Analysis of clinical and imaging features of cardiac amyloidosis:a multicenter study

ZHANG Lu; TANG Hong; CHEN Lianglong; WU Xiaoxia; CHENG Liuquan; WANG Zhanbo; WANG Ye; HUANG He; LI Jinguo; WANG Jingjing; FENG Bin; ZHI Guang

doi:10.3969/j.issn.1673-4254.2014.03.02

Abstract：Objective To summarize the features of clinical manifestations, laboratory tests and imaging findings of patients with cardiac amyloidosis (CA). Methods A total of 60 CA patients (including 41 male and 19 female patients) from 4 centers admitted between May, 2012 and November, 2013 were included in the study. The demographic data, medical history, clinical manifestations, laboratory test data, ECG, cardiac ultrasound, and cardiac magnetic resonance (CMR) imaging of the patients were analyzed. Results Two-thirds of the 60 CA patients, were middle-aged or elderly men, and 47%of the patients had AL-CA. The clinical manifestations included exertional dyspnea (73%), pedal edema (47%), hypotension (47%), and hypertrophy of the tongue (22%); abnormal laboratory test results included albuminuria (53%) and liver (15%) and kidney (28%) dysfunction; blood routine, urine and serum immunoglobulin quantification and immunofixation electrophoresis could help the screening of AL-CA. Kidney (53%) and liver (15%) involvement was common, and 86% of AL-CA patients had kidney involvement. Typical ECG characteristics included poor R wave progression (35%), low voltage in limb leads (33%), and a pseudo infarct Q wave (30%);the latter two were more frequent in AL-CA. The characteristics of ultrasound findings included left ventricle thickening (100%), left atrial enlargement (87%) and enhanced echo of the myocardial granules(92%), and diastolic dysfunction was obvious in all the CA patients regardless of the systolic function. The DT and E/e' of the mitral annulus could be used as an index to evaluate diastolic dysfunction in early stage of the disease. Left ventricular (LV) global subendocardial late gadolinium enhancement (LGE, 81%) accompanied by right ventrivular (RV) and atrial LGE was the typical characteristic of CMR, and the range of LGE in the RV and the two atria was wider in AL-CA than in non-AL-CA. NT-proBNP (97%) and cardiac troponin (53%) in CA patients were both elevated, which helped in diagnosing and assessing the severity of cardiac involvement, according to which 50%of the patients were found to be at a high risk, 43%at an intermediate risk, and 7% at a low risk. Conclusion The combination of the features of clinical, laboratory tests and imaging findings of CA have important diagnostic and prognostic value for CA.