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Hypertrophic cardiomyopathy (HCM) is a heart muscle disease that affects an estimated 1 in 500 adults in the general population. Many people with HCM live healthy lives, but its association with premature death in the young and heart failure in the middle aged raises its profile in the mind of the general public and make it a common cause for concern among healthcare professionals.
To assist practitioners and patients manage the condition, The European Society of Cardiology (ESC) has recently published a new clinical practice guideline on HCM that replaces an earlier document produced jointly with the American College of Cardiology in 2003.1 From the outset, all members of the ESC writing committee agreed to three guiding principles while preparing their recommendations. The first was that guidance should be practical and firmly rooted in real-life clinical practice; second, there was a determination that recommendations should, where humanly possible, innovate and not slavishly reproduce previous statements; and finally–and perhaps most challenging of all—recommendations should, whenever possible, be based on the best scientific evidence. At first glance, the absence of large randomised clinical trials and the relative paucity of large cohort studies in this area of medicine might seem to render such ideals futile, but the experts on the committee were able to achieve consensus on an approach to diagnosis and management that shifts the emphasis towards multidisciplinary working and individualisation of patient care. Some of the most important areas covered in the guideline are discussed below.
HCM is defined by a simple measurement of LV wall thickness on cardiac imaging. While this has been shown to be a robust and reproducible approach to clinical classification, it fails to describe the spectrum of genetic and non-genetic disorders than can present with LV thickening. One approach to rationalising this complexity is to …