Hypertrophic cardiomyopathy is globally recognized as the most prevalent genetic cardiomyopathy, affecting approximately 1 in 200 to 1 in 500 individuals. This condition is characterized by significant variability in its presentation. Nonetheless, the emergence of highly effective diagnostic and treatment strategies has transformed the identification and management of HCM, resulting in a substantial reduction in disease-related morbidity and mortality rates. Despite the appreciation of near-normal life expectancy in individuals with HCM, the high prevalence of heart failure symptoms underscores the need for further advancements in non-invasive diagnostic tools and the refinement of management strategies to accurately stratify patient risk and assess disease progression. This chapter delves into the critical distinctions in the pathophysiology, clinical profile, and manifestation of HCM-related heart failure, as well as the approach to the diagnosis and management of this disorder.

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Inherited Cardiac Conditions: Management of the Hypertrophic Cardiomyopathy Patient with Heart Failure

  • Sanjay Sivalokanathan,
  • Alejandro De Feria Alsina,
  • Anjali Owens

摘要

Hypertrophic cardiomyopathy is globally recognized as the most prevalent genetic cardiomyopathy, affecting approximately 1 in 200 to 1 in 500 individuals. This condition is characterized by significant variability in its presentation. Nonetheless, the emergence of highly effective diagnostic and treatment strategies has transformed the identification and management of HCM, resulting in a substantial reduction in disease-related morbidity and mortality rates. Despite the appreciation of near-normal life expectancy in individuals with HCM, the high prevalence of heart failure symptoms underscores the need for further advancements in non-invasive diagnostic tools and the refinement of management strategies to accurately stratify patient risk and assess disease progression. This chapter delves into the critical distinctions in the pathophysiology, clinical profile, and manifestation of HCM-related heart failure, as well as the approach to the diagnosis and management of this disorder.