Cardiac amyloidosis.

نویسندگان

  • Neil Maredia
  • Simon G Ray
چکیده

Systemic amyloidosis commonly affects the heart. Indeed, cardiac symptoms may be the first clinical indicator of underlying amyloid deposition. Using two case studies, this article reviews the latest evidence regarding cardiac amyloidosis. The diagnosis of cardiac involvement can be established through imaging with echocardiography and magnetic resonance. Supportive evidence may be gained from biochemical markers such as serum N-terminal probrain natriuretic peptide (NT-proBNP). The main clinical consequences of amyloid deposition are cardiac failure and rhythm disturbances. Attempts to cure the underlying disease process with chemotherapy and/or cardiac and/or liver transplantation have had variable results. Stem-cell transplantation is associated with significant mortality in the context of cardiac involvement. Although newer therapeutic agents are emerging, the overall outlook at this time remains poor.

برای دانلود متن کامل این مقاله و بیش از 32 میلیون مقاله دیگر ابتدا ثبت نام کنید

ثبت نام

اگر عضو سایت هستید لطفا وارد حساب کاربری خود شوید

منابع مشابه

Successful Diflunisal Desensitization in a Transthyretin Amyloidosis Patient with NSAID Allergy: A Case Report

Introduction: Amyloid diseases have been known to be hereditary, including transthyretin (TTR) amyloidosis where subunit protein mutations may occur in genes for TTR leading to the deposition of fibrils (low molecular weight subunits (5 to 25 kD) of proteins) in extracellular tissues. By reducing the formation of TTR amyloid, diflunisal, a nonsteroidal anti-inflammatory drug (NSAID), has shown ...

متن کامل

Cardiac amyloidosis

Amyloidosis is a family of disorders of the immune system in which one or more organs in the body accumulate amyloid. There are four different forms of amyloidosis: systemic amyloid light chain (AL) amyloidosis, amyloid A (AA) amyloidosis, hereditary, and senile amyloidosis. The abnormal proteins can be found as Bence-Jones proteins in urine mainly in patients with light chain (AL) amyloidosis....

متن کامل

Fatal perioperative myocardial infarction in four patients with cardiac amyloidosis.

AMYLOIDOSIS is caused by the accumulation of amyloid fibrils in vital organs, leading to functional disturbances. Approximately 40% of patients with primary amyloidosis have had cardiac amyloidosis. In cardiac amyloidosis, right heart failure is the predominant sign, whereas left heart function is preserved until late in the disease. Because of the cardiac involvement, when patients with cardia...

متن کامل

Incidence, Diagnosis and Prognosis of Cardiac Amyloidosis

BACKGROUND AND OBJECTIVES Cardiac involvement is frequent in systemic amyloidosis and is the most important determinant of the clinical outcome. The aims of this study were to assess the incidence and prognosis of cardiac amyloidosis and discuss the diagnostic issues related to cardiac amyloidosis. SUBJECTS AND METHODS We retrospectively studied all patients diagnosed with systemic amyloidosi...

متن کامل

Cardiac Involvement in Four Patients with Immunoglobulin Light-Chain Systemic Amyloidosis

In immunoglobulin light-chain (L) amyloidosis the cause of death is most commonly due to cardiac involvement. Cardiac amyloidosis is frequently diagnosed by postmortem examination. There are many reports of diagnoses of cardiac amyloidosis established by autopsy of cardiac tissue. We report four patients whom we could diagnose as having cardiac amyloidosis by echocardiogram and electrocardiogra...

متن کامل

Cardiac amyloidosis: a case review series

Amyloidosis includes a spectrum of diseases that involve the deposition of misfolded proteins in various tissues throughout the body. There are many different proteins known to cause amyloidosis and the etiology of the amyloid subtype determines where the abnormal proteins deposit. AL, ATTRwt (formerly senile systemic amyloidosis), and ATTRm (formerly familial amyloidosis) are the most common a...

متن کامل

ذخیره در منابع من


  با ذخیره ی این منبع در منابع من، دسترسی به آن را برای استفاده های بعدی آسان تر کنید

برای دانلود متن کامل این مقاله و بیش از 32 میلیون مقاله دیگر ابتدا ثبت نام کنید

ثبت نام

اگر عضو سایت هستید لطفا وارد حساب کاربری خود شوید

عنوان ژورنال:
  • Clinical medicine

دوره 5 5  شماره 

صفحات  -

تاریخ انتشار 2005