Recent ESC Guidelines to Identify Hypertrophic Cardiomyopathy Patients at High Risk for Sudden Death Are Unreliable

U.S. guidelines more reliable, according to new study in The American Journal of Cardiology

Philadelphia, PA, September 21, 2015

Hypertrophic cardiomyopathy (HCM), a genetic form of heart disease with thick heart walls, is the most common cause of sudden death in patients under 50. While only a minority of patients with HCM are at risk, the implantable cardioverter-defibrillator (ICD) can effectively prevent sudden death in those patients. Recently, the European Society of Cardiology (ESC) published new guidelines advancing an equation as the best way to determine which HCM patients should receive an ICD. However, a study conducted by U.S. and Canadian investigators challenges the ESC Guidelines. Their research found that the ESC sudden death risk score method did not perform effectively in reliably identifying the high-risk patients who need ICDs for the prevention of sudden death.

“Our data show that in HCM, strategies to identify implantable defibrillator candidates based on a rigid mathematical and statistical formula, as promoted by ESC, are unreliable for identifying high-risk patients who could benefit from lifesaving therapy with prophylactic ICDs,” stated lead investigator Barry J. Maron, MD, of the Hypertrophic Cardiomyopathy Center, Minneapolis Heart Institute Foundation, Minneapolis, Minnesota.

The method for identifying high-risk patients is called risk stratification and it is predicated on the results of certain tests (such as ambulatory ECG monitoring, echocardiogram, and stress testing), and personal and family histories. Syncope (fainting), extreme thickening of the left ventricle, and ventricular tachycardia, as well as extensive scarring of the wall detectable by MRI with contrast can be risk markers. This assessment method and risk markers are a prominent part of the U.S. Guidelines on management of HCM and identification of patients at unacceptably high-risk, and have been well accepted as such.

In evaluating the ESC Guidelines, researchers used the case records of more than 1600 patients and found that for the 35 patients who died suddenly, only 4 (11%) would have been considered high risk by ESC criteria. For the 41 patients who had a life-saving shock from a defibrillator, 27 (59%) would not have even received a defibrillator under the ESC Guidelines and theoretically would have been at risk for sudden death.

“We have retrospectively tested the mathematical ESC risk model against a large independent external cohort of individual patients with HCM,” added Dr. Maron. “We found the ESC prognostic score was unreliable in identifying most high-risk patients previously managed in accord with risk stratification practices established by U.S. HCM consensus guidelines.”


Notes for Editors
“Independent Assessment of the European Society of Cardiology Sudden Death Risk Model for Hypertrophic Cardiomyopathy,” by Barry J. Maron, MD, Susan A. Casey, RN, Raymond H. Chan, MD, MPH, Ross F. Garberich, MS, Ethan J. Rowin, MD, and Martin S. Maron, MD.  (DOI: It appears in The American Journal of Cardiology, Volume 116, Issue 5 (1 September 2015)published by Elsevier.

Full text of this article is available to credentialed journalists upon request. Contact Joan Anuels, Associate Publisher, Elsevier, at +31 20 485 2461 or or to obtain copies. Journalists wishing to interview Barry J. Maron, MD, may contact him directly at +1 612 863 3996 or

About The American Journal of Cardiology
The American Journal of Cardiology® (AJC) is an independent journal designed for cardiovascular disease specialists and internists throughout the world with a subspecialty in cardiology. This peer-reviewed journal publishes original articles that focus on the practical, clinical approach to the diagnosis and treatment of cardiovascular disease. Features report on systemic hypertension, methodology, drugs, pacing, arrhythmia, preventive cardiology, congestive heart failure, valvular heart disease, congenital heart disease, and cardiomyopathy. Also included are editorials, readers' comments, and symposia.

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