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Biatrial thrombosis in cardiac amyloidosis

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A 60 year old woman was admitted to hospital because of congestive heart failure. Primary AL amyloidosis with extensive infiltration of the heart was diagnosed. The ECG showed sinus rhythm and low QRS voltage.  Transthoracic echocardiography typically documented a diffuse thickening of atrial and ventricular walls, preserved systolic ventricular function, and dilatation of the atria. In the body of left atrium a small thrombus was visible.

 Transoesophageal echocardiography confirmed the presence of a roundish 1 × 1 cm thrombus in the corner between interatrial septum and aorta. Moreover a 2 × 3 cm non-mobile thrombus was detected in the right atrial appendage (figure). The left atrial appendage emptying velocity was much reduced.

 Ten days after starting anticoagulation (intravenous heparin then warfarin), repeat transoesophageal echocardiography showed

the resolution of the left atrial thrombus and a slight size reduction of the right atrial appendage thrombus. A few days later the patient had syncope, followed by shock and death. Necropsy revealed a massive pulmonary embolism; the right atrial appendage thrombus was still present.  In advanced forms of cardiac amyloidosis, an impairment of atrial emptying predisposes to atrial thrombosis, even in sinus rhythm. The atrial dysfunction has been ascribed to the combination of amyloid infiltration of atrial walls and an increase of atrial afterload owing to restrictive haemodynamics. Furthermore, clotting factors might play a role in thrombogenesis of primary systemic amyloidosis.