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Letter| Volume 136, ISSUE 3, e49, March 2023

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Getting to the Heart of the Issue: Demographics of Hypertrophic Cardiomyopathy

      To the Editor:
      We have read with great interest your thought-provoking article by Maron et al,
      • Maron BJ
      • Rowin EJ
      • Ambe SP
      • Maron MS
      Changing demographics in hypertrophic cardiomyopathy and implications for management: clinical research.
      which discussed the changing demographics observed in hypertrophic cardiomyopathy (HOCM) in recent years. The study concluded that patients are significantly older at presentation, with thinner septae than in previous studies, often with milder symptomatology.
      While raising important concerns about the changing phenotype of HOCM, this article does not clearly identify the diagnostic criteria for the study population to include genetic testing, imaging modality used, and the presence of symptoms. The utilization of transthoracic echocardiograms has been increasing 6%-11% annually, which could allow for greater identification of mild symptomatology than would have previously been identified by clinical assessment alone.
      • Alter DA
      • Stukel TA
      • Newman A
      Proliferation of cardiac technology in Canada.
      ,
      • Pearlman AS
      • Ryan T
      • Picard MH
      • Douglas PS
      Evolving trends in the use of echocardiography.
      Additionally, it would be important to characterize whether confirmatory genetic testing is associated with any variations in severity or age at presentation.
      Furthermore, the authors do not address whether confounding factors such as comorbidities and medications were present. Long-standing hypertension and amyloidosis are examples of comorbidities that may impact cardiac hypertrophy.
      • Vermeer AMC
      • Janssen A
      • Boorsma PC
      • Mannens MMAM
      • Wilde AAM
      • Christiaans I
      Transthyretin amyloidosis: a phenocopy of hypertrophic cardiomyopathy.
      Use of anti-remodeling agents such as angiotensin-converting enzyme inhibitors or other contributors to hypertrophy (such as tacrolimus) would be important to identify as well (G. H. Silva et al, unpublished data, 2022). We feel that more information on the population demographics, including the presence of genetic changes as mentioned above, would help elucidate the mechanisms by which these changes might be occurring. We remain curious as to whether this transformation of disease presentation as identified by the paper represents a true evolution of the disease process, or rather, a result of secondary forces from sampling bias or confounding factors. Without addressing these characteristics of the patient population studied, it is difficult to extrapolate these results to the general population of HOCM.

      References

        • Maron BJ
        • Rowin EJ
        • Ambe SP
        • Maron MS
        Changing demographics in hypertrophic cardiomyopathy and implications for management: clinical research.
        Am J Med. 2022 May 29; ([online ahead of print])https://doi.org/10.1016/j.amjmed.2022.05.006
        • Alter DA
        • Stukel TA
        • Newman A
        Proliferation of cardiac technology in Canada.
        Circulation. 2006; 113: 380-387
        • Pearlman AS
        • Ryan T
        • Picard MH
        • Douglas PS
        Evolving trends in the use of echocardiography.
        J Am Coll Cardiol. 2007; 49: 2283-2291
        • Vermeer AMC
        • Janssen A
        • Boorsma PC
        • Mannens MMAM
        • Wilde AAM
        • Christiaans I
        Transthyretin amyloidosis: a phenocopy of hypertrophic cardiomyopathy.
        Amyloid. 2017; 24: 87-91