exists. It is just so small that we do not pay attention to it. Every year about 75 000 pedestrians in the United States get struck by a car and more than 4000 of them die. We do not think of this probability but simply cross the road because we need to and because we trust the traffic system. Managed risk is a normal and accepted part of our existence, especially when the dangers we face are known and can be mitigated.

Things can get very different when we face a new significant risk for which we do not have sufficient knowledge about or power to act on, especially when the news comes as a shock. Imagine the following situation: you are in your late forties and you have been living your life quite uneventfully when you suddenly feel dizzy and your heart starts racing. Doctors tell you that you have a genetic heart condition and that it can get really bad. There is a chance that you can get a sudden cardiac arrest (usually abbreviated as SCA) and even die. They offer to put a defibrillator in your body to save you from that. Your doctors are supposed to know best but it is ultimately your decision. What do you do? Here are some suggestions.

Step 1 – Collect information to understand the situation better

Many patients who are faced with the decision of an ICD implant know little or nothing about their heart condition and this is normal: this is a highly specialized medical area full of complicated terms. We all need to trust the expertise of our doctors (hopefully you are treated by ones with a relevant experience – if not, make sure you are!) but we need to be able to make an informed choice. Best places to collect information are specialized websites like hcmbeat.com, aicarm.it or cardiomyopathy.org, scientific articles, materials that you receive from your healthcare provider and patient support groups. Note that these support groups are a self-selected sample of people who go there to share mostly the extremes of the experience spectrum – either very bad or very good ones.

Step 2 – Assess the urgency of the situation

Your doctors are the ones who should give a detailed explanation on the proposed procedure, the involved devices and the suggested timing. Under normal circumstances the decision for an ICD implant should not be under time pressure. If you are told that there is an urgency for an implant (which may well be the case), do ask for more details to understand the reasons for this urgency. Do not be afraid that your doctor might be offended by your questions – you are not questioning his or hers professional expertise but simply trying to make an informed decision about your own body.

Step 3 – Understand what risk means in general and for you personally

Risk is a relative concept which deals in probabilities and can vary widely among individuals. If you are a man in his sixties and you are told that you have 1 % yearly chance of SCA, this risk is actually not much different than the one of getting a prostate cancer. The difference is that it is much easier to visualize an SCA. Very few of us are able to make an absolutely impartial assessment of their own risks because they concern us personally and affect our well-being, our family, finances, relationships, lifestyle etc. in different ways. Personal risk perception depends mainly on your:

• Age – older people usually look at risks in a more balanced way.
• Family status – perception of outcomes changes depending on whether you alone are affected by them. This is especially true if you have dependents.
• Character – most of us have a natural inclination to be optimistic or pessimistic.
• Environment – we are influenced by the culture we have been brought in, our friends and relatives, media, etc.

Step 4 – Understand implant consequences and possible outcomes

Positive Consequences

Negative Consequences

Step 5 – Understand the alternatives

We are wired to think in binary terms: something will either happen or not. We are also mostly pessimistic by nature, maybe as a heritage from those times at the dawn of humanity when you needed to be extra careful to survive. Our minds often simplify the choice to “defibrillator implant or I will die”. In reality there are always three options: (1) Implant the device (2) do not implant the device and (3) wait and decide further down the road. While waiting seems counterintuitive, it may have the following benefits, especially in borderline cases where the urgent need for an implant is not obvious:

• More information relevant to the decision may become available (e.g. from an MRI test). It is also possible that some tests may have provided wrong information and redoing them may show the real situation. Mistakes do happen.
• Newer, better devices may become available. This field is constantly evolving in the direction of smaller and more reliable devices which can perform more actions and last longer.
• You may understand better your own idea of what is acceptable risk and what is the lifestyle you will be most comfortable with.

Of course, in case you decide to wait you have to make sure that the option of an implant will still be available to you in the future (this should be normally the case) and you have to agree with your doctor on a strategy and timeline for further tests and decisions.

