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Syncope in hypertrophic cardiomyopathy: Explaining the unexplained

Published:November 24, 2022DOI:https://doi.org/10.1016/j.ijcard.2022.11.030
      Hypertrophic cardiomyopathy (HCM) is the most common inherited cardiomyopathy, with modern estimates of prevalence suggesting it may occur in as many as 1 in 200–500 individuals. [
      • Semsarian C.
      • Ingles J.
      • Maron M.S.
      • Maron B.J.
      New perspectives on the prevalence of hypertrophic cardiomyopathy.
      ] In a recent issue of the International Journal of Cardiology, meta-analysis data revealed that syncope occurs frequently in HCM, present in 15.8% of patients over a relative short follow-up period (5.6 years). [
      • Mascia G.
      • Crotti L.
      • Groppelli A.
      • Canepa M.
      • Merlo A.C.
      • Benenati S.
      • et al.
      Syncope in hypertrophic cardiomyopathy (part I): an updated systematic review and meta-analysis.
      ] That same meta-analysis revealed a relative risk of 1.99 for sudden cardiac death (SCD) or appropriate implantable cardioverter-defibrillator (ICD) discharge. [
      • Mascia G.
      • Crotti L.
      • Groppelli A.
      • Canepa M.
      • Merlo A.C.
      • Benenati S.
      • et al.
      Syncope in hypertrophic cardiomyopathy (part I): an updated systematic review and meta-analysis.
      ] Syncope has long been recognized as an adverse prognostic indicator in HCM and remains a key component of both ACC/AHA and ESC guidelines for SCD prevention decision-making in patients with HCM. [
      • Ommen S.R.
      • Mital S.
      • Burke M.A.
      • Day S.M.
      • Deswal A.
      • Elliott P.
      • et al.
      2020 AHA/ACC guideline for the diagnosis and treatment of patients with hypertrophic cardiomyopathy: a report of the American College of Cardiology/American Heart Association joint committee on clinical practice guidelines.
      ,
      • Authors/Task Force m
      • Elliott P.M.
      • Anastasakis A.
      • Borger M.A.
      • Borggrefe M.
      • Cecchi F.
      • et al.
      ESC guidelines on diagnosis and management of hypertrophic cardiomyopathy: the task force for the diagnosis and Management of Hypertrophic Cardiomyopathy of the European Society of Cardiology (ESC).
      ] On superficial review, these data and guideline recommendations could give the mistaken impression that syncope is a well understood entity within HCM. However, despite syncope in HCM patients being common, its clinical approach has remained a quagmire, [
      • Geske J.B.
      • Gersh B.J.
      Explaining unexplained syncope in hypertrophic cardiomyopathy: a clinical dilemma.
      ] made all the more challenging given the well-recognized adverse prognostic impact.
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