Is it just a simple LVH?

02 March 2020

Case : Male, 58 yo

Dyspnea on effort during mild activity. No chest pain
Said to have thickened heart
No history of hypertension. No history of sudden death nor similar symptoms in family
Physical examination : Mild ankle edema

Echocardiography

His ECG

ECG of this patient did not showed LVH as expected. Instead his ECG showed low voltage at his limb leads. There is poor R progession at V1-V4 with ST depression and T inversion at V5-V6

Take a closer and more detailed assessment

Biatrial Enlargement

Diastolic dysfunction : restrictive pattern

Biventricular hypertrophy

Granular  /sparkling appearance of myocardium

More advanced assessment with LV strain

Average apical strain/ average basal+ mid strain > 1 was sensitive (93%) and specific (82%) in differentiating Cardiac Amyloidosis

Phelan D, et al. Heart. 2012 Oct;98(19):1442-8

EFSR = Ejection Fraction
GLS

EFSR >4.1  has sensitivity (90%) and specificity (92%), with AUC of 0.95 to differentiate Cardiac Amyloidosis

Pagourelias ED, et al. Circ Cardiovasc Imaging. 2017;10:e005588

So .. What is the most likely diagnosis of this patient ?

CARDIAC AMYLOIDOIS

Findings in Diagnosing Amyloidosis



Donnelly JP, et al. Cleve Clin J Med. 2017 Dec;84(12 Suppl 3):12-26

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