2D echocardiogram. Subcostal long axis showing severe thickening of ventricles secondary to amyloid deposition and maladaptive<unk> rdiac remodeling, as well as a pericardial effusion. Echocardiography Echocardiography is a safe and non-invasive method that<unk> n be used to assess the structural and functional disease of the heart.<unk>  presents with ventricle and valvular thickening, biatrial enlargement, restrictive filling pattern, with normal to mildly reduced systolic function and decreased diastolic filling. An echo<unk> n be used to evaluate for prognosis of the disease, measuring the different strains within the heart.<unk>  produces specific alterations to the functionality of the heart. Echocardiography<unk> n be utilized to detect this specific pattern (relative preservation of the apical myocardium with decreased longitudinal strain in the mid and basal sections), which is 90–95% sensitive and 80–85% specific for<unk> . Echocardiography<unk> n be used to help physicians with diagnosis, however, it<unk> n only be used for the suggestion of the disease, not the confirmation, unless it is late stage<unk> . ECG/EKG ECGs of patients with<unk>  usually show a low voltage in the limb leads, with an unusual extreme right axis. There is usually a normal P-wave, however, it<unk> n be slightly prolonged. For patients with light-chain<unk> , the QRS complex pattern is skewed, with poor R-waves of the chest leads. Holter ECGs<unk> n be used to identify asymptomatic arrhythmias. EKG changes may be present, showing low voltage and conduction abnormalities like atrioventricular block or sinus node dysfunction. Laboratory Tests Laboratory tests including urea and creatinine levels, liver enzymes, glucose, thyroid function, full blood count, and clotting tests. The analysis of serum and urine for presence of monoclonal immunoglobulin is also done through immunofixation for detection of the monoclonal band. Presence of the monoclonal band would be consistent with light chain<unk> . For light chain<unk> , serum immunoglobulin free light chain assay<unk> n be used for diagnosis and following of the<unk> . In light-chain<unk> , a low paraprotein level<unk> n be present.<unk> rdiac biomarkers There are two main<unk> rdiac biomarkers used in the assessment of<unk> , troponin and N-terminal proBNP. As expected, with<unk> rdiac damage and dysfunction, there<unk> n be an elevation of these markers in patients with<unk> . These markers have been incorporated into the various staging/scoring systems used by physicians to determine severity of the disease and prognosis.<unk> , H&E stain. Dark pink material showing<unk> rdiac myocytes, and light pink material interspersed throughout is amyloid. Biopsies Extracardiac biopsies of tissues of the kidney, liver, peripheral nerve, or abdominal fat<unk> n be used to confirm the presence of amyloid deposits. Amyloid deposits in biopsy samples are confirmed through the use of Congo red dye, which produces a green birefringence when viewed under polarized light. Sirius red staining or electron microscopy examination<unk> n also be done. The determination of the type of amyloid<unk> n be done by immunohisto-labeling techniques as well as immunofluorescence staining. For light-chain<unk>  patients, bone marrow biopsies could be conducted to determine the baseline percentage of plasma cells and to rule out multiple myeloma.<unk> therization Right heart<unk> theterization is the test used to test for elevated diastolic ventricular pressures. This test is more invasive and would be performed after inconclusive endomyocardial biopsy samples.<unk> rdiac magnetic resonance imaging<unk> rdiac magnetic resonance (CMR) is<unk> pable of measuring the thickness of different areas of the heart. This<unk> n be used for quantification of the deposits in the heart. CMR also shows the characterization of myocardial tissue through patterns of gadolinium enhancements. However, none of the CMR technique is able to differentiate ATTR-CM and AL-CM definitely. For AL-CM, 68% of them have symmetrical and concentric left ventricular hypertrophy. On the other hand, for ATTR-CM, 79% of them have asymmetrical left ventricular hypertrophy and 18% of them have symmetrical and concentric left ventricular hypertrophy. In T1-weighted imaging, edema in the heart<unk> n be detected with a high T1 signal. Meanwhile, enlargement of heart cells will reduce the T1 signal. Using T1 signal, Extracellular volume (ECV) is useful to determine the degree of amyloid deposition around the heart cells and detect the regression of amyloid deposits after treatment. ECV is higher in ATTR-CM than in AL-CM. In T2-weighted imaging, the T2 signal is increased in acute myocarditis (inflammation of heart muscles), and myocardial infarction (heart attack). T2 signal is also increased in AL-CM and ATTR-CM but the signal is greater in AL-CM before starting chemotherapy. Late gadolinum enhancement (LGE)<unk> n determine the severity of deposition of amyloid in heart tissue. The higher the LGE signal, the more severe the heart involvement. It<unk> n be divided into three stages: no LGE, subendocardial LGE, and full-thickness (transmural) LGE. Scintigraphy/radionuclide imaging Scintigraphy<unk> n be used to measure the extent and distribution of the amyloid throughout the body, including the liver, kidney, spleen, and heart. A radiolabelled serum amyloid P component<unk> n be administered to a patient intravenously and the P component pools to the amyloid deposit proportional to the size of the deposit. The labeling of the P component<unk> n then be pictured by a gamma<unk> mera. Technetium radionuclide scans<unk> n now reliably diagnosis<unk> , with certain scanning methods having greater than 99% sensitivity (but only 91% specific for<unk> . In this method of imaging, radiolabeled technetium is injected into the body where it binds to<unk> rdiac amyloid deposits. A subsequent scan is taken to determine where the tracer stays, therefore highlighting the amyloid deposition in the heart. This method allows for a noninvasive definitive diagnosis of<unk>  (as in the past an endomyocardial biopsy was required) Mass spectrometry Mass spectrometry<unk> n be used to determine whether the protein is light-chain or familial<unk>  by identifying the protein subunit.
Cardiac amyloidosis
Amyloid cardiomyopathy
Viral cardiomyopathy
Diabetic cardiomyopathy
HIV associated cardiomyopathy
Alcoholic cardiomyopathy
Coxsackievirus-induced cardiomyopathy
Dilated cardiomyopathy