Many HCM patients, perhaps even the majority, are currently unable to identify the specific gene behind their HCM through genetic testing. Despite this obvious difference, family screening, risk stratification and treatment standards are no different for patients who carry a HCM gene and those who do not have identified gene(s).
A recent article published in Circulation suggests that there ARE differences which should result in different treatment for this subset of patients.
In particular, non-sarcomere positive patients:
- have a better prognosis, with lower rates of heart failure, sudden death, atrial fibrillation and stroke
- Have lower incidence of family members affected by the disease
- Are more likely to have additional medical conditions such as obesity, hypertension and diabetes
The article by Dr. Hugh Watkins, a British HCM and genetic expert, suggests that:
- the risk to first degree relatives of this type of HCM patient is less than 50% and therefore, there is less need for repeated screening of relatives
- Hypertension should be treated more aggressively in these patients.
Read more about non-genetic HCM here and more about screening these patients here on HCMBeat.
According to this recent article published in the Journal of the American Heart Association, lifestyle choices can influence the development and/or progression of HCM.
In particular, the authors made the following recommendations:
- Exercise: Recreational exercise should be encouraged in HCM patients.
- This recommendation was largely based on the findings of the recent RESET-HCM study which found that moderate exercise, specifically tailored to each individual patient’s capacity, was beneficial to the patient’s general health and well being. **For an in-depth look at the RESET-HCM study, check out this recent HCMBeat interview with the authors of the study, Drs. Sara Saberi and Sharlene Day.
- Eating and Drinking:
- Patients should avoid large meals and should not exercise immediately after eating.
- Care should be taken to avoid becoming dehydrated.
- Alcohol should only be consumed in moderation.
- Healthy weight should be maintained.
- Hypertension should be treated aggressively, though treatment may be challenging, especially in obstructive HCM.
- Obstructive Sleep Apnea, which may exist in as many as 70% of HCM patients, should be treated to minimize potential for arrhythmia and to improve blood flow.