Kristen Arnt V, Perz Jolanta B, Schonland Stefan O, Hegenbart Ute, Schnabel Philipp A, Kristen Joern H, Goldschmidt Hartmut, Katus Hugo A, Dengler Thomas J
Department of Cardiology, Angiology, and Respiratory Medicine, University of Heidelberg, Im Neuenheimer Feld 410, D-69120 Heidelberg, Germany.
Eur J Heart Fail. 2007 Jun-Jul;9(6-7):617-24. doi: 10.1016/j.ejheart.2007.01.012. Epub 2007 Mar 9.
Patients with cardiac amyloidosis (CA) have increased mortality.
Clinical, electrocardiographic, and echocardiographic parameters were assessed for risk-stratification of CA.
CA was confirmed by endomyocardial biopsy in 59 patients (54.8+/-1.2 years) with light-chain (n = 43) or transthyretin amyloidosis (n = 16). Six patients without CA served as controls (NCA). Clinical symptoms, electrocardiographic, and echocardiographic parameters were analyzed for prognostic significance. Of the patients with light-chain amyloidosis, 14 died and 2 underwent heart transplantation. 1-/3-year survival was 68%/63%. Survival depended on left ventricular function (LV-EF), LV mass, radius/wall thickness, septum thickness, low voltage pattern (LVP), conduction delay, NYHA class, and stem cell transplantation. A multivariate model only contained LV-EF and LVP; the beneficial effect of stem cell transplantation was cancelled out as this treatment was withheld in patients with highest cardiac risk. Survival was most limited if both risk factors occurred. Cardiac involvement in transthyretin amyloidosis showed better survival (2 deaths, 1-/3-year survival 91%/83%). Analysis of prognostic risk factor utility in all amyloid patients (light-chain and transthyretin) again revealed LVP and LV-EF, and aetiology of amyloidosis as independent survival parameters.
Prognosis of CA is poor, but aetiology of amyloid, LVP, and LV-EF allows identification of patients at highest risk of death, who may require individual treatment approaches (heart transplantation prior to causative therapy).
心脏淀粉样变性（CA）患者死亡率增加。
评估临床、心电图和超声心动图参数以对CA进行风险分层。
59例患者（54.8±1.2岁）经心内膜心肌活检确诊为CA，其中轻链型（n = 43）或转甲状腺素蛋白淀粉样变性（n = 16）。6例无CA患者作为对照（NCA）。分析临床症状、心电图和超声心动图参数的预后意义。轻链淀粉样变性患者中，14例死亡，2例接受心脏移植。1/3年生存率为68%/63%。生存率取决于左心室功能（LV-EF）、左心室质量、半径/壁厚、室间隔厚度、低电压图形（LVP）、传导延迟、纽约心脏协会（NYHA）分级和干细胞移植。多变量模型仅包含LV-EF和LVP；干细胞移植的有益作用被抵消，因为在心脏风险最高的患者中未进行这种治疗。如果两个危险因素都出现，生存率最受限制。转甲状腺素蛋白淀粉样变性的心脏受累患者生存率较好（2例死亡，1/3年生存率91%/83%）。对所有淀粉样变性患者（轻链型和转甲状腺素蛋白型）的预后危险因素效用分析再次显示LVP和LV-EF，以及淀粉样变性的病因是独立的生存参数。
CA的预后较差，但淀粉样变性的病因、LVP和LV-EF有助于识别死亡风险最高的患者，这些患者可能需要个体化治疗方法（在病因治疗前进行心脏移植）。
