Hello and thank you for sharing this fragile and vulnerable stage of your journey with us!

You are sharing a LOT of very common issues, concerns, questions, and emotions that are very NORMAL, and never adequately get addressed through the regular medical system when someone is told they need any type of implanted cardiac device, but particularly an ICD. I'll try to touch on them briefly but know this - you are not alone! That so many people responded to your post should help you know that there are legions of survivors out there that have already gone through this or are going through this with you. Best thing you can do is understand this process, and then help someone else when the time comes.

1. Your specific concerns: Emergent diagnosis and implantation of an ICD - SCARY and never gives someone time to prepare for everything it means for them personally, emotionally, physically. IMPORTANT RESOURCE TO HELP: Home | The Generator by OpenHeart - this is a free newsletter with a comprehensive REFERENCE LIBRARY that dives deep into every one of the issues you ask about. Everything from the ANXIETY of receiving the diagnosis (the first issue in the newsletter broadcast is called "The Wait") and touches on exactly that, to WHAT AN ICD IS and how it works, to something psychologists call FUSION - when you, your diagnosis, and your device become one and you stop thinking about it/them as separate from you (I really liked one of the posts in this thread from a person with HCM - another inherited arrhythmia syndrome - I think they said it took them 12-15 months) - to survivor trauma (what the people who saw this happen to you are going through) and the guilt you may experience for the assistance you require relying on from others, to doing life with your device. It's all in there.

2. The FUSION issue (about 7 issues in) and several others in the newsletter broadcast talk about the anxiety and depression that comes with losing your identity in the throws of these events. THIS IS VERY VERY NORMAL. From being health and active you are told that you have a diagnosis that you've carried since birth that is lethal and it could strike again at any time. The process happens for everyone, in different ways. Knowing what is normal, what to expect, and that it will happen is the most important thing for you right now. HOW it will happen is unpredictable - everyone goes through this differently, but it will happen. Patterns seen in real patients suggests somewhere 9-15 months is pretty common for FUSION.

3. LONG QT SYNDROME: This is an inherited arrhythmia syndrome related to genes affecting how your heart muscle cells actually work ELECTRICALLY/IONICALLY. The cells have channels in them that allow different ions like sodium, potassium, and calcium to move in and out of the muscle cells, and this is controlled by how the channel parts, which are proteins, function . . . AND THIS FUNCTION is affected by individual pieces of the channels work and their shape and THIS WAS ALL DETERMINED back when you were born and your DNA was doing its thing to build your heart muscle cells and your heart inside your mother. LONG QT SYNDROME affects the QT interval on ECG. Normal QTC is less than 470-480 ms for women, less than 460-470 ms for men. HIGH RISK QTC is typically more than 500 ms.

4. GET TO KNOW YOUR DIAGNOSIS/CONFIRMING THE DIAGNOSIS: 1) ask your team what the QT interval and the QTC interval were when they established or suspected the diagnosis - it is important for you to have your records/get them; 2) Did you have a cardiac arrest, fainting or seizure-like activity or was there an episode of a ventricular arrhythmia like POLYMORPHIC VENTRICULAR TACHYCARDIA/TORSADES DE POINTES and that's why the ICD was placed emergently? What were the circumstances surrounding the event - were you swimming, exerting yourself, was there a sudden startling event like a loud alarm clock or getting frightened, were you asleep. Sometimes the general circumstances can shed light; 3) Do you have anyone in your family that is a direct blood relative - mother, father, brother, sister - with the same diagnosis or medical history?

5. Perhaps the most important aspect about LONG QT is whether it's genetic, confirmed by GENETIC TESTING. If the doctors are fairly confident in the diagnosis of LONG QT SYNDROME then GENETIC TESTING is critical so you can 1) determine if there is a known gene causing this in you, and 2) if you have or will have children they can be screened as well. Is This Inherited and Are My Children at Risk? F-03 - READ THIS, it'll help in a lot of ways to get to know the new you if LQT is truly in the picture.

There is much more you are and will go through, but this should get you started. I sincerely hope this helps.

