2020 AHA/ACC HCM Diagnosis & Treatment Guidelines Released

The highly anticipated 2020 American Heart Association/American College of Cardiology Guidelines for the Diagnosis and Treatment of Patients with Hypertrophic Cardiomyopathy have been released.

This document, drafted with reference to published HCM literature, and with input from a committee of HCM experts with broad expertise, updates the prior version published in 2011.  It contains clinical practice guidelines for the broad spectrum of issues which may confront medical professionals as they approach the diagnosis and treatment of patients and families affected by hypertrophic cardiomyopathy.

Some key highlights and updates to the 2011 guidelines include:

  •  Shared Decision Making:  The 2020 guidelines emphasize the process of shared decision-making between patients and medical providers.  Medical providers should provide an overview of all testing and treatment options, and engage the patient in a complete discussion of all risks and benefits.  Most importantly, the patient is encouraged to share their own goals, values, and concerns which should inform the final decision.
  • Exercise is Beneficial:  These guidelines reflect a shift in thinking about exercise in HCM patients.  Prior guidelines suggested caution with regard to exercise.  Current research reflects the cardiovascular benefit to moderate exercise.  The guidelines also encourage the avoidance of dehydration, particularly in those patients who are obstructed, and to avoid exercise in extreme heat and humidity.  Participation in rigorous and physically demanding exercise is a topic for shared decision making and risk assessment between patient and medical professional, as in the first bullet-point above.
  • Referral to HCM Specialty Centers Encouraged:  Referral to specialized HCM Centers with expertise in treatment of the disease is important.  Expert advice is especially important where the treatment path is not clearly defined, or if invasive septal reduction procedures are under consideration.
  • Reconfirmation of Genetic Testing Results:  Genetic testing results should be performed by a cardiac genetic counselor with results being reconfirmed in follow-up visits every 2 – 3 years.  New genetic variants are constantly being identified, while older mutations are sometimes later found to be harmless.
  • Atrial Fibrillation:  HCM patients with atrial fibrillation, whether it is persistent or sporadic, have a heightened risk of stroke. Therefore, all HCM patients with AFib should receive anti-coagulant drugs without regard to traditional stroke risk scoring systems, i.e. the CHADS-VASc score.
  • Updated Risk Factor Calculation:  Risk factors for sudden cardiac death continue to be refined.  New markers, such as apical aneurysm, gadolinium enhancement on MRI and decreased left ventricular systolic function have been identified, while an older risk factor – abnormal blood pressure response during exercise – is no longer considered to be a risk factor.
  • Heart Failure Treatment:  Traditional heart failure treatments should be used in patients with an ejection fraction reduced below 50%, without obstruction (which should always be addressed first with septal reduction methods). These treatments include HF medical therapy, cardiac resynchronization therapy, left ventricular assist device or even heart transplantation.

The top ten take home messages can be found here.

And you can find the entire 2020 HCM Guidelines here.

AHA Seeking Patient Input for New HCM Initiative

In November at the American Heart Association’s Scientific Sessions held in Philadelphia, AHA announced that it would be starting a three-year initiative focused on hypertrophic cardiomyopathy, also known as HCM.  The initiative is sponsored by MyoKardia, a San Francisco based company that is currently in clinical trials for mavacamten, the first drug specifically intended to treat HCM.

Last week, Cynthia Waldman of HCMBeat had the opportunity to speak with Amy Schmitz, AHA’s National Corporate Relations Director and Alexson Calahan, a Communication Manager for AHA.

What follows is a summary of their conversation about the forthcoming HCM initiative that has been edited for clarity.

Continue reading “AHA Seeking Patient Input for New HCM Initiative”

ESC Risk Assessment Tool Comes Up Short in Study

A recent study published in the American Journal of Cardiology found that the standards propounded by the American College of Cardiology Foundation with the American Heart Association (ACC/AHA) were superior in predicting which patients would benefit from an implantable cardiac defibrillator (ICD) compared to the calculator set forth by the European Society of Cardiology (ESC).  According to the study, the use of the ESC tool will result in more high-risk patients going unprotected against sudden death.

Specifically, the study found that out of a group of 288 HCM patients, 14 who experienced aborted sudden cardiac arrest (or 43%) would not have qualified for an ICD under the ESC risk model compared to 7% of patients under the ACC/AHA guidelines.

The ACC/AHA guidelines are:

ICD Guidelines rev.

A companion editorial by Dr. Andreas S. Barth pointed out the shortcomings of both models, and reaffirmed the necessity for shared decision making between physicians and patients.  Dr. Barth also expressed hope that a more accurate predictive model will evolve, though he acknowledged the impossibility of designing a model which could predict future events with certainty.



**Because so much HCM information was presented at the Summit, this is the second 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.**

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).


Lindsay Davis: Using Her Big Heart to Help Others With HCM

This article, published in this week’s Women’s Health magazine, features the former ballerina and beauty queen turned vocal patient activist. These days, Lindsay has focused her efforts on saving lives from sudden cardiac arrest.  Lindsay’s efforts in the state of Ohio have resulted in proposed legislation to identify student athletes at risk of sudden cardiac arrest, while her partnership with the American Heart Association is steadily making CPR and AED training a graduation requirement in high schools across the nation.

Watch for more life-saving advocacy from Lindsay in the future.  She is clearly much more than another pretty face!

Updated to include a video of Lindsay discussing her implantation with a S-ICD device.