Cardiac amyloidosis is a relatively rare condition that has several different causes. Management involves pharmaceutical treatment of heart failure, which is fundamentally related to diastolic dysfunction, and treatment of the underlying disease process. Evolving treatments that target the source of amyloid proteins are dramatically improving the outlook for patients. Early diagnosis is vital for the best results to be achieved.
- The systemic amyloidoses are a group of disorders caused by deposition of misfolded insoluble proteins (amyloid) in different tissues and organs.
- Cardiac amyloidosis does not develop in all patients with systemic amyloidosis.
- Patients with cardiac amyloidosis present with heart failure (with preserved ejection fraction), fatigue and arrhythmia.
- Findings on echocardiography or cardiac MRI are distinctive and may direct further investigation.
- Technetium pyrophosphate bone scanning may help in diagnostic differentiation of the subtype of amyloidosis.
- Infiltration of amyloid protein in the interstitial spaces of the myocardium causes predominantly diastolic dysfunction. Diuretics are the mainstays of treatment, and modern heart failure medications may be harmful in this setting.
- Prognosis depends on ability to treat the underlying disease process.