0413: Cardiac involvements in patients with amyloidosis

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• 作者：Thouraya Ben Salem ; ^{bensalemthouraya@yahoo.fr" class="auth_mail" title="E-mail the corresponding author} ; Molka Tougorti ; Maria Khatib ; Wafa Ben Salem ; Mounir Lamloum ; Imed Ben Ghorbel ; Mohamed Habib Houman
• 刊名：Archives of Cardiovascular Diseases Supplements
• 出版年：2016
• 出版时间：January 2016
• 年：2016
• 卷：8
• 期：1
• 页码：35
• 全文大小：47 K

文摘

Amyloidosis is a rare disease. Some types of amyloidosis may significantly affect the heart; abnormal monoclonal light-chain s (AL), transthyretin-related hereditary (TTR) and senile amyloidosis.

Patients and Methods

Patients’ files with amylodosis were studied (hospitalized in an internal medicine department, from 2000 to 2013), only patients with cadiac involvement were enrolled.

Results

Fourteen patients with cardiac amyloidosis were included; sex ratio M/F was 1. Mean age at diagnosis was 60.6 years (range 36-78 years). Cardiac manifestations revealed amyloidosis in 42.8% of cases. Patient complained of fatigue (50%), dyspnea (35.7%), chest pain (28.5%) and palpitation (14.2%). One patient presented with recurrent syncope attack due to paroxysmal atrioventricular block. Four patients had cardiac failure at amylodosis diagnosis.

Amyloidosis was systemic in all cases; Kidney localization (9 cases), hepatomegaly (6 cases), and macroglossia (4 cases). N-terminal pro-brain natriuretic peptide (NT- proBNP) test, performed in one case, was high (5375 ng/l). Chest X-Ray showed cardiomegaly in 8 cases and a cardiac bottle aspect in 2 cases. In 4 patients, low voltage aspect of the electrocardiogram was the leading sign. Echocardiography revealed hypertrophic cardiomyopathy (10 cases), thickened interventricular septum (2 cases) and granular myocar-dial sparkling (2 cases). Five patients had a pericardial effusion. Cardiac magnetic resonance imaging, performed in one case, helped to characterize myocardial tissue deposits and cardiac function. Type AL amyloidosis was observed in 57% and type AA in 21.4%.

Comments

Cardiac amyloidosis has a poor prognosis, with a risk of sudden death, arterial or ventricular arrhythmia, complete or incomplete heart block, and heart failure. Some biological tests (NT pro BNP and cardiac troponin, recently ultrasensitive troponin) and nuclear imaging may be helpful for an early assessment of cardiac involvements and treatment.