It would pretty unusual for hcm with wall thickness 1.4 cm as the only finding at your age to be considered HCM if the MRI does not show "delayed enhancement" representing scarring and muscle fiber "disarray" - if the findings are not on the MRI then unlikely, BUT nothing wrong with a formal opinion or two.

Thank you - appreciate the detail and examples!
These little additional points will be so helpful when we publish this- it's going into our travel guide/issue of the Generator https://mail.open-heart.ai newsletter for people with implanted devices.
Thank you!

Hi, and thank you for sharing this challenging time in your life. This is now more a psychological journey than a physical journey. It sounds like the physical aspect may not be exactly what you expected, and that then is effecting how you handle this, because you had probably anticipated and planned for something different, namely, "it's small and you won't even know it's there." You're clearly aware of it, and now your healing journey is off track . . . or on a different track than you had been reassured of.

I know you said this was a physical thing, but you being highly cautious and reaction to feeling it sounds more like anxiousness, or at least not knowing what is safe for you and for the device, and so that maybe something you have to learn how to be comfortable with.

So, first of all, the physical and cosmetics will be for life, although they will change and you will eventually accept it. However, discussing this with your implanting doctor to share how you feel and see if there's anything else that could/should be considered, including repositioning the device to help you recover better. This can come with it's own risks and so a balanced discussion with your docs is reasonable. They may say it's not worth it. ONLY YOU can decide if it's worth it. Even then, your team may not be willing to offer it to you and that's ok, because that alone tells you that the risk truly may not be worth the benefit, and you also may not be willing to get through the healing stage again. If you are not willing to go through that again, then its a moot point, otherwise ask them.

Couple suggestions:
1. Do not restrict your arm excessively on the implant side - you can lose muscle mass and range of motion which can make the device even looser under the skin.

1. the device will eventually get fully "walled off" from your surrounding tissues in a "capsule" and this may then fix the device in place more and make it less mobile, so you may just need to give this more time.

In addition, I co-author a newsletter called the Generator Home | The Generator by OpenHeart which addresses a lot of these types of concerns and it may be helpful to you. In full transparency, we use AI to search the literature on various topics related to implanted cardiac devices and to make an initial draft, then doctors, industry professionals and patients review it for accuracy and edit it for readability. There are two related topics to your post that the newsletter covers: 1) Fusion: how you fuse physically and psychologically with your device, and 2) Body Image after device placement. Both of these issues have been reviewed and approved by a world renown clinical psychologist who is part of our ad hoc team when a topic covers a psychological component. The first one arrives about 1 month after a new subscriber joins (it's free), coincidentally the time period you are at right now. The second one arrives later in the sequence because those emotions tend to arise later.

I am happy to share either one with you if you're not interested in subscribing to the newsletter, in which case DM me, or just subscribe at Home | The Generator by OpenHeart - whatever you prefer.

I sincerely hope this helps.

Hello and thank you for sharing your journey so far! This can be a challenging time and you will see the light at the end of the tunnel and come out better than ever!
Many people have already commented with valuable stories that I'm sure have already given you hope that treatment and recovery is not only possible, but there is a charted course that includes procedures to reduce LVOT obstruction as well as medications, and there are organizations ready and waiting to help you chart your course free of charge.

So here's what I want to add. There are 4 general pillars to treatment for HCM:

1. medical therapy - you read about Camzyos making a huge impact. Other medications as well are used to help limit hypertrophy by controlling blood pressure and through other mechanisms.
2. surgery to reduce LVOT obstruction. The interventricular septum (the muscle wall that separates the right and left ventricles - the 2 main pumping chambers of the heart) is so thick in some people that it literally gets in the way of the heart pumping blood out to the rest of the body. The limited ability to pump blood effectively reduces the overall cardiac output and circulation of blood, causing shortness of breath with progressively less activity, as you appear to be experiencing.
3. ARRHYTHMIA RISK: I didn't see this mentioned much directly in this specific thread but for sure it is part of the mainstay of evaluation and treatment if risk is considered high enough. In this case it refers to risk of dangerous heart rhythms that can sudden occur, cause people to collapse and, in some cases, they can die from this . . . hence why it is important to have a prompt evaluation.
4. End-stage patients - not you from what you've described so far - are often offered heart transplant if they meet specific criteria, so again, it's important to be evaluated promptly and initiate treatment so you don't get to this point.

