Amyloid diseases of the heart: assessment, diagnosis, and referral

• Published in: Heart (British Cardiac Society) Vol. 97; no. 1; pp. 75 - 84
• Main Authors: Dubrey, S W, Hawkins, P N, Falk, R H
• Format: Journal Article Book Review
• Language: English
• Published: London BMJ Publishing Group Ltd and British Cardiovascular Society 01.01.2011; BMJ Publishing Group; BMJ Publishing Group LTD
• Subjects: Accreditation; AL amyloid; Amyloid - chemistry; Amyloidosis - diagnosis; Amyloidosis - etiology; Amyloidosis - therapy; Biological and medical sciences; Biopsy; Biopsy, Needle; Blood Proteins - genetics; Cardiology; Cardiology. Vascular system; Cardiomyopathies - diagnosis; Cardiomyopathies - etiology; Cardiomyopathies - therapy; cardiomyopathy restrictive; Chemotherapy; Diagnosis, Differential; Disease; echocardiogram; Echocardiography; Education; electrocardiogram; Electrocardiography; familial amyloid; genetics; Heart; heart failure; Humans; Light; Magnetic Resonance Angiography; Medical prognosis; Medical sciences; Microscopy; MRI; Multiple myeloma; Mutation; Mutation - genetics; Myocardium - pathology; Nervous system; Referral and Consultation; Heart; Amyloid; Circulatory system; Diagnosis; Disease; Cardiology
• ISSN: 1355-6037; 1468-201X
• DOI: 10.1136/hrt.2009.190405

In patients with a non-cardiac biopsy showing amyloid deposition, cardiac involvement has been defined-by a consensus opinion from the 10th International Symposium on Amyloidosis 1 -as either a positive heart biopsy and or/increased left ventricular wall thickness (interventricular septal thickness >12 mm) in the absence of hypertension or other potential causes of true left ventricular hypertrophy. 8 w5 In AL amyloid, the plasma cell number, degree of clonality, the quantity of light chain, and the light chain isotype are related to survival.w4 w6 w7 Senile systemic amyloid Wild-type (non-mutant) transthyretin is the precursor protein of senile systemic amyloid (SSA) and is deposited predominantly in the heart in SSA.