Cardiac amyloidosis

Amyloidosis is a family of disorders of the immune system in which one or more organs in the body accumulate amyloid. There are four different forms of amyloidosis: systemic amyloid light chain (AL) amyloidosis, amyloid A (AA) amyloidosis, hereditary, and senile amyloidosis. The abnormal proteins can be found as Bence-Jones proteins in urine mainly in patients with light chain (AL) amyloidosis. Cardiac amyloidosis is a myocardial disease characterized by extracellular amyloid infiltration throughout the heart and is an important cause of progressive heart failure (restrictive cardiomyopathy) with a wide spectrum of clinical manifestations. Typically, cardiac arrhythmias and diastolic heart failure occur, followed by sudden cardiac death in many cases. The diagnosis of cardiac amyloidosis is a combination of clinical, electrocardiographic and imaging methods, but definite diagnosis is based on endomyocardial biopsy. Prognosis of AL amyloidosis is poor and treatment is often a challenge, although a combination of high-dose chemotherapy, autologous stem cell transplantation and heart transplantation have shown survival benefits. Reactive AA amyloidosis may respond to anti-inflammatory and immunosuppressive drugs. Heart Metab. 2011;49:29–31.