JACC Review Papers Provide Insights For Diagnosing, Evaluating and Managing HCM

A panel of clinicians and thought leaders with substantial experience with hypertrophic cardiomyopathy (HCM) provides evidence-based recommendations and expert insights into providing care to patients with HCM, detailed in two new state-of-the-art review papers published Jan. 24 in the Journal of the American College of Cardiology.

According to Barry J. Maron, MD, MACC, et al., HCM was once considered a rare inherited cardiac disease that was difficult to diagnose. Today, HCM is a highly treatable condition with relatively low morbidity and mortality, thanks to timely and accurate diagnoses and contemporary treatments. But while HCM has a general population prevalence of 1:200-1:500, just 10% to 20% of cases are identified clinically. While HCM is under-diagnosed, highly effective management strategies have emerged, they write, altering the clinical course and reducing rates of morbidity and mortality.

The first paper, focused on the diagnosis and evaluation of HCM, reinforces the critical role of reliable HCM diagnosis, which is rooted in echocardiography and cardiac magnetic resonance (CMR) imaging. In addition, family screening with echocardiography or CMR imaging and genetic testing including cascade screening is recommended. The review provides a decision pathway for screening and a guide to clinical evaluation and noninvasive testing in HCM. Along with the detailed sections on screening and evaluation, the document addresses exercise, physical activity and athletes.

The second paper, focused on the management of HCM, notes that innovations in treatment have significantly reduced morbidity and HCM-related mortality by 0.5% year. These innovations include matured risk stratification with major markers that reliably selects patients for prophylactic defibrillators and prevention of arrhythmic sudden death; low risk to high benefit surgical myectomy that reverses progressive heart failure caused by outflow obstruction; anticoagulation prophylaxis that prevents atrial fibrillation-related embolic stroke and ablation techniques that decrease the frequency of paroxysmal episodes; and occasionally, heart transplant for end-stage nonobstructive patients.

A detailed management decision pathway is provided, along with detailed sections on sudden death prevention; obstructive HCM and reversal of heart failure; alcohol septal ablation; nonobstructive HCM; and atrial fibrillation.

“After more than one-half century, the narrative surrounding HCM has changed substantially,” the authors write. “Advances in therapeutics, diagnostics, and understanding of the disease spectrum and its relevant mechanisms, pursued relentlessly by clinical investigators and practitioners using evidence-based and guideline-directed personalized treatment strategies, have transformed HCM into a starkly different disease entity.”

Keywords: Atrial Fibrillation, Prevalence, Embolic Stroke, Cardiomyopathy, Hypertrophic, Risk Assessment, Heart Failure, Cardiology, Heart Transplantation, Ablation Techniques, Magnetic Resonance Spectroscopy, Exercise, Echocardiography, Anticoagulants, Genetic Testing, Defibrillators, Death, Sudden, Athletes


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