A Sparkling Heart

56yo male, with history of systolic and diastolic CHF status post ICD, secondary to amyloidosis, presented to the ED with LLE swelling. Top differential diagnoses consisted of DVT vs. CHF exacerbation. No evidence of DVT on duplex ultrasound. CXR showed moderate right pleural effusion and atelectasis, and pacemaker leads in good position. A bedside echo/pulmonary US was performed and showed interventricular hypertrophy with “sparkling,” RVH, LVH, as well as inferior wall hypokinesis, adequate placement of pacer leads, and right pleural effusion. Notably, the patient appeared to have a normal EF by qualitative analysis on US.

It has been reported since 1981 that the myocardium in patients with amyloidosis becomes thickened and takes on a hyperechoic “granular sparkling” appearance in 2D mode. However, this finding is not specific. A review report states increased myocardial echogenicity has SN 87% and SP 81%, but in combination with increased atrial thickness the SP increases to 100%, with SN decreasing to 60%. There have been significant advancements in the use of US for assessment of amyloidosis in regards to diagnosis, progression analysis, and prognosis. Although we did not see increased atrial thickness on the ultrasound of this patient, this simple assessment of myocardial “sparkling” and thickness for amyloidosis is a great tool for the novice ultrasonographer.

Toyama J, Ikeda S, Ikeda U. Echocardiographic Assessment of the Cardiac Amyloidoses. Circulation Journal. 79;721-734. 2015. https://www.jstage.jst.go.jp/article/circj/79/4/79_CJ-14-1425/_pdf/-char/en

Rachael Tena, PGY-1 EM

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