A recent article by several noted HCM experts shares their recommendations for the screening and evaluation of patients with HCM using several scenarios commonly encountered in clinical practice. Notably, this paper makes several recommendations which differ from the 2020 ACC/AHA Guidelines for the Diagnosis and Treatment of Patients with Hypertrophic Cardiomyopathy (2020 Guidelines).
In particular, the article recommends:
- Cardiac MRI with contrast upon initial evaluation, and every 3 – 5 years thereafter. (The 2020 Guidelines recommend Cardiac MRI in certain situations, but not for all patients)
- Genetic testing only in cases where a pathogenic or likely pathogenic mutation has been identified in a family member and in limited other circumstances (the 2020 Guidelines recommends genetic testing for all as part of the initial assessment)
- Screening of family members 12 or older only (the 2020 Guidelines recommend screening of children of any age when genetic HCM is diagnosed in a family member and no later than puberty, in any event)
- That HCM patients be disqualified from competitive sports. (The 2020 guidelines instead recommend a shared decision making approach).
Continue reading “Recommendations for HCM Depart from 2020 ACC/AHA Guidelines”
A recent study published in the European Journal of Preventative Cardiology found that a 12-lead electrocardiogram (EKG) was not useful as a screening tool to determine which children were at increased risk of sudden death and therefore, a candidate to receive an implantable defibrillator.
The full article can be found here.
According to a recent paper published in Circulation, children’s risk for sudden death should be evaluated using different risk factors than those used for adults.
Two main differences seen between factors influencing the risk of sudden death of children and adults were that:
- for children, family history of sudden death was not a risk factor;
- a left ventricular outflow tract gradient in a child suggested a lower risk of sudden death.
The authors of this study propose that the risk assessment factors highlighted in this study (and not the various risk assessment tools that have been developed for adults) should be used by clinicians to evaluate the need for implantable defibrillators in children.
A recent study by Canadian researchers published in the American Heart Association journal Circulation found a much lower incidence of HCM related sudden death than expected. The study included deaths in individuals that occurred between the ages of 10 and 45 in the Canadian province of Ontario between 2005 and 2016. According to lead author Dr. Paul Dorian of the University of Toronto, the expectation was that 1 incident of sudden cardiac death would be identified in every 100 to 200 people who had HCM. Instead, researchers found that the likelihood of sudden death of HCM patients was instead only about 1 in 3,000 people/year.
The study also found that found 7 in 10 HCM-related sudden deaths occurred in people not previously diagnosed with the condition. Men had more than 5 times the risk of sudden cardiac death than women, and most deaths occurred during rest or light activity: only about 17% happened during or immediately after exercise.
As a result of the study, according to Dr. Dorian, “our findings allow us to lower the temperature on our degree of worry about the condition.”
Another clinical take away from the study, according to the researchers, is that current exercise guidelines for HCM patients may be too restrictive.
According to a paper published last week in JAMA Cardiology, doctors at Tufts University’s HCM Center have been able to identify 95% of their patients at high risk of sudden cardiac death (SCD) from HCM. Tufts applied an updated and modified version of the risk factors enumerated in the American College of Cardiology/American Heart Association Guidelines promulgated in 2011.
Continue reading “Docs Reliably Identify HCM Patients in Need of ICDs”
This article, published in this week’s Women’s Health magazine, features the former ballerina and beauty queen turned vocal patient activist. These days, Lindsay has focused her efforts on saving lives from sudden cardiac arrest. Lindsay’s efforts in the state of Ohio have resulted in proposed legislation to identify student athletes at risk of sudden cardiac arrest, while her partnership with the American Heart Association is steadily making CPR and AED training a graduation requirement in high schools across the nation.
Watch for more life-saving advocacy from Lindsay in the future. She is clearly much more than another pretty face!
Updated to include a video of Lindsay discussing her implantation with a S-ICD device.
Doctors at the University of Pennsylvania have created a new model which they say can predict the risk of sudden cardiac arrest in people without known heart disease. According to the article, published recently in Circulation, low levels of albumin, a protein commonly tested in routine blood panels, is a novel risk factor among the twelve factors identified. This article in Cardiac Rhythm News offers more details.