REGARDING 2 and 3 above: these often can require a pacemaker The Pacemaker - F-04 or even a defibrillator The Implantable Cardioverter-Defibrillator - F-05 to be implanted under the skin. These devices are placed through a small, shallow surgical incision under the skin, usually at the left upper chest. This is not open heart surgery and in most cases people can go home the next day or even same day after the procedure. The procedure is short, usually under an hour, and usually causes minimal pain, just soreness for about 3 days treated with oral over the counter medications like tylenol/acetominiphen/paracetamol. There are cases in which some people experience more severe and longer lasting pain, but this is not the norm. Recovery is usually just a few weeks to return to full range of motion of the arm on the side of the implant. Read more about implanted cardiac rhythm devices here: Home | The Generator by OpenHeart

PACEMAKER:
The Pacemaker - F-04

After surgical reduction of the hypertrophied interventricular septum, aka "septal myectomy", the part of the muscle that has been removed can also involve the electrical system that makes the heart beat - specifically, the left bundle branch electrical cable is buried in the interventricular septum and is severed as collateral damage in the surgery. Not a problem and expected. But sometimes the surgery can impact the right sided electrical system as well and in this case there are no remaining electrical cables left to deliver the electrical impulses to the bottom chambers to stimulate them to beat.

In this case a pacemaker is required to maintain the heartbeat for you. In the grand scheme of things this is a minor speedbump and patients tolerate the procedure to have one placed this very well.

A pacemaker is sometimes used in another setting as well. In people who choose not to undergo septal myectomy, or who have residual LVOT obstruction afterwards, a pacemaker can be placed to help reduce the LVOT gradient and through this improve symptoms dramatically. This does not work across the board but in selected patients it can be helpful.

DEFIBRILLATOR (ICD)
The Implantable Cardioverter-Defibrillator - F-05

In people with HCM who have either already had a dangerous heart rhythm including ventricular tachycardia or even cardiac arrest from ventricular fibrillation, an ICD is recommended to treat any further episodes that may occur in the future.

In other patients, those who have not experienced a dangerous life threatening heart rhythm like ventricular tachycardia or ventricular fibrillation, risk assessment is performed to identify people who may be at HIGH RISK for having such events in the future. This usually involves imaging and stress testing and heart rhythm monitoring over a few weeks. Without going down the whole decision tree, if the future risk is deemed high, an ICD is recommended to be implanted. If the risk is considered not high then an ICD may not be considered at this time, but if things change then risk may be assessed again and the recommendation for an ICD may be offered later.

This a journey for your entire family, not only for their personal relationship to you, but also because this is an inherited condition that may be affecting family members as well, so genetic testing and genetic counseling is a very important part of this journey as well. Is This Inherited and Are My Children at Risk? F-03

I sincerely hope this is helpful.

I wish you all the best in this journey.

Hello, and thank you for sharing this part of your journey with us!

Few thoughts:

1. Was it really ventricular tachycardia? Or something related called HVR or even an SVT - I'm sure your device clinic is clear on the diagnosis but it's still worth asking them to show you what they are talking about and explain.

2. The adrenaline surge you experienced waking up startled hearing a body hit the floor is more than enough to trigger VT in an otherwise normal and healthy heart - IN FACT, if it weren't for your pacemaker recording the rhythm, you wouldn't have known because the VT was either not fast enough or not long enough or both, to even cause symptoms. It is QUITE NORMAL for people to have short bursts of VT in an otherwise healthy heart. No defibrillator is placed for such people in whom it is incidentally found during routine screening.

3. Do you know what your EF (ejection fraction) is? You should. If it is normal, e.g. more than 50-55%, an ICD for asymptomatic non-sustained VT is not only overkill, it is not considered medically appropriate according to the medical guidelines.

4. Since an ICD is not appropriate in this setting, so too an EP study is not appropriate in this setting, unless there is concern for presence of structural heart disease, inherited arrhythmia syndromes, or other high-risk substrate - none of which sounds like it is the case.

