Case Report
We describe a case of systemic amyloidosis with cardiac involvement diagnosed by transthoracic echocardiography and hystologic study.
Patient history prior to current observation :
A 62-year-old caucasian woman was evaluated at our institution because of dizziness. She has been previously hospitalized in medical department where systemic amyloidosis with renal and liver involvement was diagnosed. For severe renal impairment the patient was in chronic dialytic treatment. Her medical history was remarkable for systemic hypertension, monoclonal gammopathy and thyroidectomy for papillifery carcinoma.
Clinical findings on admission, evolution and outcome :
Physical examination: Blood Pressure = 140/80 mmHg, Heart Rate = 87/min regular, 3/6 apical systolic murmur, clear lungs, no peripheral oedema, hepatomegaly or jugular veins' distention.
ECG: Sinus rhythm at 87 bpm with low voltages in peripheral leads and pseudo-infarction pattern in precordial leads (fig. 1).
Transthoracic echocardiography showed nondilated ventricles with wall thickening and "granular sparkling" appearance of the myocardium (fig. 2, fig. 3, fig. 4), preserved left ventricular global systolic function (fig. 5), diastolic dysfunction with a restrictive pattern (fig. 6). Aortic and mitral leaflets appeared slightly tickened (fig. 5) and color-Doppler showed mild aortic and mitral regurgitation. Atrial chamber appeared enlarged with no evidence of atrial septum thickening (fig. 5).
Immunohistochemical stainings: myocardial (fig. 7), renal (fig. 8), bone marrow (fig. 9) and colon (fig. 10) amyloid deposition.