| [Objectives]Cardiac amyloidosis(CA)is a heterogeneous group of diseases caused by the deposition of misfolded proteins,which are arranged in specific beta-pleated fibrils(immunoglobulin light chain,transthyretin,apolipoprotein,serum amyloid A)in myocardial issue,causing restrictive cardiomyopathy without specific clinical manifestations.Because of the lack of specific manifestation and sensitive non-invasive clinical methods,the median survival for amyloidosis patients with cardiac involvement is relatively shorter.Therefore,we conducted this study to summarize the clinical characteristics and to evaluate the diagnostic and prognosis value of non-invasive clinical methods in patients with CA.[Methods]This was a retrospective analysis,including the medical data of patients with diagnosed CA,who were admitted to the Peking Union Medical College Hospital between January 2010 and February 2015.We carried the follow-up through telephone to assess the prognosis value of baseline serum free light chain(sFLC),pseudoinfarction,the left atrium(LA)and interventricular septal(IVS),and conducted a systematic review and meta-analysis to evaluate the diagnostic significance of cardiovascular magnetic resonance(CMR).[Results]1.A total of 128 CA patients were included in the final analysis.Patients expressed typical manifestations at a late age(55 ± 12y).Male patients(61.7%)were more than female ones.Clinical characteristics varied in CA.In this study,44.5%cases presented with chest distress,36.7%cases edema in lower limbs,14.1%cases shortness of breath,10.9%cases weakness,and 9.4%cases diarrhea.Light chain amyloidosis could be found in 117 cases(91.4%),including 65.6%in primary amyloidosis and 25%with multiple myeloma,hereditary transthyretin-related amyloidosis 3 cases(2.3%)and unknown type 8 cases(6.3%).Electrocardiogram(ECG):low voltage in limb leads were shown in 62 patients(48.4%),poor R-wave progression in precordial leads in 61 patients(47.7%),pseudoinfarction in 57 patients(44.5%)and ST-T changes in 98 patients(76.6%).Echocardiography showed that 76 cases(59.4%)with increased IVS thickness,44 cases(34.4%)with abnormal left ventricular eject function,60 cases(46.9%)with E/A ratio>2 and 24 cases(18.8%)with myocardial ground-glass opacity.50 cases(39.1%)presented impaired left ventricular systolic function and 64 cases(50%)left ventricular restrictive diastolic function.CMR:15 patients underwent CMR,of which,14 cases revealed a distinct late gadolinium enhancement(LGE).Nuclear imaging modalities:6 cases had 18F-FDG PET/CT exam,and all without any increased radioactive uptake in myocardial tissue.2 patients had 99mTc-MIBI,one of which presented radioactive deficiency in heart.21 patients underwent 99mTc-MDP scan,and 2 cases were shown extensive soft tissue uptake.The sensitivity of biopsy of kidney(100%),tone(82.6%),heart(80%)and gingival(71.4%)were higher in patients with CA.2.Twenty-seven patients with CA were included in the study.The group with a higher dFLC(involved sFLC-uninvolved sFLC)presented a severely impaired diastolic function with a higher early/late mitral velocity ratio(E/A ratio)and the overall survival was shorter(p=0.004).A new cut-off value of 180mg/L for dFLC or a new parameter of sFLC-ratio reached a similar result.3.Pseudoinfarction pattern was observed in 40 patients(36.4%).The presence of pseudoinfarction on the ECG has a negative prognostic effect and Kaplan-Meier survival analysis showed that lifetime was significantly shorter in the pseudoinfarction group(median 4 months vs 17 months,p<0.001).4.LA enlargement was more common in patients with severe heart failure(p=0.003)and was also a significant predictor of all-cause mortality.5.A total of 107 cardiac amyloidosis patients,who were admitted to Peking Union Medical College Hospital between 2010 and 2014,were included in the final analysis,22.4%patients presenting a normal IVS thickness.The survival rate for CA patients with normal IVS thickness was not significantly different from that of those with hypertrophic phenotype.Although in present study IVS was not able to provide prognostic information to predict mortality in the entire cohort,for patients without advanced heart failure,normal IVS thickness seemed to be associated with a poor survival.NYHA,E/A ratio and pseudoinfarction had prognostic significances in the multivariate Cox proportional hazards model.6.This meta-analysis of LGE-CMR showed that the diagnostic accuracy increased with a summary sensitivity of 85%and a summary specificity of 92%.In terms of the heart tissue characterization,LGE can distinguish CA patients from healthy controls with a summary positive likelihood ratio of 7.481(95%CI:2.835-19.739).The summary diagnostic odds ratio of 71.945 detected here(95%CI:23.552-219.77)indicated that expressed the test was more accurate in the differential diagnosis of CMR than other types of diagnosis.[Conclusions]Clinically,for patients who were with manifestations of chest distress and edema in lower limbs,ECG features of low voltage in limb leads,poor R-wave progression and pseudoinfarction,myocardial hypertrophy with myocardial ground-glass opacity in echocardiography and a characteristic CMR of diffuse subendocardial delayed gadolinium enhancement,CA should be considered even without the pathological proof.Non-invasive clinical methods,like serum free light chain,pseudoinfarction and LA size,can indicate the prognosis of CA,and CMR has a better diagnostic accuracy. |
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