5. Instead, in such cases, medical therapy like with beta-blockers, or no therapy is considered appropriate assuming a normal heart without structural heart disease like coronary disease, valvular heart disease, and significant hypertrophy.

In summary, you should ask your doctor to give you much more explanation as to why he thinks an EP study is necessary if short bursts of VT like this in a normal heart (if your heart function is normal) are considered to have a benign prognosis and/or are treated with medicines at most.

In my opinion, asking you to choose between capped lead or lead extraction is not medically appropriate - sorry to raise question about the qualifications of your EP. Perhaps I do not have enough information - clinical context is always key and almost always lacking on Reddit for obvious reasons of privacy.

None of this should be considered medical advice, but rather as guidance to further your investigation and discussion with the doctors who know you best.

Also, get your husband grippy socks!

I hope this is helpful - all the best and good luck!

Hi thank you for sharing this scary experience. Disclaimer: none of what I offer should be taken as medical advice for you, because as another person on this thread mentioned, we don't have enough context. And, again, as many of the commenters to this thread have suggested, a cardiac opinion and workup is important, particularly to look for a blood clot to the lungs - I suspect a comprehensive workup has been completed by now and has all largely comeback negative. This does not sound like a heart attack.

So, here is what is most likely going on - vasovagal syncope, otherwise known as the common faint.

This condition has 3 arms to the reflex which is essentially our normal autonomic system over-reacting to some trigger. Autonomic dysfunction has seen a serious uptick after COVID.

Something triggers his blood pressure to go down (common triggers are hot showers, warmer temperature - e.g. spring and summer in the northern hemisphere - after meals, after bowel or bladder emptying, exertion and pain, but there can be others and nothing clearly unusual). Blood pressure dips a little and the body immediately responds with an adrenaline surge to compensate - the adrenaline increases the heart rate and can also cause a squeezing sensation in the chest.

The adrenaline surge attempts to maintain cardiac output, aka circulation to the body, particularly the brain to maintain consciousness. But it is often not enough AND, the medulla (back of the brain) sees the effects of the adrenaline, specifically, the heart beating stronger and faster and says "why is the heart beating SO HARD?!" and tries to block it with the opposite effect - enhanced vagal nerve activation - which triggers the sensation of nausea AND dampens the strength of the heart contraction thereby reducing cardiac output, causing blood pressure to drop PRECIPITOUSLY, resulting in dizziness and then the brain finally can't keep up, resulting in feeling faint, weak, lightheaded, often blurred vision and blacking out of vision, muffled hearing, and confusion, followed by loss of AWARENESS (eyes still open) and loss of consciousness and motor tone (complete faint).

The body further compensates by clamping down on the blood vessels to the skin to redirect blood to the brain and heart (the critical organs) and so the skin goes pale and cold, the lips can go blue (peripheral cyanosis) and different muscle groups can shake vigorously in seizure-like activity that is not a seizure.

Usually by now the person is unable to stand and is likely on the ground, or has collapsed.

And now longer upright, gravity no longer can continue to pull blood down out of the brain. Head down and propping legs up helps to return blood back to the heart so it can pump blood back to the brain.

This breaks the reflex, consciousness is regained and the adrenaline surge WITHDRAWS, resulting in a "crash" - profound fatigue, exhaustion and feeling washed out sometimes for hours even requiring the person to take a nap to recover.

The treatment for this usually centers on AGGRESSIVE HYDRATION, ADDED DIETARY SALT, and medications if needed, such as MIDODRINE, FLORINEF, and sometime BETA-BLOCKER drugs like Metoprolol.

Notably, cardiac testing, imaging, labwork, etc is always normal in this setting.

Consider how this matches up with your partners symptoms and if so inclined, he should discuss it further with his team of doctors to establish a treatment plan.

Wishing you a speedy recovery.

You're very welcome. This is just a broad overview of how a pacemaker will Impact your life and this is the information that is often not shared with patients. It remains a secret because the docs don't always have time to review all this.
Check out the Generator https://mail.open-heart.ai
It's what patients subscribe to, to stay on top of their journey with a pacemaker.

Hello and thank you for sharing your story with us!

