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.
This article, by Drs. Julio Panza and Srihari Naidu of New York’s Westchester Medical Center, describes early efforts to diagnose, categorize and treat hypertrophic cardiomyopathy, while explaining how these methods have evolved over time. A very interesting and informative read.
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.
- 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 .
Since the early 1960s, surgical septal reduction, also known as septal myectomy, has been used as a therapy for the treatment of obstructive hypertrophic cardiomyopathy. Pioneered at the National Institute of Health by cardiac surgeon Dr. Glenn Morrow, himself a HCM patient, septal myectomy has become a mainstay of the HCM treatment arsenal.
An alternative to septal myectomy, alcohol septal ablation (ASA), was first performed by Ulrich Sigwart in the United Kingdom at the Royal Brompton Hospital in London in 1994.
For many years, the indications for ASA procedures has been limited to older patients with obstructive HCM who were not otherwise healthy enough to undergo open heart surgery. However, some doctors are now advocating to expand the indications for ASA to include symptomatic younger patients.
(For more information about myectomy and ASA, click here and scroll to bottom of page).
Continue reading “Should Alcohol Septal Ablation Be Considered for Younger Patients?”
Editor’s note: This is our first interview feature on HCMBeat. In the future, we hope to feature more interviews with other HCM researchers who have published articles of interest to the HCM community.
By now, you have probably already heard the buzz about RESET- HCM – a study about the effects of exercise on HCM patients conducted by Dr. Sara Saberi and Dr. Sharlene Day at the Hypertrophic Cardiomyopathy Clinic of the University of Michigan’s Frankel Cardiovascular Center in collaboration with Dr. Matthew Wheeler and Dr. Euan Ashley of Stanford’s HCM Center. The findings were presented at the American College of Cardiology Conference on March 17, 2017 held in Washington D.C. and were the subject of this feature on HCMBeat.
Recently, Cynthia Waldman of HCMBeat had the opportunity to sit down with Drs. Saberi and Day for a detailed conversation (over Skype) about the study. What follows is a transcript of their conversation (which has been edited for readability).
Continue reading “RESET-HCM: Rethinking Exercise for HCM Patients – Interview with Dr. Sara Saberi and Dr. Sharlene Day”
Eleclazine: The Liberty HCM Trial
It appears to be the end of the road for the Gilead drug eleclazine, a late sodium channel inhibitor previously known as GS-6615. Eleclazine, with properties similar to the anti-angina drug ranolazine (which was approved by the FDA in 2006), was the subject of a recently terminated HCM clinical trial known as Liberty-HCM. The HCM eleclazine study focused on whether the drug would improve symptoms and exercise capacity in patients with HCM by increasing their peak oxygen uptake, resulting in improved VO2 max readings on exercise testing. The HCM study began enrolling patients in February 2015. Data collection had been scheduled to continue through June 2017. Continue reading “End of the Road for Eleclazine and Liberty HCM Study”
Pippa Middleton, who came to the public’s attention during the wedding of her sister Katherine to Prince William, has recently dedicated her efforts toward raising money for HCM genetic testing and research. Middleton’s efforts are in honor of her late friend Miles Frost, who was lost to sudden cardiac arrest due to HCM in 2015. Frost’s father, British journalist and media personality David Frost, died from HCM just two years earlier in 2013, but this information was never communicated to the family.
The Miles Frost Fund, a partnership with the British Heart Foundation , helps families who have lost a member to a sudden death obtain genetic testing in order to learn if other family members are similarly affected. The Frost Fund also funds research by U.K.researchers working towards finding a cure for HCM.
A study published today by Dr. Carolyn Ho, of Boston’s Brigham and Women’s Hospital, and colleagues, including Australia’s Christopher Semsarian, found that there are several factors which appear to stand out in young people who later go on to develop HCM.
The children/adolescents/young adults who participated in the study all carried at least one gene associated with HCM, and were members of families with strong histories of HCM. None of the 38 young people had clinical manifestations of HCM at the beginning of the study period, while 4 went on to develop HCM by the end of the study. In collecting the data analyzed in the study, the test results of the 4 individuals with HCM were compared to the 34 individuals that did not have HCM at the end of the study.
The factors associated with the development of overt HCM, as identified by the researchers, were: abnormal left ventricular relaxation and higher ejection fraction, EKG changes, longer mitral valve leaflets, higher NT-proBNP concentrations and troponin values.
In the conclusion, the authors acknowledged the need for further research and investigation in order to better understand the natural course and evolution of the disease.
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”