This week, MyoKardia announced positive data on its experimental drug for HCM, mavacamten (formerly known as MYK-461), for obstructive HCM.
After 6 months on the drug, of the 13 patients enrolled in the open label extension to the Pioneer study, NYHA Class improved in 8 of 10 patients, while 7 of the 8 patients were considered asymptomatic by the end of the study.
In addition to seeing an improvement in symptoms, patients also showed improvement to certain markers of cardiac function including left ventricular filling pressure,
NT-proBNP, a marker of cardiac wall stress, and left atrial volume index. Ejection fractions remained within normal levels for all patients in the study.
The complete results of this study will be presented by Oregon Health & Science University’s Dr. Steven Heitner at next weekend’s meeting of the American College of Cardiology in New Orleans.
DISCLOSURES: HCMBEAT HAS RECEIVED UNRESTRICTED EDUCATIONAL GRANTS FROM MYOKARDIA. ADDITIONALLY, CYNTHIA BURSTEIN WALDMAN OF HCMBEAT SERVES AS A PATIENT ADVISOR ON THE STEERING COMMITTEE FOR MYOKARDIA’S EXPLORER TRIAL.
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.
A recent study of U.K. HCM patients compared a racially mixed sample of 425 patients, including 163 black patients and 262 Caucasians. The study concluded that while asymmetric septal hypertrophy was the predominant pattern in both ethnic groups, black patients had more instances of apical and concentric hypertrophy, which could, in turn, be responsible for delayed diagnosis of these patients.