Racial Differences in HCM

A recent study  found that African-American patients with HCM tend to be younger and more symptomatic than their white counterparts. Additionally, these patients are less likely to undergo septal reduction therapies or have genetic testing.

The implications, according to Dr. Neal Lakdawala of Brigham and Women’s Hospital who is an author of the study, is that doctors should always consider the possibility of hypertrophic cardiomyopathy in patients with left ventricular hypertrophy.  If diagnosed with HCM, these patients should be referred to specialists with experience in treating HCM.  This could potentially help these patients avoid the two most devastating complications of HCM:  sudden death and stroke.

 

More HCM Research is Needed

A recent study in Europe found that HCM patients’ risk of death continues to exceed the risk in the general population.

This study looked at 4893 patients with hypertrophic cardiomyopathy treated at 7 different European HCM centers between 1980 and 2013. Although the statistics improved for those who were treated more recently, this study makes it clear that there is still much room for improvement in risk stratification and treatment for patients with HCM.

Overweight HCM Patients Fare Worse

This week, researchers from the eight HCM centers comprising the Sarcomeric Human Cardiomyopathy Registry [SHARE Registry] published a paper that every HCM patient should take to heart.

The sobering findings are that overweight HCM patients have a higher incidence of obstruction, heart failure and atrial fibrillation than their normal weight counterparts. As a result of this study, the researchers suggest heightened attention to weight management and exercise in order to prevent disease-related progression and complications.

Continue reading “Overweight HCM Patients Fare Worse”

Data From HCM Registry to Inform Personalized HCM Treatment in Future

Several years ago, researchers from the University of Virginia (UVA) and the University of Oxford announced a joint project involving a large international registry of hypertrophic cardiomyopathy (HCM) patients to facilitate research into HCM. Backed with funding from the National Heart Lung and Blood Institute, this project, known as the HCM Registry, includes data from 2,750 patients with HCM at 44 sites in six countries. 

This week, researchers from UVA announced their first findings from this registry which suggest that HCM patients can be separated into two basic groups:

  1.  Patients with a known genetic mutation who are not obstructed but have scarring of the heart muscle;
  2. Patients who do not have a known genetic mutation and do not exhibit scarring, but who do have a significant amount of obstruction to blood flow.

According to Dr. Christopher Kramer of UVA, this information should provide doctors with information that allows them to better assess the degree of risk to any particular patient, and to help inform a treatment strategy for each patient based on his or her unique profile.

Sudden Death in HCM Less Common than Thought

A recent study by Canadian researchers published in the American Heart Association journal Circulation found a much lower incidence of  HCM related sudden death than expected.  The study included deaths in individuals that occurred between the ages of 10 and 45 in the Canadian province of Ontario between 2005 and 2016.  According to lead author Dr. Paul Dorian of the University of Toronto, the expectation was that 1 incident of sudden cardiac death would be identified in every 100 to 200 people who had HCM.  Instead, researchers found that the likelihood of sudden death of HCM patients was instead only about 1 in 3,000 people/year.

The study also found that found 7 in 10 HCM-related sudden deaths occurred in people not previously diagnosed with the condition.  Men had more than 5 times the risk of sudden cardiac death than women, and most deaths occurred during rest or light activity: only about 17% happened during or immediately after exercise. 

As a result of the study, according to Dr. Dorian, “our findings allow us to lower the temperature on our degree of worry about the condition.”

Another clinical take away from the study, according to the researchers, is that current exercise guidelines for HCM patients may be too restrictive.

HCM Clinical Trials – the Latest News

NEWS FROM MYOKARDIA

MyoKardia recently announced a new clinical trial of its drug, mavacamten (formerly known as MYK-461) which will compare the clinical results of mavacamten with septal reduction therapies currently used in clinical practice, i.e. the open heart surgical procedure known as septal myectomy and the catheter based procedure known as alcohol septal ablation.

The study will be run by the Cleveland Clinic with Dr. Milind Desai serving as principal investigator.  MyoKardia expect to begin enrolling patients in early 2020.

Read the press release here.

 

NEWS FROM CYTOKINETICS:

Cytokinetics released positive data from its Phase 1 study of the drug currently known as CK-274 in a poster session at the HFSA 23rd Annual Scientific Meeting in Philadelphia.  The study found that CK-274 was safe and well tolerated, while no serious adverse events or negative changes to vital signs, ECGs or laboratory tests were observed.

The company will now be moving into a Phase 2 clinical trial of CK-274 in patients with obstructive hypertrophic cardiomyopathy, expected to begin in late 2019.

Read their press release here.

 

 

DISCLOSURES:  HCMBEAT HAS RECEIVED PAST UNRESTRICTED EDUCATIONAL GRANTS FROM MYOKARDIA.  ADDITIONALLY, CYNTHIA BURSTEIN WALDMAN OF HCMBEAT SERVES AS A PATIENT ADVISOR ON THE STEERING COMMITTEE FOR MYOKARDIA’S EXPLORER TRIAL.

2 Companies Testing Drugs for HCM

Two San Francisco based companies are now conducting clinical trials for three drugs specifically targeting HCM.

Continue reading “2 Companies Testing Drugs for HCM”

Should Children from HCM Families be Screened Earlier?

A recent study by doctors at Toronto’s Hospital for Sick Children suggests that current screening guidelines for children from HCM families are inadequate and should instead recommend earlier screening exams. In the U.S., screening begins at age 12 pursuant to American College of Cardiology (ACC)/American Heart Association (AHA) guidelines.  In Europe, screening begins at age 10 pursuant to the European Society of Cardiology (ESC) guidelines.

Continue reading “Should Children from HCM Families be Screened Earlier?”

Scientists Get $10 Million Grant to Develop HCM Treatments

A group of scientists led by Stanford University’s Dr. James Spudich, working together with researchers from the University of California-Santa Barbara, the University of Washington and the Institut Curie in Paris, has recently been awarded a $10 million grant by the National Institute of General Medical Sciences to develop novel treatments for hypertrophic cardiomyopathy (HCM). 

The researchers hope that the added resources from this grant will help them find ways to correct pathological heart protein changes they believe to be at the root of HCM. The team then plans to partner with pharmaceutical companies to develop more personalized approaches to HCM treatment.

Dr. Spudich has long been involved in HCM research and has been a founder of two separate companies which are currently engaged in drug trials for potential HCM treatments:  MyoKardia and Cytokinetics.

A story about Dr. Spudich and the inspiration for his work was featured in this recent post on HCMBeat.

Can a Smartwatch Detect HOCM?

According to a limited study recently published in Nature, researchers were able to detect obstructive HCM (HOCM) using a noninvasive optical sensor contained in many commercial smartwatches.

How the Technology Works

These watches used photoplethysmography, a noninvasive optical method used to detect blood volume changes in the microvascular bed at the skin surface.  The same technology is used in clinical pulse oximeters and is now widely incorporated in commercial smartwatches that have heart rate detection.

Continue reading “Can a Smartwatch Detect HOCM?”