Share Registry Study Focuses on So-Called “End Stage” HCM

Researchers from the Sarcomere Human Cardiomyopathy Registry (or the acronym “SHaRe” for short) have again joined forces to look at a subset of hypertrophic cardiomyopathy (HCM) patients who have what is known as “end stage” HCM.  This term, which is a bit of a misnomer, is used to describe patients who experience left ventricular systolic dysfunction (LVSD) which occurs when the left ventricular ejection fraction (LVEF) is less than 50%.

According to the study published last week in the journal Circulation, of a total of 6,793 HCM patients included in the study, 553 or 8% fell into this category.   The paper goes on to highlight some important observations about patients that fall into this category which are highlighted below.

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Atrial Fibrillation: Treatable HCM Complication

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.

 

 

 

One Size Does NOT Fit All: Treatments Differ by Stage in HCM

There are distinct stages of HCM and treatments will vary according to the stage. Therefore, it is important, according to a recent article in the Netherlands Heart Journal, for patients to seek treatment from teams experienced in the the treatment of HCM.  These professionals are able to recognize transitions in the course of the disease as they occur, and then implement necessary changes in treatment.

In this thorough overview of HCM and its treatment, Dr. Iacopo Olivotto and his colleagues in the Netherlands observe that HCM is seen infrequently by community cardiologists (as compared to more common heart conditions like coronary artery disease). Hence, one of the major difficulties in HCM practice has been identification of patients at highest risk.

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