Clinical Analysis Of Cardiac Amyloidosis

Background:Amyloid deposit on various organizations is the characteristics ofamyloidosis，with the destruction of normal structure and function, leading tocorresponding organizational dysfunction, so, amyloidosis is a systemic diseaseto trigger multisystem damage.The kinds of extracellular amyloid depositionshows that the diversity of pathological types. Congo red staining showsamyloid deposition is no structure pink material, apple green birefringenceunder polarized light, fold structure under the electron microscope. Its clear thatthere are more than30kinds of protein can lead to amyloidosis, including lightchain immunoglobulin、thyroid hormone protein、fibrinogen Aa、acute phasereaction protein A、apoA etal. Has been clear about the causes of amyloidosis、tissue damage mechanism and the mechanism of hereditary amyloidosis, etc. Itimproves the effective treatment of hope.Amylaceous kind goes heart is damaged is a kind of disease that deposit of amylaceous appearance material causes pathological change at the heart,its called cardiac amvloidosis(CA).The obvious characteristic is cardiac stiffness,ventricular filling limited, loss of myocardial compliance, show the diastolicfunction is impaired. So, the most commom performance of CA is restrictivecardiomyopathy. It is the typical representative of restrictive cardiomyopathyand study the natural model of diastolic dysfunction. Objective:This study aims to summarize the clinical characteristics of CA, providesthe basis for clinical further understanding and the treatment of the diseaseMethods：Amyloid cardiomyopathy patients that treated in our hospital2010—2015are retrospectively analyzed the clinical characteristics, echocardiography andelectrocardiogram(ECG), laboratory examination and diagnosis methods,summarize the clinical characteristics of amyloidosis cardiomyopathy.Results：There are5males(83%) and1female (17%) in6patients diagnosedCA.Age from52to78years（65±10）. It takes1～14months（8±5） from thepathogenesis to confirmed. Clinical characteristic is the progressive heartfailure,hypotension3cases,multiple myeloma2cases.ECG showed that all thepatients were low voltage in the limb leads,3patients had pseudoinfarction,atrial fibrillation(AF)2cases,sinus bradycardia(SB) in1case,complete rightbundle branch block(CCCB)2cases.Ultrasonic cardiogram(UCG) showed thatall the patients were possessed of enlarged left atrium, normal left ventricle,increased thickness of the left ventricular walls and interventricular septums aswell as diffuse weakness of the wall motion.The pathological biopsy showedSubcutaneous fat biopsy5cases and renal biopsy1case.All of the pathologicalexaminations were positive in Congo red stain.Conclusion：Amyloidosis cardiomyopathy heart damage in patients have various clinical manifestations, lack of specificity.Often involved a variety of other tissue and organ. The misdiagnosis rate is high, the prognosis is poor.Has the following clinicalfeatures:(1) Cardiomyopathy diastolic is early performance;(2) Restrictive Cardiomyopathy、Right heart failure,Some patients are intractable congestive heart failure (RCHF);(3) Refractory arrhythmia;(4) Pulmonary hypertension and pulmonary heart disease;(5) Characteristic ECG:low voltage in the limb leads, poor R wave progression in the precordial leads and ST-T changes,combining with AF and conduction block.(6) Characteristic UCG:enlarged left atrium, normal left ventricle, increased thickness of the left ventricular walls and interventricular septums as well as diffuse weakness of the wall motion.