Docs Reliably Identify HCM Patients in Need of ICDs

According to a paper published last week in JAMA Cardiology, doctors at Tufts University’s HCM Center have been able to identify 95% of their patients at high risk of sudden cardiac death (SCD) from HCM.  Tufts applied an updated and modified version of the risk factors enumerated in the American College of Cardiology/American Heart Association Guidelines promulgated  in 2011.

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Reporting Issue with 50,000 Medtronic Fidelis ICD Leads

According to a story broken by Kaiser Health News this week, due to a reporting waiver granted to Medtronic by the FDA, as many as 50,000 problems with the Medtronic Fidelis lead were not reported to the FDA.  Ordinarily, the FDA uses its MAUDE database to collect reports of adverse events in medical devices.  In these cases, the MAUDE database was circumvented.

Medtronic responded by saying that instead of using MAUDE, they disclosed the issues in summary fashion to the FDA, as well as reporting them to physicians and to the public.

The following stories provide additional details about this controversy:

CNN

Minneapolis Star Tribune

ArsTechnica

MassDevice.com

 

Wall Street Journal Highlights Risks in Genetic Testing

This story in the Wall Street Journal about genetic testing shows the speed of changes in the medical community’s understanding of how and whether certain genes cause hereditary disease.

The article quoted Dr. Jodie Ingles, a geneticist from the University of Sydney in Australia who specializes in HCM and has published a recent article on the subject.  Dr. Ingles said that 22 out of 33 genes comprising a genetic testing panel commonly used to test for HCM had either limited or no evidence of being disease causative.

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Positive Results for MyoKardia Drug Mavacamten

This week in the journal Annals of Internal Medicine, MyoKardia reported positive results from its open label phase 2 clinical trial of its drug mavacamten (formerly known as MYK-461) for obstructive hypertrophic cardiomyopathy. The study was conducted at 5 HCM centers and enrolled 21 subjects with  obstructive HCM. All subjects saw some degree of improvement to their condition after taking mavacamten.

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When Do You Screen Your Kids For HCM?

A recent study published in Circulation suggests that clinical testing of kids who are first degree family members of HCM patients (i.e. siblings and children of those who have already been diagnosed with HCM) could be improved by starting testing at a younger age. And, genetic testing should further improve diagnosis and treatment for this group.

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How a Louis L’Amour Book about the Southwest Inspired a HCM Discovery

When Stanford biochemist Jim Spudich settled down in bed with a book recommended by his wife, he had no idea that the book would inspire one of the biggest discoveries of his career. Spudich drifted off to sleep while reading The Haunted Mesa, a science fiction novel by Louis L’Amour. His scientific discovery was based on an image he saw in his dreams when the image of a mesa morphed into a myosin molecule.

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Hackers and ICDs: What to Know About Today’s Medtronic Warning

Today, it was announced by the U.S. Department of Homeland Security that 750,000 implantable defibrillators manufactured by Medtronic could potentially be vulnerable to hacking.

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MyoKardia Announces Positive Result for Mavacamten for Treatment of HOCM

This week, MyoKardia announced positive data on its experimental drug for HCM, mavacamten (formerly known as MYK-461), for obstructive HCM.

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Are Your Genetic Test Results Valid?

A recent study by several HCM genetics researchers around the globe, led by Australia’s Dr. Jodie Inglesfound that 2/3 of genetic mutations previously reported to patients as HCM causative may actually NOT trigger HCM.

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The High Hanging Fruit: Treatment for Non-Obstructive HCM – Commentary by Dr. Sharlene Day

A recent study conducted in the U.K. evaluated whether the anti-anginal drug trimetazidine would improve symptoms and exercise capacity for those patients with non-obstructive hypertrophic cardiomyopathy. 

Unfortunately, this study which was conducted by Dr. Perry Elliott and his colleagues at University College London, found that trimetatazidine did not improve exercise capacity in these patients. Following the results of this study, trimetazidine will now join ranolazine and spironolactone in the compost heap of drugs which tried and failed to improve HCM symptoms.  While a third drug, perhexiline, was found to improve symptoms for non-obstructive HCM, its limitations, including potentially serious side effects, stand in the way of its common usage.

In a companion editorial to this study entitled “Non-Obstructive Hypertrophic Cardiomyopathy-the High Hanging Fruit,” Dr. Sharlene Day of the University of Michigan’s HCM Center discusses the difficulties seen in drug trials related to non-obstructive HCM.

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