The Big Hearted Echos Blog provides content in the following areas: News, science reviews, HCMA news, and perspectives.

From the desk of the Hcma ceo:

Salberg

A Man Called Otto

By Lisa
Posted in

A true MUST-see for the HCM Community. Today I should be calling my sister and signing… “when I’m 64”, but instead, I will remember the beautiful soul we lost when she was only 36. Yesterday I went to see the new movie “A Man called Otto”, in this movie, Tom Hanks character Otto has HCM.…

Responding to Sudden Cardiac Arrest: The Damar Hamlin teachable moment

By Lisa
Posted in

Commotio Cordis appears to have occurred at the Bill’s game, Monday, January 2, 2023, with the collapse of Damar Hamlin. If you don’t know what this is… sudden cardiac arrest occurs after a chest blow. While uncommon, it speaks to the need for AEDs on all playing fields. The HCMA shares in the hopes for…

The Audacity of Grief

By Lisa
Posted in

Can we talk about grief for a moment? We all experience loss, and each loss impacts us differently. There is no rule book for processing grief, and that’s a beautiful thing – processing grief is unique to the individual it happens to. Grief is defined as a specific type of suffering that is often tied…

The Mavacamten Marathon

By Lisa
Posted in

Camzyos™ Approval This is an exciting time for the HCM Community.  The FDA has approved Mavacamten under the brand name CAMZYOS, the first-in-class medication for symptomatic obstructive hypertrophic cardiomyopathy and the first drug developed specifically for HCM. Read the press release here CAMZYOS Patient Portal CAMZYOS Medication Guide CAMZYOS U.S. Prescribing Information CAMZYOSTM REMS (Risk Evaluation and Mitigation Strategy) Our…

Ever Wonder Who’s at the Center of Patient-Centered Care?  Look Again

By Lisa
Posted in

Of all the players in the healthcare system, patient advocacy organizations are often the most disadvantaged when a drug hits the market and they find themselves pitted against health providers, payers, pharmaceutical companies, and policymakers, all of whom want their piece of the financial pie.

Lori

The day that changed my life: June 12, 1995

By Lisa
Posted in

The day that changed my life was June 12, 1995, a Monday. The phone rang at 5:50am, which is never a good thing. I managed to roll over with my 8-month pregnant belly and answer the phone on the second ring. The voice was familiar but panicked it was my mother. “She is not breathing…

Lisa, age 12

Lisa, we have your heart. How soon can you be here?

By Lisa
Posted in

So there I was holding my heart in my hands while a new heartbeat was in my chest. Talk about surreal moments! The path that led to this moment encompasses 36 of my 48 years on this earth and in this one moment, I came face to face with the very thing that I had hated, feared, and loved… my heart.

HCMA Blog Roll

blog roll

Cardiac implantable device infections

By admin

The HCMA participated in an American Heart Association summit of more than 40 health care professionals to bridge gaps in awareness, detection, and appropriate treatment of cardiac implantable electronic device (CIED) infections.

CYTOKINETICS AFICAMTEN RESEARCH

By admin

REDWOOD-HCM OLE REDWOOD-HCM OLE: First Long-Term Data from Open Label Extension Cytokinetics’ also announced positive data relating to aficamten from REDWOOD-HCM OLE (Randomized Evaluation of Dosing With CK-274 in Obstructive Outflow Disease in HCM Open Label Extension) and the results from two additional analyses of omecamtiv mecarbil from GALACTIC-HF (Global Approach to Lowering Adverse Cardiac Outcomes Through Improving Contractility in Heart Failure), all presented in Late-Breaking Science Sessions at Heart Failure 2022, an International Congress of the European Society of Cardiology.…

National Mental Health Month

By ross

In recognition of Mental Health Month, the HCMA is working to raise awareness of trauma and the impact it can have on the physical, emotional, and mental well-being of children, families, and communities. Mental health is important at every stage of life, from childhood and adolescence through older adulthood. SAMHSA’s National Helpline, 1-800-662-HELP (4357) (also known as…

Many cats have HCM.

Updated thinking on HCM genetics

By Gordon Fox

Since around 1990, HCM has been regarded as a disease caused entirely by single mutations in genes affecting a protein in the cardiac sarcomeres. There is no doubt that sarcomere mutations play an important role. But in the last several years, there has been a growing realization among researchers that this cannot be the entire explanation for the causes of HCM. This is because a majority of HCM patients do not appear to carry these mutations, and many people who do carry them never develop HCM. The development and inheritance of HCM are more complicated than was previously thought. However, for patients, many of the lessons previously taught about HCM genetics remain true. Current research may open the way to a much-improved understanding of HCM.

Myosin and actin

Mavacamten instead of septal reduction?

By Gordon Fox

On April 2, Dr. Milind Y. Desai, a leading HCM researcher and practitioner, will present initial results on mavacamten’s use in severely obstructed HCM patients.

Two new state-of-the-art reviews on HCM

By Gordon Fox

An expert panel of HCM doctors published two state-of-the-art reviews in the Feb. 1, 2022 issue of Journal of the American College of Cardiology. The reviews are aimed at doctors who treat patients with HCM. But they include much information of interest to patients.

