Cardiac amyloidosis produces specific alterations to the functionality of the heart. Echocardiography can 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 cardiac amyloidosis. Echocardiography can be used to help physicians with diagnosis, however, it can only be used for the suggestion of the disease, not the confirmation, unless it is late stage amyloidosis. ECG/EKG
ECGs of patients with cardiac amyloidosis usually show a low voltage in the limb leads, with an unusual extreme right axis. There is usually a normal P-wave, however, it can be slightly prolonged. For patients with light-chain amyloidosis, the QRS complex pattern is skewed, with poor R-waves of the chest leads.Holter ECGs can 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 amyloidosis. For light chain amyloidosis, serum immunoglobulin free light chain assay can be used for diagnosis and following of the amyloidosis. In light-chain amyloidosis, a low paraprotein level can be present. Cardiac biomarkers
There are two main cardiac biomarkers used in the assessment of cardiac amyloidosis, troponin and N-terminal proBNP.
New data suggests that orthotopic heart transplant followed by melphalan and stem cell transplant produces results similar to non cardiac amyloidosis indicated heart transplant. To treat complications, medications can be prescribed including midodrine for autonomic neuropathy, amiodarone for patients with atrial fibrillation to prevent arrhythmias, and warfarin used after a cardioembolic episode. Beta-blockers should be avoided due to the usual symptom of hypotension.Familial (ATTRm-CM) Treatment: In recent years there have been developments in the treatment of Familial/Transthyretin cardiac amyloidosis including methods to suppress transthyretin production, stabilize amyloid fibrils, and medications that can destroy already existing fibrils. For familial amyloidosis, ACE-inhibitors and beta-blockers can be prescribed if there is no autonomic neuropathy. Suppression of transthyretin production: liver transplantation and medications that decreases the activity of the transthyretin genes (patisiran and inotersen). In patients with familial transthyretin mutations, liver transplantation can provide the body with a source of normal transthyretin. By changing the source of transthyretin from the original liver that contains the mutated transthyretin to a healthy liver, there will be no more production of the abnormal protein. However, liver transplant does not reverse the disease. The goal of liver transplant is to prevent additional amyloid deposition and prevent new symptoms/complications from happening. These medications bind to the mRNA of transthyretin and prevent the production of the transthyretin protein, thus decreasing the overall amount of transthyretin that can accumulate in the body. Stabilization of abnormal transthyretin:  There are medications that can stabilize the normally folded transthyretin, preventing misfolding and subsequent amyloid deposition.