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Light-chain amyloidosis with echocardiographic features of hypertrophic cardiomyopathy

Danya L Dinwoodey1, Martha Skinner, Martin S Maron

  • 1Section of Cardiology, Department of Medicine, Boston Medical Center, Boston, MA, USA.

The American Journal of Cardiology
|March 1, 2008
PubMed

Insights

Cardiac amyloidosis can mimic hypertrophic cardiomyopathy with left ventricular outflow tract obstruction. This overlap highlights the need for early diagnosis of amyloidosis for timely treatment interventions.

Area of Science:

  • Cardiology
  • Medical Imaging

Background:

  • Hypertrophic cardiomyopathy (HCM) and cardiac amyloidosis (CA) both cause left ventricular thickening visible on echocardiography.
  • Distinct hemodynamic features like left ventricular outflow tract (LVOT) gradient and systolic anterior motion of the mitral valve (SAM) are typical of HCM, but rarely seen in CA.

Purpose of the Study:

  • To report a case series of patients with cardiac light-chain amyloidosis (AL-CA) presenting with LVOT obstruction at rest.
  • To investigate the echocardiographic overlap between HCM and AL-CA.

Main Methods:

  • Retrospective review of echocardiographic findings in patients with AL-CA over a 2-year period.
  • Analysis of hemodynamic parameters and specific echocardiographic features, including LVOT gradient and SAM.

Main Results:

  • Four patients with AL-CA were identified with resting LVOT obstruction.
  • The prevalence of these HCM-like echocardiographic findings in the AL-CA cohort was approximately 4%.

Conclusions:

  • There is a significant echocardiographic overlap between HCM and AL-CA, particularly concerning LVOT obstruction.
  • Increased awareness of this overlap can improve early diagnosis of AL-CA, enabling prompt treatment when therapeutic options are most effective.

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