[Cardiac amyloidosis].
PREREQUIS: Amyloidosis is a rare infiltrative disease characterized by multiple clinical features. Various organs are involved and the cardiovascular system is a common target of amyloidosis. Cardiac involvement may occur with or without clinical manifestations and is considered as a major prognostic factor.


OBJECTIVE
To analyze the clinical features of cardiac involvement, to review actual knowledgement concerning echocardiographic diagnostic and to evaluate recent advances in treatment of the disease.


METHODS
An electronic search of the relevant literature was carried out using Medline and Pubmed. Keys words used for the final search were amyloidosis, cardiopathy and echocardiography. We considered for analysis reviews, studies and articles between 1990 and 2007.


RESULTS
Amyloidosis represents 5 to 10% of non ischemic cardiomyoparhies. Cardiac involvement is the first cause of restrictive cardiomyopathy witch must be evoked in front of every inexplained cardiopathy after the age of forty. The amyloid nature of cardiopathy is suggered if some manifestations were associated as a peripheric neuropathy, a carpal tunnel sydrome and proteinuria > 3g/day. Echocardiography shows dilated atria, a granular sparkling appearance of myocardium, diastolic dysfunction and thickened left ventricle contrasting with a low electric voltage. The proof of amyloidosis is brought by an extra-cardiac biopsy, the indications of endomyocardial biopsy are very limited. The identification of the amyloid nature of cardiopathy has an direct therapeutic implication: it indicates the use of digitalis, calcium channel blockers and beta-blockers. Today the treatment of amyloidosis remains very unsatisfactory especially in the cardiac involvement. An early diagnosis before the cardiac damage may facilitate therapy and improve prognosis.
Boussabah
Elhem
E
Service de Cardiologie, Hôpital des FSI, La Marsa.
Zakhama
Lilia
L
Ksontini
Iméne
I
Ibn Elhadj
Zied
Z
Boukhris
Besma
B
Naffeti
Sana
S
Thameur
Moez
M
Ben Youssef
Soraya
S
D000686:Amyloidosis / Q000145:classification / Q000150:complications / Q000175:diagnosis / Q000628:therapy; D009202:Cardiomyopathies / Q000145:classification / Q000150:complications / Q000175:diagnosis / Q000628:therapy; D002318:Cardiovascular Diseases / Q000209:etiology; D006801:Humans