| Early diagnosis of CA is difficult and the prognosis is always poor. There are few investigations about the clinical features, diagnosis and prognosis in Chinese patients with CA. This study will focus on the analysis of clinical and imaging features and the role of myocardial mechanical parameters in diagnosis and prognosis in Chinese patients with CA using echo velocity vector imaging (VVI).First, we builded up data base of 60 CA patients, including clinical features, ECG information, echocardiography and CMR imaging, from 4 hospitals of Grade Ⅲ Level A between 5,2012 and 11,2013. We found (1) The 60 CA patients, who came from 12 provinces, autonomous regions or municipalities, including Han nationality and Mongolian nationality, the Hui nationality, the Chaoxian nationality and the Uigur nationality were mainly middle-aged or elderly men. (2) CA patients were found mainly in cardiology department and other internal medicine departments; CA is easy to misdiagnosed. (3) Biopsy tissue mainly included fat, kidney and spleen; the typical pathological feature of CA was that Amyloid fibrils binded Congo red stain showed the pathognomonic apple-green birefringence under cross-polarized light microscopy. (4) clinical manifestations included exertional dyspnea (73%) and pedal edema(47%); kidney(53%) and liver(15%) were commonly involved. (5) Myocardial biomarkers increased significantly(NT-proBNP>332pg/ml in 58 cases). (6) Typical ECG characteristics included poor R wave progression (35%), Low voltage in limb leads (33%) and a pseudo infarct Q wave(30%). (7) Echo characteristics included thickening left ventricle(100%), left atrial enlargment(87%), enhanced echo of myocardial granules(92%) and diastolic disfunction, which gradually aggravated with the progress of CA. (8) The typical characteristic of CMR was LV global subendocardial late gadolinium enhancement (LGE,81%), accompanied with RV and atrials LGE. (9) Primary CA(AL-CA) was the most common subtype of CA(47%), and kidney was usually involved(86%); Low voltage in limb leads and a pseudo infarct Q wave were more often in AL-CA than in Non-AL-CA. Conclusion All these features provide scientific reference for fast recognition and accurate diagnosis of CA.Secondly, we analyzed the parameters of echocardiographic myocardial mechanics in 40 CA patients using VVI, such as LV segments, walls and global systolic endocardial(ENDO) and epicardial(EPI) longitudinal(LS), circumferential (CS), radial strain (RS), LV rotation and twist. We summarised:(1) CA resulted in an impairment of LV deformation, LSENDO in LV 16 segments and LV global walls, CSENDO and CSEPI at basal level, RS, LV ROT at basal and apical level and LV TWIST were obviously reduced than those of the contrast gourp. The result suggested that the LV subendocardial and basal myocardial cells were damaged especially.Thirdly, we compared parameters of echocardiography myocardial mechanics using VVI in the two confusing cardiopathy——AL-CA and hypertrophic cardiomyopathy (HCM) patients. We found LSENDO in LV 16 segments,6 walls and global LV were obviously decreased in AL-CA. However, those changes in HCM were variable. Basal LSENDO was sensitive(85%) and specific(85%) in differentiating CA from HCM (ROC area under the curve was 0.916). So, CA was characterised by concordently decreased LSENDO in LV 16 segments,6 walls and glabal LV, and Basal LSENDO might be a new sencitive and specific index in CA diagnose.Fourthly, in prognosis study, a total of 40 consecutive CA patients were followed up from May,2012 to March,2015. The primary end point was all-cause mortality. Clinical manifestation and ECG feature, biomarkers and standard echocardiographic parameters and LSENDO for 16 segments were tested as potential independent predictors of survival. We observed the average survival time of CA was 21.8±1.9 months and the survival period of AL-CA is shorter than oher subtype. NYHA classification, pro-BNP and septal basal LSENDO were independent predictors of CA. |
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