Amyloid cardiomyopathy is a form of restrictive cardiomyopathy and can result in significant progressive diastolic congestive heart failure.

The familial form of amyloid cardiomyopathy is caused by a gene mutation resulting in a form of amyloid deposition called transthyretin. This can progress rapidly and can result in end-stage diastolic heart failure. Senile amyloid cardiomyopathy occurs much more slowly and is from the “wild type” or naturally occurring transthyretin amyloid protein. Diagnosis is made by myocardial biopsy.

The only major therapy for familial amyloid cardiomyopathy is combined heart-liver transplantation (the liver is included since it is the location of the transthyretin production). Medical management is unsuccessful and is directed at improving symptoms.