Tag: hypertrophic cardiomyopathy

Doctor with HCM Calls for Patient Access to Medical Device Data

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In an editorial entitled “It’s My Heart, Why Not My Data” by Dr. Ira Nash in the January 2, 2018 edition of Circulation, Nash calls for device manufactures to allow patients to access the data generated by their implantable devices.

Nash, a cardiologist who himself has hypertrophic cardiomyopathy, calls the paternalistic practice of limiting data access to physicians anachronistic in a world which has become more and more focused on empowering patients to make collaborative decisions with their physician.

Dr. Nash explains in a postscript to the article that after being denied patient access to the data generated by his implantable loop recorder, he was ultimately granted access to his data as a physician. Most of us don’t have that ability.  It would certainly be nice if patients were given the option to access their data simply because it is most important and relevant to them.  It is the patient’s life, after all, which is at stake.

Lifestyle Influences HCM

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According to this recent article published in the Journal of the American Heart Association, lifestyle choices can influence the development and/or progression of HCM.

In particular, the authors made the following recommendations:

  •  Exercise:  Recreational exercise should be encouraged in HCM patients.
    • This recommendation was largely based on the findings of the recent RESET-HCM study which found that moderate exercise, specifically tailored to each individual patient’s capacity, was beneficial to the patient’s general health and well being.  **For an in-depth look at the RESET-HCM study, check out this recent HCMBeat interview with the authors of the study, Drs. Sara Saberi and Sharlene Day.
  • Eating and Drinking: 
    • Patients should avoid large meals and should not exercise immediately after eating.  
    • Care should be taken to avoid becoming dehydrated.
    • Alcohol should only be consumed in moderation.
  • Healthy weight should be maintained.
  • Hypertension should be treated aggressively, though treatment may be challenging, especially in obstructive HCM.
  • Obstructive Sleep Apnea, which may exist in as many as 70% of HCM patients, should be treated to minimize potential for arrhythmia and to improve blood flow.

Sudden Cardiac Death in Young Athletes

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Editor’s Note:  This post originally appeared on the blog of Dr. Howard J. Luks. Dr. Luks  wrote this blog entry in collaboration with HCM expert Dr. Srihari S. Naidu of New York’s Westchester Medical Center. You can find the original post here.  You can find both Dr. Luks and Dr. Naidu on Twitter @hjluks and @SrihariNaiduMD.

Sudden cardiac death in young athletes continues with alarming frequency.  The most common cause of sudden death in the young athlete is hypertrophic cardiomyopathy or HCM.  Simply put, HCM means the heart muscle is bigger.  Many of us believe that bigger muscle means stronger muscle.  That is not always the case with the heart.  The heart is a mechanical pump with a complex arrangement of chambers which store the blood. How that pump works is controlled by a very complex electrical system.  Hypertrophic cardiomyopathy can interfere with one or both of these critical functions of the heart and lead to sudden cardiac death.

Continue reading “Sudden Cardiac Death in Young Athletes” →

HIGHLIGHTS FROM THE INTERNATIONAL HCM SUMMIT VI, PART III

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**Because so much HCM information was presented at the Summit, this is the third of multiple blog entries.  Stay tuned to HCMBeat for more highlights from the HCM Summit.  To see Part I of this series of highlights from the HCM Summit VI, click here and to see Part II of this series click here.**

The symposium was organized by long time HCM expert Dr. Barry Maron and his son, Dr. Martin Maron.  Both Marons are now affiliated with Tufts Medical Center’s Hypertrophic Cardiomyopathy Center.

What follows are summaries from selected talks presented at the meeting.  The presenter and their hospital affiliation are noted below, along with the topic of their presentation.  When possible, you may access the presenters’ slides via hyperlink by clicking on the name. (Note that not all presenters made their slides available).

Continue reading “HIGHLIGHTS FROM THE INTERNATIONAL HCM SUMMIT VI, PART III” →

Exercise Testing Important for HCM Patients

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According to a recent study by doctors at Tufts HCM Center in collaboration with colleagues in Italy, exercise testing is an invaluable tool in the assessment of hypertrophic cardiomyopathy patients.

In particular, two types of exercise testing are most valuable for HCM patients:

  • Exercise Echocardiogram:  These tests are valuable in determining whether a patient has obstruction.  It provides a physiological way to measure whether or not a patient has an obstructed left ventricular outflow tract and hence, may potentially be in need of an invasive procedure to treat the obstruction.  According to the article, approximately 1/3 of HCM patients have latent obstruction which may only be seen during or after exercise.  This obstruction is not always apparent from their resting echocardiogram.

and

  • Cardiopulmonary Exercise Testing:  These tests help determine functional capacity and provide a quantifiable indicator of heart failure symptoms.  This test  can identify patients in need of more aggressive treatment options, or who are potentially in need of transplant.  A particularly valuable piece of data from this test is the “VO2 max” score, which is a measure of the maximum rate of oxygen consumption during exercise which reflects the cardiorespiratory fitness level of a person.

