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Comprehensive evaluation of hypertrophic cardiomyopathy: European Journal of Heart Failure expert consensus document - PubMed

14 hours ago
  • #Hypertrophic cardiomyopathy
  • #Cardiac disease
  • #Multidisciplinary care
  • Hypertrophic cardiomyopathy (HCM) is the most common inherited cardiac disease and a leading cause of heart failure and sudden cardiac death (SCD) in young adults.
  • A structured and multidisciplinary approach to care is essential due to HCM's complex pathophysiology, phenotypic diversity, and rapidly evolving therapeutic landscape.
  • The manuscript proposes a six-pillar framework to standardize and optimize HCM evaluation and management.
  • Pillar 1: Establishing the correct diagnosis using multimodal imaging and genetic testing to differentiate sarcomeric HCM from phenocopies like amyloidosis, Fabry, or mitochondrial disease.
  • Pillar 2: Evaluating symptoms and left ventricular outflow tract obstruction (LVOTO) through dynamic assessment with exercise echocardiography to guide treatment.
  • Pillar 3: Risk stratification for SCD integrates risk scores and imaging data to inform implantable cardioverter defibrillator decisions.
  • Pillar 4: Genetic evaluation and family management enable cascade testing, early detection, and counseling.
  • Pillar 5: Managing comorbidities such as atrial fibrillation, hypertension, obesity, and sleep-disordered breathing for holistic care and symptom control.
  • Pillar 6: Education and lifestyle guidance focus on safe sport participation, avoiding dehydration and vasodilators, and reproductive counseling in a multidisciplinary setting.