摘要
目的 分析心肌淀粉样变(cardiac amyloidosis,CA)患者的临床资料,提高对心肌淀粉样变的认识.方法 回顾性分析104例CA患者临床表现、实验室检查、影像学资料和治疗特点.结果 104例CA患者,男57例,年龄(55.3±13.2)岁;淀粉样轻链蛋白(AL型)77例(74.0%);79.8%患者B型钠尿肽(BNP)或N末端B型钠尿肽前体(NT-proBNP)升高,肌钙蛋白I升高占52.8%.最常见症状为下肢水肿(74.1%)和气短或呼吸困难(64.4%);51.0%患者纽约心功能分级Ⅲ级或Ⅳ级;合并肾脏受累41例(39.4%);心肌活检阳性率91.3%(21/23),肾脏活检阳性率90.9%(10/11);心电图30例(28.8%)出现不同程度的心律失常,62例(59.6%)出现肢导低电压,52例(50.0%)R波进展不良;超声心动图示:左心室肥厚84例(80.8%),限制性舒张功能减低60例(57.7%),心包积液35例(33.7%).行心脏核磁共振显像钆延迟强化显像10例,其中6例呈弥漫性分布,3例呈心内膜下分布,1例为局限性分布.57例(74.0%)AL型患者接受不同方案的化疗,使用的药物包括地塞米松(80.7%)、左旋苯丙氨酸氮芥(50.9%)、沙利度胺(42.1%)、环磷酰胺(31.2%)、硼替佐米(26.3%)、泼尼松(22.3%)、来那度胺(8.7%)、利妥昔单抗(3.4%)、苯丁酸氮芥(1.8%).结论 CA常合并肾脏受累、心力衰竭和心律失常,肢导低电压伴左心室肥厚是CA的特征,心肌活检和肾活检阳性率最高,心脏核磁主要特征为心内膜下为主的延迟强化显像.糖皮质激素、左旋苯丙氨酸氮芥、沙利度胺、环磷酰胺及硼替佐米是使用较多的治疗轻链型淀粉样变的化疗药物.
Objective To analyze clinical data of cardiac amyloidosis(CA)to enrich the knowledge of it.Methods Clinical manifestations,laboratory tests,image data and medical therapy were analysed retrospectively in 104 patients with CA.Results One hundred and four patients with an average age of 55.26±13.21,including 57(54.8%)males and 77(74.0%)type AL cases were enrolled.Troponin I and BNP/NT-proBNP were elevated in 52.8%and 79.8%cases,respectively.The most common symptoms were limb edema(74.1%)and shortness of breath(64.4%).Fifty three(51.0%)cases had poor heart function of NYHAⅢorⅣ,and forty one(39.4%)were complicated by renal involvement.Positive congo red dye was found in 21(91.3%)out of 23 patients who underwent endomyocardial biopsy(EMB)and 10(90.9%)out of 11 patients who received renal biopsy.Electrocardiogram showed various arrhythmias in 30(28.8%)cases,low voltage of limb lead in 62(59.6%)and poor R-wave progression in 52(50.0%).Echocardiography revealed left ventricular hypertrophy in 84(80.8%)cases,restrictive diastolic dysfunction in 60(57.7%)and pericardial effusion in 35(33.7%).Ten cases underwent cardiovascular magnetic resonance(CMR),in which six cases with global,three with subendocardial and one with localized late gadolinium enhancemen(LGE).Fifty seven(74.0%)cases underwent different chemotherapy regimens with dexamethasone(80.7%),melphalan(50.9%),thalidomide(42.1%),cyclophosphamide(31.2%),bortezomib(26.3%),prednisone(22.3%),lenalidomide(8.7%),rituximab(3.4%)and chlorambucil(1.8%).Conclusions CA is frequently complicated with renal involvement,heart failure,arrhythmia and characterized by low voltage in limb leads combined with left ventricular hypertrophy.EMB and renal biopsy show the highest positive rate of amyloidosis among all the biopsy.Myocardial late gadolinium enhancement pronounced in the subendocardial layers in CA.Chemotherapy with glucocorticoid,melphalan,thalidomide,cyclophosphamide or bortezomib are the main options in the CA patients with type AL.
作者
冀晋
方理刚
方全
朱文玲
Ji Jin;Fang Ligang;Fang Quan;Zhu Wenling(Department of Cardiology,Peking Union Medical College Hospital,Peking Union Medical College,Chinese Academy of Medical Sciences,Beijing 100730,China)
关键词
淀粉样变
心肌
回顾性研究
Amyloidosis
Myocardium
Retrospective studies
