A recent article by several noted HCM experts shares their recommendations for the screening and evaluation of patients with HCM using several scenarios commonly encountered in clinical practice. Notably, this paper makes several recommendations which differ from the 2020 ACC/AHA Guidelines for the Diagnosis and Treatment of Patients with Hypertrophic Cardiomyopathy (2020 Guidelines).
In particular, the article recommends:
- Cardiac MRI with contrast upon initial evaluation, and every 3 – 5 years thereafter. (The 2020 Guidelines recommend Cardiac MRI in certain situations, but not for all patients)
- Genetic testing only in cases where a pathogenic or likely pathogenic mutation has been identified in a family member and in limited other circumstances (the 2020 Guidelines recommends genetic testing for all as part of the initial assessment)
- Screening of family members 12 or older only (the 2020 Guidelines recommend screening of children of any age when genetic HCM is diagnosed in a family member and no later than puberty, in any event)
- That HCM patients be disqualified from competitive sports. (The 2020 guidelines instead recommend a shared decision making approach).
Continue reading “Recommendations for HCM Depart from 2020 ACC/AHA Guidelines”
The July edition of the the American College of Cardiology’s magazine features a cover story about HCM.
The article surveys the history of HCM and then moves through the evolution into contemporary treatments. The article also contains a summary of important topics from the new 2020 AHA/ACC Guidelines and highlights EXPLORER-HCM, the recent groundbreaking clinical trial of mavacamten.
Check it out when you can.
Watch the video or read the transcript of Dr. Steve Ommen’s recent interview on Medscape.
In this interview, he discusses the recent AHA/ACC treatment guidelines for hypertrophic cardiomyopathy (HCM), his thoughts about the new HCM drug mavacamten, and the importance of collaboration between your local care team and the team at a HCM specialty center.
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.
Continue reading “2020 AHA/ACC HCM Diagnosis & Treatment Guidelines Released – Updated With New Links”
The European Society of Cardiology recently published new guidelines for exercise for patients with HCM.
These guidelines offer more flexibility for HCM patients that past guidelines, stating that exercise may be permissible for HCM patients, but that it depends on individual situations. The focus should be on the shared decision process between patient and doctor to determine whether exercise may be appropriate for each individual patient. Additionally, annual follow up for adults, with more frequent follow up for adolescents and young adults is also recommended.
Section 5.5.1 of the guidelines focuses on Hypertrophic Cardiomyopathy. These guidelines state:
- The risk of sudden cardiac death or arrest (SCD or SCA) during exercise is likely lower than previously thought and therefore, a total ban on exercise is not warranted.
- Exercise for physical and psychological well-being should be encouraged, following a careful baseline assessment of patients with HCM which should include:
- Personal and family history
- Risk Factors for SCD/SCA
- EKG at rest and during exercise
- Echocardiogram/Degree of Obstruction
- Degree of late gadolinium enhancement indicative of cardiac fibrosis as seen on cardiac MRI
- Exercise testing and blood pressure response to exercise
- ESC Score of HCM Risk
A recent study by doctors at Toronto’s Hospital for Sick Children suggests that current screening guidelines for children from HCM families are inadequate and should instead recommend earlier screening exams. In the U.S., screening begins at age 12 pursuant to American College of Cardiology (ACC)/American Heart Association (AHA) guidelines. In Europe, screening begins at age 10 pursuant to the European Society of Cardiology (ESC) guidelines.
Continue reading “Should Children from HCM Families be Screened Earlier?”
A recent study published in Circulation suggests that clinical testing of kids who are first degree family members of HCM patients (i.e. siblings and children of those who have already been diagnosed with HCM) could be improved by starting testing at a younger age. And, genetic testing should further improve diagnosis and treatment for this group.
Continue reading “When Do You Screen Your Kids For HCM?”
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:
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.
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?”