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” →

Greater Certainty in Genetic Testing Results at HCM Specialty Centers

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A recent study published by members of the SHaRe Cardiomyopathy Registry found that genetic test results for HCM are more definitive and helpful to patients when testing has been carried out at a high volume HCM center – especially a center that shares genetic data with other HCM centers. 

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Echo Measurement May Help to Guide HOCM Treatment Plan

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Doctors at the Cleveland Clinic recently published a study in the Journal of the American Heart Association suggesting that the measurement of left ventricular global longitudinal strain (LV-GLS), as determined by routine echocardiogram, may be helpful in determining treatment strategy for patients with obstructive HCM. 

In particular, the researchers found that a poor LV-GLS measurement seemed to correlate with a higher incidence of sudden cardiac arrest and appropriate ICD discharge.  Worsening LV‐GLS of less than -14% was associated with poorer prognosis, while myectomy seemed to improve LV‐GLS.

The researchers also found that a small number of HCM patients (including post-myectomy patients) with severely reduced LV‐GLS (worse than ≈ −7%) appeared to be in need of aggressive treatment, potentially including heart transplantation.

Chapter 3: MRI Safety for ICD & Pacemaker Patients

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Long awaited results of the MagnaSafe study regarding the safety of MRIs in patients with implantable devices were published in February.   The MagnaSafe study established a multi-center prospective registry for patients undergoing MRI scans despite their having an implanted device not deemed safe for MRI scanning by the FDA.

Continue reading “Chapter 3: MRI Safety for ICD & Pacemaker Patients” →

Atrial Fibrillation: Treatable HCM Complication

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A recent paper by doctors at Tufts University’s HCM Center found that transient episodes of atrial fibrillation (AF) are treatable and do not often progress to permanent AF.

This study found that AF was not a frequent cause of death by heart failure or sudden cardiac arrest.  However, the researchers identified AF as an important cause of stroke in HCM patients.  Therefore, they recommend a low threshold for starting HCM patients on anti-coagulants following an initial AF episode.

Researchers in this study analyzed statistics from 1558 HCM patients, 20% of whom experienced AF.  74% experienced only sporadic episodes, while 26% went on to develop permanent AF.

At the time of publication, 91% of the 277 of the patients included in the sample were still alive and between the ages 49 and 75 years old.

According to an accompanying editorial by Italian HCM expert Dr. Paolo Spirito, the outlook for HCM patients with atrial fibrillation has improved over the last twenty years due to significant advances in HCM treatment over that time period such as ICD implantation and myectomy, along with aggressive anti-coagulation for atrial fibrillation patients.

Spirito also noted that it is difficult to predict whether a given HCM patient will go on to develop permanent Afib after a single episode since many will not.  Additionally, permanent afib can be well tolerated when there is contemporaneous control of heart rate.  Therefore,  anti-arrhythmic medications, which can cause unpleasant side effects, may not be necessary for HCM patients with afib as long as anti-coagulation measures are taken.

 

 

 

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.

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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.

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Guest Blogger – When a Seizure is not a Seizure – by Wendy Borsari

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It’s strange to think that a chaotic arrhythmia in the heart might actually appear to be a seizure caused by something that has gone haywire in the brain, but with sudden cardiac arrest (SCA) this can sometimes happen.

This is the true story of what happened to my daughter.

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Could Your Fitbit Detect AFib?

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The same Fitbit you wear to count your steps may soon have an additional purpose:  it could detect atrial fibrillation.

Atrial fibrillation is an irregular heartbeat which could cause a stroke if not properly treated.  Yet, it often goes unnoticed by a person experiencing it.

According to this story in Time, Fitbit is  developing software which would enable its existing trackers to detect afib, and thus allow time for appropriate action before it is too late.

This technology could be of great benefit to heart patients.  Not only does the Fitbit encourage you to exercise, it might just save your life!

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.