Category: News

How to Improve Alcohol Septal Ablation

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Alcohol septal ablations (ASA) have been available to HCM patients as a treatment option for the last 20 years.  While the procedure has been the subject of great controversy, some physicians have recently advocated for expanded indications of the ASA procedure.

An editorial in this week’s Journal of the American College of Cardiology from the Netherlands argues that the safety of ASA has been firmly established because mortality rates from ASA have been shown to be comparable to those from septal myectomy.  The Dutch doctors maintain that past concern about ventricular arrhythmia resulting from the scar left by the ablation have not born out.

Making ASA Safer

Now, they argue, the focus should shift from justifying the procedure toward perfecting the procedure.  In particular, the need for additional or repeat procedures must be reduced.  Additional procedures have been necessary due to incomplete resolution of obstruction and/or the need for pacemaker implantation due to heart block, neither of which are a common consequence following myectomy.  1 in 10 patients require a pacemaker following ASA, while only 1 in 25 require one following a myectomy. 1 in 13 patients require a subsequent intervention after ASA (either another ASA or a myectomy), which is 15 times the rate of re-intervention after a myectomy.

The researchers’ suggestions for improvement include:  1) performing ASA only in hypertrophic cardiomyopathy centers of excellence that perform high volumes of the procedure; 2) improving patient selection through the use of a multi-disciplinary team which includes a cardiologist specializing in imaging, a cardiac surgeon, and an interventional cardiologist; 3) using 3D myocardial contrast echocardiography in order to select the best vessels; and 4) use of a small targeted amount of alcohol.

Impact of 3D Myocardial Contrast Echocardiography

In particular, the researchers explain that 3 dimensional myocardial contrast echocardiography (MCE) has proven to be a helpful tool in selection of the appropriate septal perforator.  The use of MCE has resulted in a change in strategy in 15% to 20% of cases:  either by a change in which blood vessel is selected for the alcohol or by prompting the immediate discontinuation of a procedure if the MCE shows that other parts of the heart could be affected.  MCE has also improved the success rate of ASA, while allowing for a more compact scar.

Counterpoint Editorial Advocates National Registry to Quantify Results

An accompanying editorial by Dr. Paul Sorajja from Minneapolis Heart Institute argues that we do not have the data necessary to reconcile the differences in outcome between myectomy and ASA.  In order to better understand the long-term potential and risks of ASA, mandatory reporting should be required.  He points out that this is what is done in other multidisciplinary transcatheter-based therapies, e.g. transcatheter aortic valve replacement for the treatment of aortic stenosis and transcatheter repair of mitral regurgitation with MitraClip.  These procedures require: 1) the use of multidisciplinary teams; 2) participation in a national registry (i.e., The Society of Thoracic Surgeons/American College of Cardiology Transcatheter Valve Therapy Registry);  and 3) comprehensive reporting of procedural and 1-year outcomes.

Therefore, Dr. Sorajja proposes a national registry created that includes the following information:

  • risk factors for sudden cardiac death
  • LVOT gradients
  • Standardized definitions for procedure success

Pippa Middleton’s 1st Post-Wedding Appearance at HCM Fundraiser

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Pippa Middleton, (sister of Dutchess Katherine of Cambridge and sister-in-law of Prince William) and her brand new husband, James Matthews,  made their first public appearance as a married couple at a fundraiser for the Miles Frost Fund.  The Frost Fund, founded in memory of Middleton’s late friend Miles Frost who died from undiagnosed HCM, raises money for genetic testing of family members who have lost a close relative to sudden cardiac arrest, as well as funding HCM research.

Pippa, just back from her honeymoon to French Polynesia and Australia, looked refreshed in her white jumper and carried a red heart-shaped clutch to emphasize the purpose of the occasion.

The fundraiser also attracted other royals such as Princess Eugenie and Sarah Ferguson, Duchess of York.

Here is a video of Wilfred Frost, brother of Miles Frost and son of Sir David Frost, talking about his father and brother’s deaths and the formation of the charity.