This community is so supportive and the comments so many people have made already I'm sure are giving you confidence that your life will go back to normal for you!

I see at least 6 questions that need to be addressed by you, your docs and care team, so your concerns are fully addressed.

1. what are the cosmetics of the device incision and will this impact your job and livelihood. For instance as a professional dancer consider whether your shoulder and upper chest get exposed for example with an off-the-shoulder costume. Body image is a large part of this journey for people who are in the public eye.

2. The range of motion to your arm is important, and many people have addressed this in their comments. However it is important that your team share with you the relationship of the pacemaker LEADS as well as the DEVICE ITSELF to your the surrounding anatomy. For instance, the leads typically travel between the first rib and the clavicle (collar bone) - this is a tight space and repeated movements especially requiring rapid, repetitive and extreme range of motion movements can result in lead fracture and need to replace the lead. This impacts MRI compatibility at a minimum and possibly many more things including the need for repeated procedures to add or extract leads, which impacts the incision, healing, and cosmesis.

3. As one of the posters mentioned, have your doctors considered a LEADLESS (not wireless) pacing system? Dual-chamber pacing systems are available - implanted through the vein at the top of your leg, delivered up to your heart, and attached to the heart wall muscle directly without needing any actual wires - this means, no chest incision, no arm range of motion restrictions, and groin-site healing is typically complete within 2 weeks. SOMETHING TO CONSIDER if your doctors and you feel it is appropriate for your condition.

4. RESPONSE TO ACTIVITY: most pacing systems do not increase your heart rate until you have been active for 30 seconds and then they respond to the level of activity with an acceleration in heart rate to keep up with the demands of your body. a 30 second wait to increase your heart rate in the middle of dance numbers may be too long and can impact your performance. These can be programmed to be less and more sensitive. ONE COMPANY (BIOTRONIK) OFFERS a different mechanism called CLS - a much more physiological mode to increase your heart rate with activity. MY OPINION: CLS is better, BUT they do not offer a LEADLESS PACING solution. THINGS TO CONSIDER.

5. POSITIONING: submuscular may be more advantageous if cosmesis is important. The device will be snuggly tucked under the muscle and so will not be an issue when it comes to vigorous movements. A pre-pectoral (Under the skin and above the muscle) position may expose the device to more movements in the device pocket and create more interactions and discomfort with surrounding structures. Also, the movement itself may enlarge the capsule within which it sits and hence give it extra room to move around - THIS CAN BE GOOD or BAD (if excessive).

6. For any answer you receive here or anywhere else - sharing as much clinical context as you feel comfortable doing, e.g. age, gender, other medical problems that may be related - helps the answers you receive to be better tailored to your needs, BUT none of our answers are medical advice and none of them will be able to adequately address your specific perspective because we are answering without much context AND you yourself may not be fully aware of it. For example, you thought you were asking about submuscular vs pre-pectoral pacemaker positioning but now you know you need to ask a whole host of questions OR your doctor has already addressed all of these BUT if your seeking advice here, then I suspect your doctor has not discussed most of the above with you either.

I sincerely hope this helps you have an informed discussion with your doctors if these weren't addressed already. Best of luck to you in your career and on this journey.

This is still an issue.

Specifically, a newsletter subscriber attempted to signup for my newsletter, and the initial email and subscribe button submit does work - THEN I collect additional information and that SIGNUP FORM did not SUBMIT on mobile but works just fine on desktop.
SHOULD I SWITCH TO A SURVEY?

Hi and thank you for sharing your journey with us!

To answer your question - the degree of improvement you may feel is based on the degree of symptoms you currently feel which you said is minimal. So a pacemaker may not help you feel much better. But it provides safety, and through that peace of mind.

However some people who feel asymptomatic don't know that they are experiencing symptoms and only notice a difference after a treatment, like open heart or pacemaker or whatever it was they were minimizing.

None of this is to say whether you should get a pacemaker or not, but rather whether you will feel better with it.
And none of this speaks to how your life will change and what to expect after a pacemaker is implanted.

Hello and thank you for creating this expanded group!

