The American College of Cardiology (ACC) and the American Heart Association (AHA) have jointly released a new clinical guideline for managing individuals with hypertrophic cardiomyopathy (HCM), an inherited cardiac condition that causes the heart muscle to become too thick. The guideline emphasizes collaborative decision-making with patients and provides updated treatment recommendations for both adult and pediatric patients. HCM affects approximately 1 in 500 individuals, with some cases going undiagnosed until a sudden death occurs. Symptoms of HCM may include fainting, chest pain, shortness of breath, or irregular heartbeats.
The updated guideline includes recommendations for using new pharmacologic treatments, such as cardiac myosin inhibitors, for patients with symptomatic obstructive HCM who do not respond well to initial drug therapy. These medications specifically target the thickened heart muscle and aim to improve symptoms and quality of life. However, they are monitored under the FDA’s Risk Evaluation and Mitigation Strategies (REMS) program, which may require additional steps for both clinicians and patients. Specialized training is needed to prescribe these medications, and regular screenings are necessary for patients.
In addition to medication treatment, the guideline highlights the benefits of exercise for HCM patients. Low to moderate intensity recreational exercise is recommended as part of managing overall health for HCM patients, with some individuals possibly able to participate in competitive sports under the guidance of HCM clinical specialists. The guideline emphasizes the importance of shared decision-making between clinicians and patients in determining appropriate levels of physical activity.
Poorly managed HCM can lead to serious complications, including sudden cardiac death (SCD). The guideline provides recommendations for assessing and managing the risk of SCD by identifying risk markers and integrating them with tools to estimate an individual’s SCD risk score. This information can help guide shared decision-making regarding the placement of implantable cardioverter defibrillators, taking into account an individual’s risk tolerance and treatment goals.
The guideline also includes specific recommendations for pediatric patients with HCM, emphasizing the importance of Pediatric risk stratification for SCD and the need for specialized HCM centers with expertise in pediatric care. Exercise stress testing is recommended for children diagnosed with HCM to assess functional capacity and provide prognosis feedback. The guidelines aim to provide clinicians with evidence-based recommendations for effectively managing HCM in both adult and pediatric patients, with the goal of improving outcomes and quality of life for individuals with this condition.
In conclusion, the new clinical guideline for managing HCM released by the ACC and AHA emphasizes collaborative decision-making with patients and includes updated treatment recommendations based on the latest evidence. Recommendations for medication treatment, including the use of cardiac myosin inhibitors, are provided for patients with symptomatic obstructive HCM who do not respond well to initial therapy. The benefits of exercise for HCM patients are highlighted, with low to moderate intensity exercise recommended as part of overall health management. The guideline also addresses the risk of sudden cardiac death in HCM patients and provides recommendations for assessing and managing this risk to guide treatment decisions. Special attention is given to pediatric patients with HCM, with specific risk stratification recommendations and exercise testing guidelines included in the guideline. Overall, the new guideline aims to provide clinicians with the tools and information needed to effectively manage individuals with HCM, ultimately improving outcomes and quality of life for patients with this condition.
