Objective: Amyloidosis is a systemic disease potentially afflicting the heart. In this study we retrospectively studied patients presenting with major cardiac amyloidotic involvement.
Methods and results: From 1997 until 2000, eight patients with major cardiac involvement of amyloidosis resulting in heart failure were diagnosed. All patients presented with heart failure. Diagnostic work-up, clinical, electrocardiographic, echocardiographic characteristics and treatment modalities are presented. Seven patients with acquired (both primary AL and postinflammatory AA) amyloidosis out of a total of eight patients died within one year after diagnosis, as a result of intractable cardiac failure due to both systolic and diastolic left ventricular dysfunction despite state-of-the-art medical treatment of heart failure. Only one patient with hereditary amyloidosis undergoing liver transplantation survived.
Conclusions: Despite optimal medical cardiac failure treatment, acquired cardiac amyloidosis carries an ominous prognosis probably because patients are already in a very advanced stage of the disease at presentation.