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Clinical Update on Hypertrophic Cardiomyopathy Management Guidelines

This clinical update presents recommendations for the management of hypertrophic cardiomyopathy (HCM), including diagnostic criteria, adverse events, pathophysiology, shared decision-making, family management, and treatment strategies such as septal reduction therapy and atrial fibrillation management. It emphasizes the importance of risk assessment for sudden cardiac death, genetic testing, and personalized care in patients with HCM.

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Clinical Update on Hypertrophic Cardiomyopathy Management Guidelines

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  1. AHA Clinical Update ADAPTED FROM: 2024 AHA/ACC/AMSSM/HRS/PACES/SCMR Guideline for the Management of Hypertrophic Cardiomyopathy

  2. Table 1. Applying Class of Recommendation and Level of Evidence to Clinical Strategies, Interventions, Treatments, or Diagnostic Testing in Patient Care • • • • • • • • • • • • • • • • • • • • • •COR and LOE are determined independently (any COR may be paired with any LOE). • • • •A recommendation with LOE C does not imply that the recommendation is weak. Many important clinical questions addressed in guidelines do not lend themselves to clinical trials. Although RCTs are unavailable, there may be a very clear clinical consensus that a particular test or therapy is useful or effective. •*The outcome or result of the intervention should be specified (an improved clinical outcome or increased diagnostic accuracy or incremental prognostic information). • †For comparative-effectiveness recommendation (COR 1 and 2a; LOE A and B only), studies that support the use of comparator verbs should involve direct comparisons of the treatments or strategies being evaluated. • • • • •‡The method of assessing quality is evolving, including the application of standardized, widely-used, and preferably validated evidence grading tools; and for systematic reviews, the incorporation of an Evidence Review Committee. •COR indicates Class of Recommendation; EO, expert opinion; LD, limited data; LOE, Level of Evidence; NR, nonrandomized; R, randomized; and RCT, randomized controlled trial.

  3. Hypertrophic Cardiomyopathy Prevalence and Characteristics Inheritance Pattern Disease Prevalence Sex Triggers for Evaluation Distribution -/- +/- +/- Differential Diagnosis: Non-HCM Causes of LV Hypertrophy

  4. Defining Hypertrophic Cardiomyopathy in 2024 • • • Diagnostic Criteria in Children Diagnostic Criteria in Adults

  5. Adverse Events Associated Hypertrophic Cardiomyopathy Although some patients with HCM have a normal life expectancy without limiting symptoms, many will have important consequences Progressive Functional Limitation Sudden Death Atrial Fibrillation Heart Failure Thromboembolism

  6. Pathophysiology of Hypertrophic Cardiomyopathy Dynamic LVOT Obstruction Myocardial Ischemia Mitral Regurgitation Atrial and Ventricular Arrythmias Metabolic/Energetic Dysfunction Diastolic Dysfunction

  7. Pathophysiology of HCM: LV Outflow Tract Obstruction

  8. Pathophysiology of HCM: Diastolic Dysfunction • • • Diastolic Dysfunction • • •

  9. Pathophysiology of HCM: Mitral Valve Abnormalities • • •

  10. Shared Decision-Making in HCM Discussions should involve Discussions should involve: : • • • Shared decision discussions should be applied to: Shared decision discussions should be applied to: • • • • •

  11. Patient Centered Team-Based Care • • • • • • • • • • •

  12. Septal Reduction Therapy • •

  13. Diagnosis and Initial Evaluation • • •

  14. Clinical Features in Patients with “HCM Mimics” LIFE STAGE LIFE STAGE SYSTEMIC FEATURES SYSTEMIC FEATURES POSSIBLE ETIOLOGY POSSIBLE ETIOLOGY DIAGNOSTIC APPROACH DIAGNOSTIC APPROACH • • • • • • • • • • • • • • • • • • •

  15. Guidance for Family Management

  16. Guidance for Individuals Diagnosed with Clinical HCM Guidance for Individuals Diagnosed with Clinical HCM • • Phenotype Positive Phenotype Positive • • • • •

  17. Screening with Electrocardiography and 2D Echocardiography Screening with Electrocardiography and 2D Echocardiography Recommendations in Asymptomatic Family Members* Recommendations in Asymptomatic Family Members* AGE OF FIRST AGE OF FIRST- -DEGREE RELATIVE DEGREE RELATIVE INITIATION OF SCREENING INITIATION OF SCREENING REPEAT ECG, ECHO REPEAT ECG, ECHO

  18. Genetic Testing Genetic Testing

  19. Heart Rhythm Assessment in HCM 1 1 1 1 1 1 2b 2b

  20. Risk Assessment of Sudden Cardiac Death (SCD) in HCM Assess the following (Class 1): Assess the following (Class 1): • • • •

  21. ICD Placement in High-Risk Patients with HCM • • • • • • • • •

  22. Pharmacologic Management of Obstructive and Non Pharmacologic Management of Obstructive and Non- -Obstructive HCM HCM Obstructive • • • • • • • In highly selected patients with apical HCM with severe dyspnea or angina (NYHA class III or class IV) despite maximal medical therapy, and with preserved EF and small LV cavity size, apical myectomy by experienced surgeons at comprehensive centers may be considered to reduce symptoms • • • • • •

  23. Invasive Management of Obstructive HCM Invasive Management of Obstructive HCM • • • • • • • • • • •

  24. Hypertrophic Cardiomyopathy with Advanced Heart Hypertrophic Cardiomyopathy with Advanced Heart Failure Failure • • • • •

  25. Management of Atrial Fibrillation in Patients with HCM Management of Atrial Fibrillation in Patients with HCM 1 1 1 1 1 1 2a 2a 2a 2a 2a 2a 2a 2a

  26. HCM-AF Risk Calculator: Risk for Atrial Fibrillation in Hypertrophic Cardiomyopathy

  27. Management of Ventricular Tachycardia in Patients with Management of Ventricular Tachycardia in Patients with HCM HCM

  28. Recreational Physical Activity and Competitive Sports in HCM 1 1 1 1 2a 2a 2a 2a 2b 2b 3: No 3: No Benefit Benefit 3: 3: Harm Harm

  29. Occupation Recommendations in HCM 2a 2a 2a 2a 2b 2b

  30. Pregnancy in HCM 1 1 2a 2a 1 1 2a 2a 1 1 2a 2a 1 1 2a 2a 3: 3: 1 1 Harm Harm

  31. Unmet Needs and Future Directions

  32. Acknowledgments Dr. Ijeoma Eleazu Dr. Ahmed Kazem Dr. Dennis Narcisse Dr. Jessica Regan Dr. Raymond Yeow

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