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.

You can find 30 slides compiled by a group of HCM physicians which summarize these guidelines here.

September 2021 Article in The Lancet by Dr. Steve Ommen and Dr. Chris Semsarian:               Hypertrophic Cardiomyopathy: a practical approach to guideline directed management

And you can find the entire 2020 HCM Guidelines here.