The Clinical Characteristic Of Primary Systemic Amyloidosis With Cardiac Involvement: A Case Report And Review Of The Literature

ObjectiveTo study the pathology, manifestations, diagnosis and prognosis of primary systemic amyloidosis involving the heart. MethodsCase report. A 41-year-old woman with primary systemic amyloidosis presented congestive heart failure,kidney and lingua damage. The data of manifestations, ECG, echocardiography ,pathology and the literature of the disease were analysed. ResultsPrimary systemic amyloidosis is a disease complex resulting from the extracellular diposition of amiloid, an insoluble proteinaceous material in different organs. Congo red stain is positive and/or typical amyloid fibrils can be found under electron microscopy in biopsy of the suspected organ or involved tissue. Widespread extracellular depositionof amyloid interferes with the function of normal tissues and organs. The heart, kidney and liver are often involved in it. Patients often presented with heart failure, cardiac arrhythmia, proteinuria, macroglossia and so on. Indentification between the disease and hypertrophic cardiomyopathy, constrictive pericarditis, restrictive cardiomyopathy is important. The final diagnosis needs biopsy. The disease management is still unsatisfactory, the prognosis is poor, especially when the heart is involved and the patient has congestive heart failure. ConclusionBecause the clinical symptom are various and most of them are non-specific. It is easy to give incorrect diagnosis. Biopsies of endocardium, liver, kidney, rectum, abdominal wall fat must be done in order to comfirm the diagnosis.