The Path Forward a HCOM Update

This has taken me so long to post because I am still trying to process a lot of stuff and weighing a lot of pros and cons. I went to my HCM Cardiologist (Hypertrophic Cardiomyopathy Specialist) recently. I had an ECHO first then saw him. It was not good news from the ECHO. Even though I feel no symptoms with the Camzyos, I will need to take drastic measures in the future, though the timeline wasn't really given. But my gradients are not improving. Last time my gradient at Valsalva was significantly elevated with 100% obstruction. This time it was still elevated and still 100% obstruction at Valsalva.

You know that it's serious when the doctor comes in, gets close to you like he is a friend, softens their voice, and says while the medicine is keeping me symptom free, my pressures are too high and I need to start thinking of either a Septal Myectomy or an Alcohol Septal Ablation. If it was one of their family members, they would send them to a major center of excellence like Mayo Clinic or Cleveland Clinic for the myectomy. They just started doing the Alcohol Septal Ablation at a local hospital and the doctor that does it was trained by a leading World Specialist. They could do the septal myectomy, but for this they are not a Center of Excellence, and having the procedure done at a Center of Excellence gives you less than 1% chance of death.

So I have choices to make. I don't even know if my insurance would pay for me to go to one of these major centers, and then you're without a family network being that far away. Then there is my workplace. They recently terminated a long-time employee over FMLA documentation issues. So I don't have confidence that if I have to go out for a long time with the myectomy, I would still have a job. I know that FMLA is supposed to protect you, but it is a fear with the current political climate and business-friendly state laws.

If I do the alcohol ablation, which I am leaning toward, I can get that done locally. This procedure is a controlled heart attack, and the scar tissue is supposed to shrink the muscle and lessen any obstruction so the heart can pump out more oxygenated blood. It has a much shorter recovery time: 3 days in the ICU and likely back to work in 2 weeks.

The same doctor who was trained to do the alcohol ablation is also going to do a heart catheterization in December. The specialist says they need all the details about my heart they can get before making final decisions. My recent cardiac MRI showed 3.5% diffuse LGE, which indicates some fibrosis in the heart muscle. They want the cath to get a complete picture of what's going on with blood flow and pressures throughout my heart.

Then there is going to be a new medicine, a second-generation med called Aficamten that should launch next year. It has a safer profile if the Government ever opens back up and gets approved by the FDA in December as scheduled. I could transition if my insurance approves it, and it has several advantages over Camzyos, but it still may just keep me asymptomatic.

I have been having issues with my insurance company, mostly due to the REMS program requirements, and I believe this has not helped. I went almost 2 weeks recently trying to get my Camzyos for one reason or the other. I know that Camzyos has a half-life of 6 to 9 days staying in the system, but I was starting to feel like I used to at about day 6. So with my insurance also giving me problems with out-of-pocket determinations (one moment I am, the other I am not because of copay accumulator rules).

So with all these issues, I just have been in a kind of haze. I am leaning toward the alcohol septal ablation, but with only a 70 percent success rate compared to over 90 percent with the more invasive myectomy, I am not sure. Because the doctor made it a point that with the alcohol ablation, I could have another obstruction, then the go-to will have to be a myectomy, which is now riskier and trickier. I don't want to go through something where in the future they find this or that shouldn't have been done, like with my Cryoablation in 2016 for AFib. But they didn't know that Cryo was not ideal for HCM patients. Only RF ablation at specific spots should have been done. 1  But Alcohol ablation has been around for a while.

Why all the urgency? Because my chances of dropping over dead from sudden cardiac death (from arrhythmia) or acute heart-failure decompensation.

Just for context, here is a plain explanation for what Valsalva is:

What is Valsalva? The Valsalva maneuver is a breathing technique used during an echocardiogram to stress-test the heart. You're asked to take a deep breath and bear down (like you're straining or trying to blow up a stiff balloon) while holding your breath. This increases pressure in your chest and temporarily changes how blood flows through your heart. In people with HCM, this maneuver often makes the obstruction worse and causes the gradient (pressure difference) to increase dramatically. Doctors use it to see how severe the obstruction really is under stress, since many HCM patients have worse obstruction during physical exertion or strain. The gradient measurements "at rest" show how your heart is doing normally, while the "at Valsalva" measurements show how bad the obstruction gets when your heart is under stress.

My ECHO History
I've had multiple echocardiograms tracking my HCM progression. The pattern shows persistent severe septal hypertrophy with dynamic left ventricular outflow tract obstruction. My gradients at Valsalva have consistently been significantly elevated, ranging from moderate to severe obstruction. My left atrium has progressively dilated from normal to moderately-severely dilated over time, which is concerning for long-term outcomes. Despite Camzyos keeping me symptom-free, the structural changes and obstruction patterns remain significant.

Before starting Camzyos, I was very symptomatic with systolic anterior motion of the mitral valve and resting gradients that were quite elevated. The medication has improved my quality of life dramatically, but the underlying obstruction during stress remains a concern that points toward needing a more definitive intervention.

 [^1]  Nedios, S. et al. "Characteristics of left atrial remodeling in patients with atrial fibrillation and hypertrophic cardiomyopathy in comparison to patients without hypertrophy." Scientific Reports 11, 12411 (2021). https://doi.org/10.1038/s41598-021-91892-y - This study found that radiofrequency ablation is preferred over cryoablation for HCM patients with atrial fibrillation due to more advanced atrial remodeling.