The American College of Cardiology (ACC) and the American Heart Association (AHA) have announced a new clinical guideline for managing individuals diagnosed with hypertrophic cardiomyopathy (HCM). The guideline emphasizes the importance of working together with patients who have HCM and provides updated recommendations for the most effective treatment options for both adult and pediatric patients.A new article discussing the management of patients with HCM and offering updated recommendations for effective treatment pathways for both adult and pediatric patients. HCM is a genetic heart condition that thickens the heart muscle, making it difficult to pump blood effectively. It affects about 1 in 500 individuals, but many cases go undiagnosed due to lack of symptoms. In some cases, HCM is only discovered after a sudden death. Symptoms, when present, can includeThe article discusses the updated guidelines for the treatment of Hypertrophic Cardiomyopathy (HCM). It mentions the importance of incorporating the most recent data to equip clinicians with the latest recommendations. Dr. Steve R. Ommen, the medical director of the Mayo Hypertrophic Cardiomyopathy Clinic and chair of the guideline writing committee, emphasizes the significance of these new guidelines in helping patients with HCM return to their normal daily lives with proper care and management. The updated recommendations in the guideline reflect recent evidence about HCM treatment and management, including new forms of pharmacologic management.The guideline covers a variety of topics related to hypertrophic cardiomyopathy (HCM), including the role of genetic testing in diagnosis and management; the use of implantable cardioverter-defibrillators (ICDs) in high-risk patients; the participation in vigorous recreational activities and competitive sports; and the risk assessment for sudden cardiac death (SCD) with a focus on pediatric patients.
Among the recommendations in the guideline is the addition of cardiac myosin inhibitors, a new type of medication for patients with symptomatic obstructive HCM who do not experience adequate relief from first-line drug therapy. Symptomatic obstructive HCM is a form of HCM in which the heart muscle is restricted. Cardiac myosin inhibitors are the first class of medication approved by the FDA to specifically target the thickening of the heart muscle rather than just treating the symptoms. However, The FDA’s Risk Evaluation and Mitigation Strategies (REMS) program requires additional steps and time for both clinicians and patients. Clinicians need special training to prescribe the medication, and patients need regular screenings.
“These new drugs provide an option for patients who have not responded to initial treatment and may want to postpone or possibly avoid more aggressive options,” Ommen stated. “With this guideline, we’re giving clinicians immediate guidance on effectively using this evidence-based treatment and enhancing their patients’ quality of life.”/p>
Research is growing to show that exercise can have more benefits than risks for patients with HCM, in addition to taking medication. It is recommended that patients with HCM incorporate low to moderate intensity recreational exercise into their overall health management. Some HCM patients may even be able to participate in competitive sports after consulting with HCM clinical specialists.
Ommen mentioned, “Recommendations for physical activity are constantly changing as new research emerges. As a part of a healthy lifestyle, patients with HCM are now encouraged to participate in low-to-moderate intensity physical activities. We are learning about the potential benefits of vigorous physical activities for these patients as well.”Reasonable for some people. With shared decision-making between the healthcare provider and the patient, some patients may even be able to return to competitive sports.”
Poorly managed HCM can lead to many complications including SCD. The new guideline includes recommendations for evaluating and managing the risk of SCD by establishing clear risk markers. Guidance for integrating risk markers with tools to estimate an individual patient’s SCD risk score is recommended to help with the patient/healthcare provider shared decision-making regarding implantable cardioverter defibrillator placement, taking into account a patient’s personal level of risk tolerance and preference.The new guideline includes specific treatment goals that focus on improving quality of life. It also extends recommendations to pediatric patients, emphasizing the importance of a specific pediatric risk stratification for sudden cardiac death (SCD). It also stresses the need for risk calculators tailored to children and adolescents and highlights the importance of expertise in pediatrics at hypertrophic cardiomyopathy (HCM) centers. Additionally, the new guideline recommends exercise stress testing for children diagnosed with HCM to assess functional capacity and provide prognostic feedback.
