Tag: obstruction

Myectomy: Still the Gold Standard for HOCM

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An expert panel comprised of many of the world’s top HCM experts recently published a retrospective analysis in The American Journal of Cardiology which looked at septal myectomy over the last 60 years of practice.

 Beginning with the introduction of the procedure at the National Institute of Health in the early 1960s, this paper surveys the history of the procedure until the present day.  The conclusion of the paper is that myectomy remains the best treatment for patients with obstructive hypertrophic cardiomyopathy.

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Increased COVID Risk for HCM Patients

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A recent retrospective study of HCM patients with COVID-19 concluded that HCM patients are at increased risk of hospitalization and death from a COVID-19 infection. 

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Experts Put Mavacamten in Perspective

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An article published today in Circulation by HCM experts Dr. Steve Ommen of Mayo Clinic and Dr. Martin Maron of Tufts Medical Center, discusses the prospective use of mavacamten as a treatment for obstructive hypertrophic cardiomyopathy. The doctors conclude that while mavacamten (assuming that it is FDA approved in early 2022) will have its place in the HCM tool kit, it should not replace septal reduction therapy for severe HOCM.

In particular, the article points out that the EXPLORER-HCM study showed modest improvements in symptoms and functional capacity (peak V02), comparable to those seen in the RESET-HCM study, which highlighted the ability of regular exercise to improve functional status in HCM.  

The article notes that there has not yet been a study directly comparing mavacamten with septal reduction therapies such as septal myectomy and alcohol septal reduction. The VALOR-HCM study, which is currently recruiting, will look at these therapies compared head-to-head.  It is noteworthy that the majority of patients in the EXPLORER trial had Class II heart failure and were not the more severely compromised Class III and IV patients most likely to benefit from myectomy or alcohol septal ablation.

This article compared historical myectomy data against the findings from EXPLORER, concluding that septal myectomy produces a better result for patients, with gradients abolished in more than 95% of patients compared to only 50% of patients with mavacamten.  And, the article points out that 25% of the patients in the EXPLORER trial continued to have left ventricular outflow tract gradients greater than or equal to 50mmHg, which still qualified them for septal reduction therapy.

Maron and Ommen’s take-home message is that mavacamten will be a welcome addition to the arsenal of HCM drugs and is potentially suitable for patients who do not have severe symptoms, who do not have access to septal reduction at a HCM specialty center, or who wish to avoid more invasive therapies.  It also may be used in the same way as disopyramide, to defer surgery by improving symptoms to a tolerable level.

Lastly, this article points out that there is a need for longer term follow up to study the effects of cardiac remodeling caused by mavacamten.

While it is wonderful to have options, it is important that patients and their medical team consider all available information, including potential benefit and risk, before moving forward with medical therapy. 

HCM Treatment: The View from OHSU

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If you are looking for a good survey of current practices in the treatment of HCM, a recent article published in the journal Structural Heart by Dr. Ahmad Masri and the team at Oregon Health and Sciences University (OHSU) provides an informative overview of thirty controversies and considerations in the treatment of HCM. This article explains in some detail how the doctors at this HCM Center approach these situations. 

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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 .

 

 

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

MyoKardia HCM Drug Has Success in Cats

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MyoKardia’s experimental drug MYK-461, currently in Stage 2 trials for humans, has now been shown to eliminate left ventricular obstruction in five cats with HCM. It has already been shown to inhibit traits of HCM in mice.

Addressing these findings, Associate Professor Joshua Stern, chief of the Cardiology Service at the University of California, Davis, veterinary hospital, stated:

“There has been little to no progress in advancing the treatment of HCM in humans or animals for many years,” Stern said. “This study brings new hope for cats and people.”

Based on these positive results, U.C.Davis is hoping to conduct a clinical trial of MYK-461 to determine whether it could become the standard of care for cats with HCM.

The full text of the article published in Plos One can be found here.

Guest Blogger – Surgical Myectomy: A Twice in a Lifetime Experience – By Jill Celeste

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I have had the joy of being a Registered Nurse for over 40 years. I was born wanting to be a nurse and started bandaging teddy bears at the age of three. By the age of 5, I was creating “medicines” by spinning blades of grass mixed with clover flowers in the front wheel well of an upside down tricycle.

As I got older, I moved on to be a Candy Striper and a Nurse’s Aide, and then I went on to get my degree as a RN, a BSN, and MSN and became a teacher, administrator, and researcher. All of this cannot REALLY prepare you for; “Being on the other side of the bed” which is what happens when a health care professional who is used to caring for patients becomes a patient themselves. Continue reading “Guest Blogger – Surgical Myectomy: A Twice in a Lifetime Experience – By Jill Celeste” →