Non-Genetic HCM – Reduced Screening?

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According to researchers at the University of Michigan, family members of HCM patients who have tested negative for genes associated with HCM and without a family history of HCM will usually be found to be free of HCM during routine family screening.  And, in accordance a recent Australian study,  relatives of these patients may be able to benefit from less rigorous screening protocols.

Patients with a known sarcomere mutation appear to have a different clinical profile, according to the researchers:  they have more hypertrophy; they are younger when diagnosed; they have a higher risk for adverse events;  and they are more likely to have a family history for the condition.

In contrast, when the initial diagnosis is made in a patient who is 50 or older with no known genetic mutations, a negative family history, and sigmoidal septal pattern hypertrophy, reduced family screenings may be appropriate and less burdensome.  In addition, hypertension, large family size with no other affected family members,  less severe hypertrophy, and lack of life-threatening complications related to HCM may provide additional comfort to families of newly diagnosed HCM patients.

The reduced protocol would consist of a single screening of adult family members, with the caveat that if and when any additional family additional member is found to have HCM, a more traditional screening protocol be instituted.

(Note that standard screening guidelines recommend screening of all first-degree family members of patients beginning with adolescence, repeated annually through the end of adolescent growth, and repeated every 3 – 5 years for life.)

 

Outlook Positive for Most HCM Patients

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According to an editorial published in yesterday’s issue of Circulation by Drs. Barry and Martin Maron together with Dr. Ethan Rowin, the outlook for most HCM patients is much rosier than previously thought.

According to current estimates, which take into account contemporary disease treatments and risk management strategies, the risk of death from HCM is only .5% per year or 2.5% over 5 years, which is similar to the degree of risk in the general non-HCM population.

Hence, according to the editorial, HCM patients should be reassured about their long term prognosis.

 

Could a Tarantula Help to Unravel the Mysteries of HCM?

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Researchers from around the globe have joined together to study an unlikely subject in order to understand the genetics of HCM according to a paper published today in the journal  eLIFE.

Dr. Christine Seidman, a cardiologist from Harvard Medical School, Dr. James Ware  a geneticist from the MRC London Institute of Medical Sciences at Imperial College London, and Dr. Raúl Padrón, a structural biologist at the Venezuelan Institute for Scientific Research, have joined forces in order to study the tarantula.

The reason for their focus on the tarantula is because the proteins comprising the muscles inside the furry spider are actually very similar to proteins inside the human heart.

Dr. Seidman, who had taken note of Dr. Padrón’s work with spiders, sought him out at a meeting to discuss the similarity of heart proteins to those in tarantula muscles and asked him whether they might collaborate.

By studying the way that the spider proteins interact with one another, the scientists hope that they will gain further insight into whether and how certain genes cause different types of hereditary cardiomyopathy, including hypertrophic and dilated.

I hope that they find the answers soon, before any tarantulas escape from their lab!

EEEEEEEKKKK!

 

ICD May Not Bar Competitive Sports

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It may be possible for some athletes to continue playing competitive sports despite having an implantable defibrillator according to a recent study published in this week’s Circulation.

The study followed 440 athletes with ICDs who participated in organized sports over a 4 year period. Diagnoses included HCM, Long QT Syndrome, and arrhythmogenic right ventricular cardiomyopathy (ARVC) .  Common sports for the patient-athletes were running, basketball and soccer.

Over the period of the study, 121 of the patient-athletes received a total of 184 shocks:   7% while participating in competition or practice, 5% during other physical activities, and 6% while resting.  No deaths were reported over the approximately 44 months study. “Even though some people did receive shocks while they were participating in sports, no harm came to patients,” said lead author Dr. Rachel Lampert, a professor of internal medicine at Yale School of Medicine.

The study did note that patients with ARVC who engaged in sports were more likely to experience life-threatening ventricular arrhythmias requiring therapy, and, were more likely to receive repeated shocks from their devices.  Hence, competitive sports may be inadvisable for ARVC patients.

For more details about this study, see this story from Health Day News and from Yale News.

