Abstract

A 40-years-old man presented with orthopnea, lower limb edema and exertional dyspnea, markedly worsening over last 1 month. He was diagnosed as severe heart failure (NYHA4) and admitted to our hospital in April 2016. An electrocardiogram showed low QRS voltage in the limb leads and poor R progression in the precordial leads (V1-4). Prominent left ventricular (LV) dilation with diffuse LV hypokinesis (LVDd/Ds 68.5/64.2 mm), severe impairment of LV systolic function (LVEF 14%) and left atrium dilatation (55.3 mm) were revealed by echocardiography. Thickness of interventricular septum was 12.3 mm in diastole, without “granular sparkling sign”. Immediately after the admission, medical therapies with intravenous dobutamine, carperitide, furosemide were initiated. His body weight was decreased to 61 kg (−20.4 kg), serum BNP concentration was improved from 1970 pg/dl to 26 pg/dl. On X-ray coronary angiography, there was no significant coronary arterial stenosis. Both myocardial biopsy and subcutaneous fat biopsy demonstrated amyloid deposition, diagnosing him as having AL cardiac amyloidosis. Interestingly, after medical treatment, low QRS voltage were disappeared in limb leads, and the range of QS pattern in the precordial leads became small (only in V1-2). We've reported a case of cardiac amyloidosis with dilated LV chamber and significant improvement of low QRS voltage in limb lead after standard medical therapy.

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