*Editor’s note – Exercise testing was particularly informative and important in my own HCM treatment.  It was only after my doctors performed an exercise echo that the extent of my obstruction became apparent.  The symptoms I had been suffering appeared to be out of proportion to what was visualized on my resting echo.  The exercise echo helped my doctors understand the cause of my symptoms which made the next step, in my case a myectomy, much clearer.

You can read my full story here .

 

 

HIGHLIGHTS FROM THE INTERNATIONAL HCM SUMMIT VI, PART I

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**Because so much HCM information was presented at the Summit, this will be the first of multiple blog entries.  Stay tuned to HCMBeat for more highlights from the HCM Summit.  You will find Part II of this series by clicking here.**

The 6th International HCM Summit was held October 27, 28 and 29th in Boston, Massachusetts.  This symposium brings together HCM professionals from around the world who are there to learn about and discuss the latest developments in the treatment of HCM.

The symposium was organized by long time HCM expert Dr. Barry Maron and his son, Dr. Martin Maron.  Both Marons are now affiliated with Tufts Medical Center’s Hypertrophic Cardiomyopathy Center.

What follows are summaries from selected talks presented at the meeting.  The presenter and their hospital affiliation are noted below, along with the topic of their presentation.  When possible, you may access the presenters’ slides via hyperlink by clicking on the name. (Note that not all presenters made their slides available).

Continue reading “HIGHLIGHTS FROM THE INTERNATIONAL HCM SUMMIT VI, PART I” →

More is Needed for Women With HCM

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A recent study published in the European Heart Journal by doctors from the Mayo Clinic showed that women with hypertrophic cardiomyopathy (HCM) have a statistically reduced rate of survival as compared to men with HCM.

Continue reading “More is Needed for Women With HCM” →

A Risk Calculator for Sudden Death -Results of HCM-EVIDENCE Study

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The HCM Risk–SCD Score

In 2014, the European Society of Cardiology (ESC) introduced a numerical predictor (the “HCM Risk–SCD score”) to assist physicians in identifying those patients at highest risk for sudden cardiac death who would benefit from the implantation of a prophylactic implantable cardioverter-defibrillator.

Using an algorithm generated by the answers to a series of questions, the tool estimates the 5-year risk of sudden cardiac arrest for any particular patient.  You can find the tool online here.

Continue reading “A Risk Calculator for Sudden Death -Results of HCM-EVIDENCE Study” →

Multiple Mutations in HCM

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A recent paper by researchers in Australia, published this week in Circulation: Cardiovascular Genetics, found more than one rare HCM gene in 4% of patients in a 758 patient sample group.

The researchers found that those patients with multiple rare HCM genes tended to present with HCM at a younger age, were more likely to experience cardiac arrest or death from other causes, and were more likely to require a heart transplant.

In general, few patients have multiples of mutations commonly associated with HCM.  See this Canadian study from April of this year which found that multiple mutations were less prevalent and harmful than previously thought.

Encouraging Results for MyoKardia HCM Drug

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MyoKardia’s stock prices jumped today after their recent Stage II trial of the experimental drug mavacamten (formally known as MYK-461)  demonstrated a statistically significant reduction to left ventricular outflow tract gradients as well as improvement to aerobic capacity in patients with obstructive hypertrophic cardiomyopathy.  

Of the 10 patients who completed the study, 8 saw their gradient reduced to normal levels after 12 weeks on the drug.  The study also showed improvements in both peak oxygen consumption (peak VO2) and New York Heart Association classifications:  7 patients moved up one NYHA class while 2 patients improved by two classes.

The drug seemed to have mild to moderate side effects, though one patient was forced to drop out of the trial due to a recurrence of atrial fibrillation which necessitated discontinuation of mavacamten and a return to anti-arrythmic drugs which had been discontinued due to participation in the trial.

MyoKardia hopes to enroll between 200 and 250 patients in its next phase trial (Explorer HCM) which it plans to begin before the end of 2017.

MyoKardia also plans a clinical trial of mavacamten in non-obstructive HCM patients in the second half of 2017.

For more information on MyoKardia and  recent drugs being developed for HCM read these past blog entries:

MyoKardia HCM Drug Has Success in Cats

End of the Road for Eleclazine and Liberty HCM Study

HCM Drug Trial Advances to Next Round

Drug for Non-Obstructive HCM Moves Along