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.
A recent retrospective study conducted by doctors at Yale -New Haven Health System found that patients with hypertrophic cardiomyopathy fare best when treated at a specialty center using a team approach to HCM. The study found that this was especially true for patients coming from disadvantaged backgrounds who often fare worse outside of a HCM specialty setting.
The findings of this study suggest that patients with HCM are best served when referred to HCM specialty care instead of receiving care solely from general cardiologists.
The August 16, 2018 online version of the New England Journal of Medicine contains an broad overview of the current state of clinical knowledge and treatment of HCM written by HCM expert Dr. Barry Maron. It is entitled “Clinical Course and Management of Hypertrophic Cardiomyopathy.”
Dr. Maron discusses the many advances that have been made in the diagnosis and treatment of hypertrophic cardiomyopathy since it was first described 55 years ago, noting that life expectancy and qualify of life have dramatically improved in this period of time. According to Dr. Maron, the contemporary management paradigm for HCM have reduced “the risk of adverse cardiovascular events and death to levels below the levels among patients with other cardiac or non-cardiac disorders.”
According to several news reports, CNN chief and former NBCUniversal head Jeff Zucker is taking six weeks off to undergo elective surgery to treat his hypertrophic cardiomyopathy. Specific details about the surgery were not revealed. New York Magazine reported that in 2010 he visited Minneapolis Heart Institute where he was told he needed an implantable defibrillator.
The most common surgery for the treatment of HCM symptoms is a septal myectomy.
See these stories for more info:
Wall Street Journal
Atlanta Journal Constitution
Los Angeles Times
HCMBeat wishes Mr. Zucker the best of luck during his surgery and recovery.
Here is a link to some resources we have collected for patients who are going through myectomy: Resources for Patients About Myectomy
Yet another study has confirmed the safety of MRIs in patients with non-MRI conditional ICDs and pacemakers.
The latest study, from the University of Pennsylvania and published in the New England Journal of Medicine, found no adverse effects from 1.5 Tesla MRI scans performed on 1509 patients who underwent a total of 2103 scans. 58% had pacemakers and 42% had ICDs.
This study confirms previous findings relating to the safety of MRIs in ICD and pacemaker patients reported by HCMBeat here and here.
You can also read my personal experience trying to get a brain MRI in Los Angeles last year here.