Co-Executive Director of Le Bonheur Children’s Heart Institute Jeffrey A. Towbin, MD, recently published the state-of-the-art reviews “Diagnosis and Evaluation of Hypertrophic Cardiomyopathy” and “Management of Hypertrophic Cardiomyopathy” with an expert panel for the Journal of the American College of Cardiology (JACC).
These consensus documents create a comprehensive best care model for hypertrophic cardiomyopathy (HCM) based on clinical practice experience, personal research and peer-reviewed literature. Towbin is also chief of Cardiology and medical director of the Cardiomyopathy, Heart Failure and Heart Transplant Program at Le Bonheur Children’s, medical director of Cardio-Oncology and Cardio-Hematology at St. Jude Children’s Research Hospital and professor of Pediatric Cardiology at the University of Tennessee Health Science Center.
According to the reviews, HCM is an under-recognized disease that occurs in one in 200 to one in 500 individuals worldwide. Because HCM is highly treatable, has seen advances in care and provides family screening opportunities, a timely diagnosis is crucial. In the first article, the JACC expert panel systematically reviewed a range of issues related to HCM, from best practices for initial evaluation to genetic testing to exercise and physical activity in an effort to provide a best care model for this patient group.
The second article covered the management of HCM including sudden death prevention, surgical options for reversing heart failure and considerations for heart transplant.
“Comprehensive initial patient evaluations are important for reliable diagnosis, accurate portrayal of HCM and family history, risk stratification and to distinguish HCM forms,” said Towbin. “In recent years, effective management strategies for major HCM complications have emerged which improve clinical course, lower mortality and morbidity rates substantially and enhance the likelihood of normal longevity and good quality of life.”
“Through these guidelines, our panel aimed to express key principles for HCM in ‘real-world’ clinical language, largely focused on young adults,” said Towbin. “While we support and promote the advantages of HCM Centers of Excellence like Le Bonheur’s, an equally important objective was to more expansively inform cardiovascular practitioners caring for HCM patients in general cardiology environments.”
Better understanding of the disease, improved diagnostic technologies and advances in therapeutics have consequently transformed the treatment and management of HCM. This has evolved from management primarily through medication to management with devices and interventional therapies. As a result, HCM mortality has been reduced tenfold.
“Evidence-based and guideline-directed personalized treatment strategies have transformed HCM into a starkly different disease entity,” said Towbin. “More widespread implementation of these advances in regional and community-based populations and worldwide, remains an important challenge for this disease that has now emerged from the darkness.”
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