Clinical experience with cardiac amyloidosis.
OBJECTIVE
Amyloidosis is a systemic disease potentially afflicting the heart. In this study we retrospectively studied patients presenting with major cardiac amyloidotic involvement.


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.
Goeminne
Ann
A
Departments of Cardiology, St. Jan Hospital, Brugge, Belgium.
Missault
Luc
L
Bauwens
Frank
F
De Pauw
Michel
M
De Sutter
Johan
J
D000328:Adult; D000368:Aged; D000686:Amyloidosis / Q000175:diagnosis*; D004562:Electrocardiography; D004586:Electrophoresis; D005260:Female; D006331:Heart Diseases / Q000175:diagnosis* / Q000209:etiology; D006333:Heart Failure / Q000209:etiology / Q000628:therapy; D006801:Humans; D008297:Male; D008875:Middle Aged; D011379:Prognosis; D012189:Retrospective Studies; D018487:Ventricular Dysfunction, Left / Q000209:etiology