Cardiac amyloidosis: updates in diagnosis and management

Arch Cardiovasc Dis. 2013 Oct;106(10):528-40. doi: 10.1016/j.acvd.2013.06.051. Epub 2013 Sep 23.


Amyloidosis is a severe systemic disease. Cardiac involvement may occur in the three main types of amyloidosis (acquired monoclonal light-chain, hereditary transthyretin and senile amyloidosis) and has a major impact on prognosis. Imaging the heart to characterize and detect early cardiac involvement is one of the major aims in the assessment of this disease. Electrocardiography and transthoracic echocardiography are important diagnostic and prognostic tools in patients with cardiac involvement. Cardiac magnetic resonance imaging better characterizes myocardial involvement, functional abnormalities and amyloid deposition due to its high spatial resolution. Nuclear imaging has a role in the diagnosis of transthyretin amyloid cardiomyopathy. Cardiac biomarkers are now used for risk stratification and staging of patients with light-chain systemic amyloidosis. Different types of cardiac complications may occur, including diastolic followed by systolic heart failure, atrial and/or ventricular arrhythmias, conduction disturbances, embolic events and sometimes sudden death. Senile amyloid and hereditary transthyretin amyloid cardiomyopathy have better prognoses than light-chain amyloidosis. Cardiac treatment of heart failure is usually ineffective and is often poorly tolerated because of its hypotensive and bradycardiac effects. The three main types of amyloid disease, despite their similar cardiac appearance, have specific new aetiological treatments that may change the prognosis of this disease. Cardiologists should be aware of this disease to allow early treatment.

Keywords: (99m)Tc-3-3-diphosphono-1-2-propanodicarboxylic acid; (99m)Tc-DPD; 2D-GLS; AL; ASCT; ATTR; Amyloidosis; Amylose; Atteinte cardiaque; Autologous stem cell transplantation; Cardiac involvement; Cardiac magnetic resonance imaging; Chaînes légères; ECG; Electrocardiogram; Hereditary transthyretin-related amyloidosis; LC; LGE; LS; LV; LVEF; Late gadolinium enhancement; Left ventricular; Left ventricular ejection fraction; Light-chain; Light-chains; Longitudinal strain; Management; N-terminal prohormone of B-type natriuretic peptide; NT-proBNP; RV; Right ventricular; SSA; Systemic senile amyloidosis; TTE; TTR; Traitement; Transthoracic echocardiography; Transthyretin; Transthyrétine; Two-dimensional global longitudinal strain; cMRI; light-chain amyloidosis.

Publication types

  • Review

MeSH terms

  • Amyloid Neuropathies, Familial / diagnosis
  • Amyloid Neuropathies, Familial / therapy
  • Amyloidosis / diagnosis*
  • Amyloidosis / immunology
  • Amyloidosis / therapy*
  • Cardiomyopathies / diagnosis*
  • Cardiomyopathies / immunology
  • Cardiomyopathies / therapy*
  • Diagnostic Imaging / methods
  • Early Diagnosis
  • Electrocardiography
  • Humans
  • Predictive Value of Tests
  • Treatment Outcome

Supplementary concepts

  • Amyloidosis, Hereditary, Transthyretin-Related