To date, risk assessment in patients with hypertrophic cardiomyopathy (HCM) has largely focused on whether there is a need for an implantable defibrillator by evaluating an individual patient’s short term risk of sudden death, but has not looked at the long term risks of heart failure and other adverse outcomes over time.

A recent retrospective study by European HCM specialists asked the question of whether the addition of advanced imaging could assist in pinpointing patients at high risk of heart failure and/or other serious HCM complications over time. 

This study found that late gadolinium enhancement (LGE) as seen on cardiac MRI, as well as patients who present with New York Heart Class II or greater and who also have non-sustained ventricular tachycardia, are important variables to consider when assessing the long term risk from HCM.  

Additional factors which were associated with increased risk were:

• Increased levels of BNP or NT-proPNP
• Positive genotype
• Apical aneurysm
• impaired global longitudinal strain as seen on cardiac imaging
• left ventricular ejection fraction less than 50%
• atrial fibrillation

The authors of this study suggest that current risk assessment tools be updated to incorporate data from the latest imaging technology. This would improve focus on the long term HCM picture for each patient. The authors suggest that future risk assessment tools combine imaging findings with clinical features and then the addition of artificial intelligence to synthesize all findings together. In combination, these modalities will lead to the development of machine learning algorithms for risk assessment.

An editorial comment to this paper by Dr. Steve Ommen of Mayo Clinic responded to this paper by saying that knowledge of the presence or absence of risk factors is not likely to change management of HCM patients until we are able to develop specific interventions to prevent the progression to heart failure or atrial fibrillation.

Dr. Ommen concluded his comment with this advice:

“In the end, caring for patients with HCM includes teaching the patients about HCM and the fact that adverse outcomes are not predestined for many patients. Normal longevity and quality of life are completely compatible  with HCM.”