 

 

 

Aspirin May Cause More Harm than Good in Afib

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According to new research presented at last week’s meeting of the Heart Rhythm Society, aspirin is not effective in preventing strokes in patients with atrial fibrillation, and in some instances may actually do more harm than good.

Note that previous studies have demonstrated that aspirin is not effective in preventing strokes from Afib.

In fact, the study, led by Dr. Jared Bunch from Intermountain Healthcare system, Salt Lake City, UT, found that patients who were prescribed aspirin following catheter ablation procedures to treat atrial fibrillation were significantly more likely to suffer gastrointestinal or genitourinary bleeding than those who took other anticoagulants like warfarin, or those who received no treatment at all.

For more, see these articles from  Medical News Today  and Science Times (with link to video).

 

Cardiac Volume Reduced in Patients with HCM Gene

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Researchers in Norway have demonstrated that patients who carry a HCM gene show reduced cardiac volume when compared to healthy individuals.  Patients with overt HCM show even further reduction to their cardiac volume than those who merely carry the gene.

Although the gene positive individuals lacked the characteristic left ventricular wall thickening of HCM, diastolic and systolic volumes were reduced when compared to healthy individuals. Hence, the researchers concluded that a person who is gene positive for the disease may show reduced volume before developing hypertrophy.

The study included 180 patients with left ventricular hypertrophy, 100 patients who carried the HCM gene but did not show signs of left ventricular hypertrophy, and 80 healthy individuals.

The researchers theorize that early changes in HCM result from the gradual stiffening of the left ventricle, which contributes to filling changes before anatomical thickening is apparent. These changes will likely worsen as the disease progresses.

The researchers suggest future long term studies of gene positive individuals with small cardiac volume who show signs of diastolic and systolic dysfunction. These patients, they suggest, are the most likely to go on to develop HCM.

 For more on early signs of HCM click here.

Safety of MRIs With Abandoned Leads

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Two posters presented at this weekend’s Heart Rhythm Society meeting in Chicago show that patients who have abandoned pacemaker or ICD leads may safely undergo MRI exams.

These posters follow the earlier MagnaSafe study which demonstrated the safety of MRI for patients with pacemakers and ICDs, but which excluded patients with abandoned leads from the findings.

The first, by researchers at Mayo Clinic, included 57 patients with 63 abandoned leads who underwent 70 MRI exams in a 1.5 Tesla machine.  The authors saw no clinical problems and no device malfunction following the scans.

The researchers also monitored blood troponin levels in 35 of the patients following the scans and did not see any elevation which could indicate distress to the heart from the scan.

The second poster, from the University of Pennsylvania, involved 24 patients with abandoned leads who underwent 34 MRI exams.  The results of this study also failed to demonstrate any clinical problems or patient discomfort resulting from the MRI scans.

With any luck, everyone will soon be able to obtain an MRI and will not be denied due to any kind of implantable cardiac device.

For an account of my personal experiences seeking MRI with an ICD, read this.

Can Your Apple Watch Spot Afib?

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According to preliminary data presented this week at the Heart Rhythm Society’s annual meeting, the Apple watch. together with an app called Cardiogram, spots atrial fibrillation with 97% accuracy. 

Start-up tech company Cardiogram paired up with electrophysiologists at the University of California, San Francisco to try out the technology on patients awaiting cardioversion for atrial fibrillation.  51 patients at UCSF agreed to wear Apple Watches during their cardioversion procedures.

Heart rate samples were obtained before the procedure, when the patient was in atrial fibrillation, and again afterward when heart rhythm had been restored to normal. The researchers found that the Apple Watches were able to detect afib 97% of the time.

The Cardiogram and UCSF teams hope to publish their findings in a peer-reviewed journal while Cardiogram hopes it can make this information useful to consumers.  One possibility would be to have the watch send a notification to the wearer that s/he appears to be in afib should contact her/his care provider immediately.

If you are interested in participating in this research, click here.

Read more about it at TechCrunch, BuzzFeed, Apple Insider and CNET,