Special Considerations for the Diagnosis and Treatment of Hypertrophic Cardiomyopathy

Cardiovascular medicine is evolving rapidly and decision-making is becoming more complex. Sometimes, even the best scientific evidence cannot address the ambiguities in clinical presentation and responses to treatment. This is especially true in the clinical course of hypertrophic cardiomyopathy or HCM. Healthcare providers’ limited knowledge of HCM juxtaposed to the nature of risk stratification in disease posits shared decision-making as nearly mandatory in provider-patient interactions.

Tune into these videos to learn more as the speakers share relatable anecdotes, personal insights, and straightforward instructions to explore shared-decision making in the treatment and management of HCM.

Together Everyone Achieves More: Shared Decision Making in HCM

Nurse to Nurse Conversation

Thriving with HCM: I Am My Own Best Advocate

Patient to Patient Conversation

Transcripts of the Hypertrophic Cardiomyopathy Conversations

Nurse to Nurse Conversation

Johana  0:08  

Hey Norma, did you hear the news about that soccer player that died? The news said that it was cardiac arrest. But based on his age, I wonder if he could have cardiomyopathy.

Norma 0:18  

I thought the same thing. Maybe it was HCM or hypertrophic cardiomyopathy, which is a genetic disease that is characterized predominantly by left ventricular hypertrophy in the absence of another cardiac, systemic or metabolic disease, capable of producing the magnitude of hypertrophy, for which a disease-causing sarcomere variant is identified, or the genetic etiology remained unresolved. However, the diagnosis of HCM can be sometimes delayed or misdiagnosed.

Johana  0:54  

You know, as a nurse practitioner, I don't want to misdiagnose someone or delay someone's diagnosis. What should I do to identify a patient that may be at risk?

Norma  1:02  

You can refer the patient for a comprehensive workup because it will take an entire multidisciplinary team to treat the patient successfully.

Johana  1:13  

You know I’ve worked with genetic counselors before. What other multidisciplinary team members might be involved in the care of hypertrophic cardiomyopathy patients?

Norma 1:22  

Cardiologists, preferably from an HCM Center of Excellence, or those who specialize in treating patients with HCM. You can also have genetic counselors, electrophysiologists, cardiac surgeons, or interventional cardiologists involved.

Johana  1:39  

As a nurse practitioner or as a nurse working on the floor, what else can I do to help my patient?

Norma  1:45  

It is important to take every opportunity to teach the patient how to manage their symptoms daily. HCM is a different kind of heart failure. The muted sarcomere genes trigger myocardial changes, leading to hypertrophy and fibrosis, which ultimately results in a small and stiff ventricle would impair systolic and diastolic performance despite the preserved left ventricular ejection fraction. You can teach them about increasing fluid intake, taking their beta blockers, but more importantly, a shared decision making is recommended in developing a plan of care, including activity, lifestyle, and therapy choices.

Johana  2:33  

Do you think an implanted cardiac defibrillator could’ve saved that soccer player's life?

Norma  2:40  

According to the 2020 AHA/ACC guidelines, among referral-based cohorts of patients with HCM, 30 to 40% will experience adverse events, including sudden death events. Studies also report that for patients who are at risk, the application of contemporary cardiovascular therapies and intervention have lowered HCM mortality rates to less than 1% per year. One of the major treatment initiatives responsible for lowering this mortality has been the evolution of sudden cardiac death risk stratification that can identify adult patients with HCM at greatest risk of sudden death, who are then candidates for implantable cardioverter-defibrillator or ICD placement.

Johana  3:39  

Wow, this has been really helpful. I feel much more prepared to treat my patients now. But where can I learn more?

Norma  3:48  

PCNA has a lot of helpful resources online. Just go to www.pcna.net/hcm for more information about HCM.

