Preventing sudden death in hypertrophic cardiomyopathy: ESC guidelines (HCM-EVIDENCE)

August 29, 2017

Barcelona, Spain - 29 Aug 2017: A large study conducted across North America, Europe, the Middle East and Asia has validated the ESC recommendations for predicting and preventing sudden cardiac death (SCD) in patients with hypertrophic cardiomyopathy (HCM).

The HCM-EVIDENCE study, presented at ESC Congress 20171, tested the ESC's recommendations in a diverse cohort of patients, said study investigator Constantinos O'Mahony, MRCP(UK).

"Since 2014, the ESC has recommended using a special risk prediction tool called the HCM Risk-SCD to identify which patients are at highest risk for sudden death and would benefit most from having a prophylactic implantable cardioverter defibrillator," explained Dr O'Mahony, from St. Bartholomew's Centre for Inherited Cardiovascular Disease, St Bartholomew's Hospital, and the University College London Centre for Heart Muscle Disease, Institute of Cardiovascular Science, London.

"But, whenever a risk model is created, there are concerns about how generalisable it is outside the original study population. Since we developed the HCM Risk-SCD tool using patients treated exclusively in Europe, it was important for us to demonstrate its applicability in other healthcare systems with different medical expertise and potentially different patterns of disease."

ESC guidelines recommend using the HCM Risk-SCD risk calculator to estimate a patient's 5-year risk of SCD, with the recommendation that high-risk patients receive implantable cardioverter defibrillators (ICD) to prevent fatal ventricular arrhythmias.

For the current study, investigators retrospectively evaluated 3,703 patients to see if their HCM Risk-SCD score accurately predicted whether they eventually went on to suffer SCD.

Overall, the analysis showed that the tool could distinguish well between high- and low- risk patients, with good agreement between what it predicted and their actual 5-year SCD rates.

Specifically, patients classified as low risk (predicted to have a SCD incidence of <4% at 5 years) , had a 5-year SCD incidence of 1.4%, while those classified as high risk (predicted to have a SCD incidence ?6% at 5 years) had an incidence of 8.9%.

"We calculated that for every 13 high-risk patients who receive an ICD as recommended by ESC guidelines, one patient could potentially be saved from SCD," noted Dr. O'Mahony.

"The study also shows that the HCM Risk-SCD calculator can be used to avoid unnecessary ICD implants in low risk patients, supporting the 2014 ESC recommendation not to implant ICDs in these individuals."

He emphasised that despite the good prognostic performance of the HCM Risk-SCD calculator, no strategy will ever be able to predict and prevent all SCDs, especially for intermediate risk patients (defined as a 5-year SCD risk of 4-6%).

Nevertheless, "quantification of risk enhances the shared decision-making process," he concluded.
-end-


European Society of Cardiology

Related Hypertrophic Cardiomyopathy Articles from Brightsurf:

New medication may treat underlying causes of hypertrophic cardiomyopathy
Mavacamten, a new investigational cardiac medication, may improve heart function for people with thickened heart muscle leading to obstructed blood flow through the heart, a condition known as obstructive hypertrophic cardiomyopathy.

Study reinforces drug's potential to treat hypertrophic cardiomyopathy
WSU research sheds new light on a molecule that may be used to treat heart conditions that can lead to stroke, heart attack and other forms of heart disease.

Shared protein fingerprint could simplify treatment of common inherited heart disease
University of Wisconsin-Madison scientists discovered that many different genetic mutations result in surprisingly similar changes to heart muscle proteins in patients with the most severe manifestations of hypertrophic cardiomyopathy.

Trial supports first specific treatment for obstructive hypertrophic cardiomyopathy
Mavacamten improves heart function and symptoms in patients with obstructive hypertrophic cardiomyopathy, according to results of the EXPLORER-HCM trial presented in a Hot Line session today at ESC Congress 2020.

Comparing stress cardiomyopathy before, during COVID-19 pandemic
Patients with acute coronary syndrome presenting during the COVID-19 pandemic were compared with patients presenting at times prior to the pandemic to investigate the incidence of stress cardiomyopathy during the pandemic in this observational study.

Connecting the dots between heart disease, potential for worse COVID-19 outcomes
People with certain heart diseases may be more susceptible to worse outcomes with COVID-19, but the reason why has remained unknown.

Mayo researchers create, test AI to improve EKG testing for hypertrophic cardiomyopathy
An approach based on artificial intelligence (AI) may allow EKGs to be used to screen for hypertrophic cardiomyopathy in the future.

New gene mutation associated with Fabry cardiomyopathy
The A143T variant of the GLA gene is associated with an increased risk of Fabry cardiomyopathy, according to a new study.

Tracking a silent killer: New biomarker identified for arrhythmogenic cardiomyopathy
Scientists have identified a metabolic biomarker that could help track the progression of arrhythmogenic cardiomyopathy (AC) -- an inherited heart condition that can kill swiftly and without warning -- in a study of heart tissue and plasma from patients with AC.

How race is associated with differences among patients with hypertrophic cardiomyopathy
Researchers in this observational study looked at how race was associated with difference in symptoms, access to care, genetic testing and clinical outcomes among 2,467 patients (8.3% black and 91.7% white) with hypertrophic cardiomyopathy, a condition where the heart muscle becomes abnormally thick, which can make it harder to pump blood.

Read More: Hypertrophic Cardiomyopathy News and Hypertrophic Cardiomyopathy Current Events
Brightsurf.com is a participant in the Amazon Services LLC Associates Program, an affiliate advertising program designed to provide a means for sites to earn advertising fees by advertising and linking to Amazon.com.