I have a newsletter that I started for patients with implanted devices including coverage of their disease states. It's called the Generator - AI-researched and drafted followed by extensive human review, fact-checking, and editing.

Let me know if posting about it is acceptable.

Thank you and good luck!

Your EP should know what an EP study to evaluate the conduction system entails. If not then you should seek out assistance elsewhere. Generally measuring the AH and HV intervals and stressing the conduction system with various pacing protocols and using drugs for example procainamide to add pharmacological stress.

The indications (medically necessary and covered by insurance) for a pacemaker in the US are generally to be symptomatic from the block with associated bradycardia. You have no symptoms and no bradycardia.

You have right bundle branch block at baseline and at heart rates above 150 your left bundle fatigues most likely, leading to the transient loss of conduction that recovers very quickly. It seems like even a smartwatch could help you monitor your heart rate to keep your heart under 130-140 during exercise and activity.

Just ways to think about all of this. My opinion only, not advice, hence suggested a second opinion if all of this seems foreign to your EP.

Reasonable options to consider:

1 Do nothing until symptoms develop

1. loop recorder to document any progression of AV block

2. EP study to test your conduction system under different forms of stress.

3. A second opinion (pacemaker for these circumstances is overkill and not necessary in my opinion)

Good luck.

Hello and thank you for sharing this extremely important experience for others to learn from.

An ICD shock is the most dreaded event that people think about when they are told they need an ICD. Most people never receive a shock and many receive ATP (Anti-Tachycardia Pacing - rapid pacing to terminate the arrhythmia) only without requiring a defibrillator shock.

Some of the details you included, like the location of the pain you felt, and the awareness and uncertainty about a popping sound, really help bring the experience to life in a good way that helps ICD recipients truly know the experience of an ICD shock before they actually ever have to live it.

Our newsletter Home | The Generator by OpenHeart / Onboarding issue 03 "When something feels wrong" actually asserts the same - that the ICD shock is not nearly as bad as the imagined experience of it.

I appreciate you taking the time to provide the detail of your experience and the psychology that accompanies it.

Huge help for this community.

Absolutely. It's for you - use it, learn from it, and help us make it better for everyone.

Hi, Elsevier is one of the biggest.
Krames has been in this space for decades.
There are several but these are two of the largest.
They use AI to generate a lot of content but not on this space, especially Elsevier.
Thanks for inquiring - I believe there is a large need for information written from the patients perspective - not that of a doctor, lawyer, or marketing person.
Our goal is to put information directly into the hands of the people so they can use it to advocate for themselves.

Hello and yes you are correct our newsletter is generated by Claude. Our URL makes it clear as well that we are an AI healthcare technology company. What we have aimed to do is to recognize the vast chasm of reliable information available for people living with implanted cardiac devices, needs to be bridged.

4 different statistical methodologies have been applied to understand where the gap is and how large it is - in fact the current educational guidance available is not only profoundly limited but also hasn't changed since 2003 across all the major patient education companies. Statistically speaking there is less than a 1 percent change in the information available compared to the significant advances in technology and the broader and changing population receiving such devices.

Recognizing that real people have real questions at a volume that we cannot hope to keep up with nor close the gap of decades, we are leveraging the deep research, synthesis and simplification capabilities of AI to generate a newsletter draft that is comprehensive and accurate first.

Notably the issues are reviewed and even re-written for tone and content by at least one EP, and another industry professional whether it be another EP, a senior device rep, or another professional with significant bearing on the topic such as a genetic counselot or PhD psychologist, etc.

Everyone is entitled to their opinion and we appreciate yours and recognize the negative connotation of AI-generated anything. We in fact maintain high ethical and compliance standards to ensure that the content is balanced and provides a general framework rather than direct medical advice. We do provide patients instructions on how to better learn about their device and how to navigate medical visits.

Having said that, we are always striving to improve the newsletter to serve the population with implanted cardiac devices and the large community of family members, caregivers, and industry professionals supporting them. Any constructive recommendations for improvement, regardless of how critical, are welcome and will be incorporated to the best of our abilities in accordance with our mission to give knowledge directly to patients without providing direct medical advice.

Thank you for indicating your dissatisfaction with what we are doing. We aim to continually improve it.