Step 6 – Understand the actors in the process and their motivations

Apart from you, the patient, there are several other parties who have a vested interest in the outcome of your decision whether to implant a defibrillator or not. These parties are:

• Doctors and hospitals – the doctors, who perform the implantation, are paid for it. Implantation also ensures more and more regular visits to your healthcare provider which correspondingly leads to a higher income for them. Finally, doctors would normally err on the side of caution as a possible cardiac arrest and a lethal outcome for the patient in case of a non-implant might have reputational, psychological or even legal consequences for them (this is why you are made to sign all these disclaimers).
• Device manufacturers  – they obviously have the interest to sell more devices (in other words, to have more implants). The price of these devices is significant, profit margins are big and the overall size of the industry exceeded $ 11 billion in 2021. It is projected to grow to $ 18.2 billion in 2026. They also have the interest that, in case you have decided on an implant, you (or, more likely, your doctors) select one of their models instead of those of the competition. This is a part of a long-standing debate for the relationship  between physicians and the pharmaceutical industry.
• Health insurance (state or private) – insurers normally cover all costs for the device implantation (or a large part of them). As such, they have the interest to approve the cheapest available alternative and to minimize their associated costs.

The interests of these parties are sometimes contradicting and do not necessarily coincide with the patient’s best interest. This is a point to be considered. A study in 2021 compared thousands of HCM patients from US and non-US sites. It found that primary prevention ICDs are implanted much more frequently in patients in US vs. non-US sites. There was a lower rate of appropriate ICD therapy in US patients. The rate of ICD implants in the US was double that in other countries but the rate of negative outcomes on the whole population was the same. It is logical to assume that this is related to the interests of the actors described above.

A real-life story

Here is the story of Sean H., an US patient (shared with patient’s permission):

“I was diagnosed when I was 8 years old. At 15 I had a cardiac arrest and my coach gave CPR and used an AED to bring me back. They put an AICD/pacemaker in the next day. Fast forward 10 years later: I was traveling in Vietnam with some friends when all of a sudden I started getting shocked. It knocked me down in the middle of the street and then again out of bed onto the floor. I immediately flew back to Japan (where I was living at the time) and they determined my ICD was malfunctioning and I needed to fly back to the US to get it removed. They attempted to remove the wires, but, as they did, the wire broke inside my heart because heart had grown larger since the implantation at 15 and had encompassed the wires into the muscle over the 10 years it was in there. They then needed to perform a surgery to cut my heart open and remove the wires. After that they said they wanted to put another ICD in, but I refused. I am now 33 years old and still ICD free. My doctors continually warn me each year that I am “living on borrowed time.”  They’re probably right.

I am extremely active, I have a purple belt in Brazilian Jiu Jitsu and compete regularly.  Normally that would probably be super healthy for those with a good ticker. For me, it’s probably the last thing I should be doing. But it has become a part of my life and is honestly what has helped me get this far.

I live with a constant fear of sudden cardiac death. But I also remember living with a constant fear of a malfunctioning device. It’s a double edged sword.  Not to mention that the combat sport I am active in would not be kind to a computer in my chest and would only be another recipe for a malfunction.

I bring an AED with me to my gym and to my competitions, but I refuse to get another ICD until the technology improves.”

Understood – but should I ultimately go for it?

Well, this is the million dollar question, isn’t it? It is indeed up to you and you alone to make this decision. There is no doubt that the vast majority of patients will outsource this decision to their doctors and this is fine – after all, you entrusted the care for your health to them. This does not mean that you should do anything impulsively and that you should not give the matter a careful consideration. Just make sure that you are properly informed and that you have received at least the answers to these questions:

1. Do the doctors define the implantation of a device as “absolutely necessary”?
2. Was the reasoning for this recommendation clearly explained to you?
3. Do you understand what the implantation procedure involves?
4. Do you understand the technical characteristics of the device which is to be implanted?
5. Do you understand what changes in your lifestyle will the implantation bring?
6. Do you think that the implantation of the device will bring you psychological relief?
7. Will the implantation have a major effect on your work and income?
8. Are you comfortable with the concept of a foreign object being a part of your body?
9. Is the recommendation for the implant done by an HCM specialist?
10. Do you feel in a good physical and psychological shape to take the decision for the implant?

It also helps to make a list with all pros and cons, rank them in terms of importance and consider your decision carefully, especially in the case of borderline recommendations. Your life is on the line – but also the way you live it!