[Visit pcna.net/hcm to learn more]

Patient Conversation

[Phone rings]

Lisa  0:11  

Hi Ross, thanks for contacting the Hypertrophic Cardiomyopathy Association for your navigation call. And today, we're going to be going over some of the records that you have sent over, and making sure that you have a full understanding of your anatomy and some of the questions that you may want to go over with your healthcare provider team. You ready?

Ross  0:31  

Yes. Thanks for taking the time to meet with me. I just got my results from my cardiologist. I've been having some chest pain, and shortness of breath, which led me to the hospital. And my sister had passed quite early, and I just wanted to talk to you a little bit more about my results. I have apical HCM. And I just need to know a little bit more.

Lisa  0:59  

Ross, the first thing about HCM is to understand your anatomy, and then break it down to understanding the hemodynamics of your heart, and then understanding your risk factors for sudden cardiac arrest. So first, if we were to look at the imaging, the echo, the stress echo, and the MRI combined, we would be able to look at the results to see what the anatomy tells us. In your case, it looks like there is an apical presentation, which means that the thickness is isolated to the bottom portion of the left ventricle. So that is just your anatomy. It's not good, it's not bad. It's just how your HCM is presenting. There has been enough research that tells us that a certain percentage of scar indicates a higher risk of sudden cardiac arrest. And that number is 15% or greater. You’re indicating that your scar burden is 17%. And that would mean a conversation with your health care provider about a potential implantable defibrillator to protect you from cardiac arrest would be a wise next step.

Ross  2:08  

My hospital doesn't do the ICDs. I do have a pretty good relationship with my cardiologist here, but he doesn't feel comfortable continuing with my care. Do you recommend that I continue going to this larger hospital? Or do you think he can handle it locally?

Lisa  2:28  

We believe at the HCMA that a combination of care models does actually work best, meaning you have a relationship with your high volume Center of Excellence. You can find a list of those at 4hcm.org. You can also use your local cardiologists, if they're comfortable. You can help them learn about HCM, do lots of programs such as PCNA.net, or HCM Academy. There are other learning opportunities available. And maybe we can help your local cardiologists get a little bit more comfortable working with you so that you can have the level of care that you need both at home and at your center of excellence.

Ross  3:10  

Okay, thanks so much that really, it's a load off my mind. I also, I've been short of breath. And I've been having some chest pains. And there's just so much information to try to keep track of, I wasn't even sure what to bring to this meeting. Do you have any advice on just how to keep track of all the numbers in your head?

Lisa  3:33  

Well, it's probably a little challenging to keep them in your head. We have many tools available at hcma. One of our patient journal books is one idea. PCNA.net also has other trackers that you can use. And there are other things available online. But we definitely recommend that you track your symptoms. Log if you notice any changes in your symptoms related to particular activities, food, stress. Make note of all of these factors so that you can have a very detailed and articulate conversation with your chosen health care providers. Additionally, you'll want to track things like who in my family needs to be screened. When did I bring my children for their last screening, and make sure that these are being done consistently and that the family knows to be screened. This is a communication process, and it is a lifelong disease. So you need to make sure that you're tracking things over time and not forgetting to follow up with your physicians.

Ross 4:37  

The nurse at my local office said that I need to be an advocate for my health. And I'm typically not that kind of person. What are some things that I should be advocating for?

Lisa  4:47  

Great question, self advocacy. It's a challenge, especially when maybe culturally we were told not to be too problematic for our doctors and not to ask too many questions because they're really busy. You want to log your feelings, emotional and physical. You want to ask good questions and get answers that you understand. And when you don't understand the way somebody is answering you, you might want to look for alternative ways to understand that information.

Ross  5:19  

Thank you for helping me out. It's kind of a scary time. It's a lot of information. But I really appreciate you taking the time to speak with me and making my life a little bit easier.

Lisa  5:32  

We're happy to be here, and I encourage you to stay online and look at PCNA.net and 4hcm.org to get more information on HCM and also just good general information on how to advocate for yourself in healthcare. 

Ross 5:47

Thank you.

[Visit pcna.net/hcm or 4hcm.org to learn more]