Echocardiography shows typical features in cardiac amyloidosis: concentric left (and sometimes right) ventricular wall thickening, infiltration of the atrial septum, dilation of the atria, often inceased echogenicity of the valves.The myocardial texture is particular, with increased echogenicity and sometimes granular sparkling (an aspect that is not specific for amyloidosis).Cardiac magnetic resonance imaging (MRI) was performed, which confirmed LV hypertrophy with preserved LV global systolic function, and showed diffuse subendocardial late gadolinium enhancement, suggestive of cardiac amyloidosis (Figure 3). The histological preparations stained with Congo red were examined under a microscope with polarized light and amyloid deposits were identified.
Echocardiography shows typical features in cardiac amyloidosis: concentric left (and sometimes right) ventricular wall thickening, infiltration of the atrial septum, dilation of the atria, often inceased echogenicity of the valves.The myocardial texture is particular, with increased echogenicity and sometimes granular sparkling (an aspect that is not specific for amyloidosis).Tags: Math Problem Solving PracticeI Need Help With My Math Homework For FreeNordstrom Research PapersSocial Network Business PlanThesis Publishers UkDessay Miracle D UneWomen Scientist Essays
Most patients already have a systemic involvement at the time of cardiac involvement diagnosis.
Neurologic symptoms can include autonomic neuropathy that can lead to postural hypotension and syncope, peripheral neuropathy, with sensory disturbances, and carpal tunnel syndrome.
Two years ago he underwent a coronary angiography, in the setting of atypical angina with an ECG showing R-wave amputation in the right precordial leads, showing normal epicardial coronaries.
One year ago he presented at the local hospital with several episodes of paroxysmal atrial fibrillation, for which treatment with amiodarone and oral anticoagulants was started.
Based on the clinical history and examination as well as the above investigations, the diagnosis was of congestive heart failure (functional class NYHA III) due to restrictive cardiomyopathy (CMR). Still frame from cine image in 4 chamber-view showing concentric LV hypertrophy, dilated left atrium and small pericardial effusion. Delayed sequences after gadolinium administration in short axis shows diffuse intramyocardial delayed enhancement.
The association between increased left ventricle wall thickness on ultrasound and low QRS voltage on ECG pointed towards a diagnosis of infiltrative cardiomyopathy, with a first line suspicion of cardiac amyloidosis beeing the most frequent cause of CMR. In order to obtain a histological confirmation of the disease, an abdominal fat aspirate was performed.
These findings are typical for patients which develop AL amyloidosis (primary amyloidosis). A serum free light chains assay was performed, showing a marked increase of λ light chains, with a κ/λ ratio of 0.09, indicating the presence of a population of plasma cells producing clonal λ free light chains.
To complete the work-up for the extension of the disease, a lower limb electromiography was performed, which showed sensitive and motor polineuropathy.
Cardiac amyloidosis is a myocardial disease resulting from the extracellular amyloid infiltration of the heart.
Amyloid deposits occur in the ventricles and atria, as well as perivascularly (particularly in the small vessels) and in the valves. The infiltrative process results in biventricular wall thickening with nondilated ventricles, and atrial dilation due to elevation of filling pressures.