Fitness in HCM

By Sabrina Cuddy

Did you resolve to get more active this year? There is a lot of confusion about exercise for HCM patients. Some doctors advise “don’t do anything physical,” while others say, “do whatever you want.” But we can likely agree on one thing – they are both wrong, and the best advice is somewhere in the…

Thanksgiving and gratitude

By Gordon Fox

It’s Thanksgiving in the U.S. But we’re not going to list things we should be thankful for, or rehash the history of the holiday. Instead, let’s think about gratitude, a response to some benefit given to you by someone (or something) else. Mental attitudes have been shown to affect our health. And this seems to…

ICER Publishes Final Evidence Report and Policy Recommendations on Mavacamten for Hypertrophic Cardiomyopathy

By ross

ICER has released its evidence report and recommendations. Here is a list of highlights: All stakeholders have a responsibility to facilitate meaningful patient access to multidisciplinary centers of excellence for HCM in ways that do not exacerbate disparities. The manufacturer of mavacamten should commit to sponsoring research that will address the lack of evidence on…

Weight loss improves HCM patients’ heart health

By Gordon Fox

Weight management is a challenge for many. Losing weight is a challenge for nearly everyone who tries it. It can be more difficult for many HCM patients because we often take drugs that slow us down. It’s also hard to study: researchers can assign different groups to different diets, but how can they tell who…

News - HCMA and HCM related news

1News

HCM and Mental Health

By Gordon Fox

Learning that you have a serious chronic disease can be stressful. Now a group of researchers in Korea provide evidence (Park et al. 2022) that mental health problems can be an important consequence of diagnosis with HCM. Methods The research team was led by Hyung-Kwan Kim of Seoul National University. These scientists compared the risk…

Mavacamten Affects Diastolic Dysfunction

By Gordon Fox

Background HCM patients commonly suffer from diastolic dysfunction (also called heart failure with preserved ejection fraction (HFpEF), or left-sided heart failure): their hearts do not fill adequately. As a result, even after septal reduction therapy (SRT: myectomy or alcohol septal ablation), some patients are quite symptomatic. Can mavacamten (Camzyos) help relieve their symptoms? Now a…

Expert consensus on syncope in HCM

By Gordon Fox

Syncope (fainting) is common among HCM patients, but its causes are frequently unknown. An expert panel has published guidelines for diagnosis and treatment of syncope in HCM.

Septal Myectomy Today

By Gordon Fox

What’s the status of septal myectomy today? A recent review paper by an Expert Panel for the American Journal of Cardiology (Maron et al. 2022) concludes that “Surgical myectomy remains the time-honored primary treatment for hypertrophic cardiomyopathy patients with drug refractory limiting symptoms due to LV outflow obstruction.” One of the safest of heart procedures…

Can HCM Patients Use Alcohol, Chocolate, & Coffee?

By Gordon Fox

We hear many different claims about what HCM patients (or heart patients in general) should or shouldn’t eat and drink. This is a big topic, and for much of it, the science doesn’t have simple answers – at least not yet. But it turns out that there are some answers for three things that many people like (and many people won’t touch): wine, chocolate, and coffee.

NYHA Class Categories

By ross

Doctors usually classify patients’ heart failure according to the severity of their symptoms. The table below describes the most commonly used classification system, the New York Heart Association (NYHA) Functional Classification1. It places patients in one of four categories based on how much they are limited during physical activity. In the HCM community, this NYHA…

ACC: ORIGINAL INVESTIGATIONS Myosin Inhibition in Patients With Obstructive Hypertrophic Cardiomyopathy Referred for Septal Reduction Therapy

By ross

BACKGROUND Septal reduction therapy (SRT), surgical myectomy, or alcohol ablation, is recommended for obstructive hypertrophic cardiomyopathy (oHCM) patients with intractable symptoms despite maximal medical therapy but is associated with morbidity and mortality. OBJECTIVES This study sought to determine whether the oral myosin inhibitor mavacamten enables patients to improve sufficiently to no longer meet guideline criteria…

Training and Experience Count: Myectomy is Safest at High-Volume Centers

By Gordon Fox

Myectomy is safest at high-volume centers.

Cardiac implantable device infections

By admin

The HCMA participated in an American Heart Association summit of more than 40 health care professionals to bridge gaps in awareness, detection, and appropriate treatment of cardiac implantable electronic device (CIED) infections.

CYTOKINETICS AFICAMTEN RESEARCH

By admin

REDWOOD-HCM OLE REDWOOD-HCM OLE: First Long-Term Data from Open Label Extension Cytokinetics’ also announced positive data relating to aficamten from REDWOOD-HCM OLE (Randomized Evaluation of Dosing With CK-274 in Obstructive Outflow Disease in HCM Open Label Extension) and the results from two additional analyses of omecamtiv mecarbil from GALACTIC-HF (Global Approach to Lowering Adverse Cardiac Outcomes Through Improving Contractility in Heart Failure), all presented in Late-Breaking Science Sessions at Heart Failure 2022, an International Congress of the European Society of Cardiology.…