Cardiac Amyloidosis
AL amyloidosis is commonly associated with cardiac involvement complicated by CHF, both diastolic and systolic, with restrictive cardiomyopathy as it progresses. In addition to progressive exertional dyspnea and fatigue, AL amyloidosis is associated with syncope as well as sudden death. Sudden death from amyloid is due to infiltration of the amyloid into the cardiac conducting system which is--the system of nerves that provide the electrical impulses necessary for coordinating the efficient sequential pumping of blood through the different chambers and that also adjust both heart rate and strength of cardiac muscle contraction, as needed, to properly perfuse critical organs. When amyloid disrupts these critical conducting pathways, the patient can suffer bradycardic loss of consciousness, impaired cardiac output (secondary to atypical rhythms), or even sudden death (from prolonged loss of conduction or severe rhythm abnormalities).
Typically, patients with amyloid cardiac involvement will present hypotensive or normotensive (rather than hypertensive) due to the progressively impaired cardiac filling secondary to restrictive changes from the infiltration of amyloid protein into the heart muscle.
Patients with amyloid are suspected of cardiac involvement in the presence of evidence of early symptoms of CHF such as progressive dyspnea and fatigue, particularly in the absence of other common causes such as chronic hypertension and ischemic heart disease and have elevations of N-terminal pro b-type natriuretic peptide (NT-Pro BNP).
Cardiac amyloidosis medical expert witness specialties include hematology, cardiology, pathology, thoracic surgery, internal medicine, family medicine, hospitalist medicine, critical care medicine, and forensic pathology.