Cardiac amyloidosis causing cardiac dysfunction: analysis of 54 necropsy patients.
Cardiac amyloidosis causing cardiac dysfunction: analysis of 54 necropsy patients.
复制标题
心脏淀粉样变性导致心脏功能障碍：54 名尸检患者的分析。
DOI：
10.1016/0002-9149(83)90084-x
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发表时间：
1983
期刊：
影响因子：
--
通讯作者：
B. Waller
中科院分区：
文献类型：
--
作者：
W. C. Roberts;B. Waller
54例尸检患者（32例男性[59%]），年龄21 ~ 97岁（平均64岁），心肌淀粉样蛋白沉积广泛，足以引起致命的心功能障碍。慢性充血性心力衰竭（CHF） 46例（85%）。已知39例患者的CHF持续时间为1至108个月（平均18个月），25例患者（64%）持续时间≤12个月。8例非CHF患者均意外猝死。43例患者在生命的最后3个月记录了全身动脉压：所有患者的间接收缩压峰值≤130 mm Hg，舒张压< 90 mm Hg。40例患者在生命最后6个月记录的心电图均异常：35例（63%）低电压；“心肌梗死型”33例（83%）；QRS轴异常29例（73%）；心律失常29例（73%）；1、2、3度心脏传导阻滞28例（45%）；完整束支阻滞7例（18%）。在30例患者中，测量了所有12根导联的QRS振幅：15例男性的QRS振幅范围为60至197 mm（平均99）（10 mm = 1 mV）， 15例女性的QRS振幅范围为58至199 mm（平均109）。只有18例（33%）患者在世时通过非心脏器官或组织活检确诊淀粉样变性。在生活中，5例患者模拟了肥厚性心肌病，3例患者模拟了缩窄性心包疾病，4例患者模拟了冠心病（由于心绞痛）。尸检发现，这些心脏的重量在300到900克之间（平均554克），除了1颗外，其余的心脏都呈“橡胶状”，不听话。淀粉样蛋白沉积物除了存在于心肌间质（53例）和冠状动脉壁内（54例）外，所有54例患者的心内膜和46例（85%）的心内膜均可见淀粉样蛋白沉积物。43例（80%）患者心室未扩张，但54例患者双心房均扩张。14例（26%）患者存在心内血栓。当血压正常、心电图显示低电压和“心肌梗死愈合”时，任何无胸痛的老年慢性CHF患者都必须考虑心脏淀粉样变。
Clinical and morphologic findings are described in 54 necropsy patients (32 men [59%]) aged 21 to 97 years (mean 64) with cardiac amyloid deposits extensive enough to cause fatal cardiac dysfunction. Chronic congestive heart failure (CHF) was present in 46 (85%). The duration of CHF, known in 39 patients, ranged from 1 to 108 months (mean 18) and lasted ≤ 12 months in 25 patients (64%). All 8 patients without CHF died suddenly and unexpectedly. Systemic arterial pressures were recorded in the last 3 months of life in 43 patients: the peak indirect systolic pressure was ≤ 130 mm Hg and the diastolic pressure < 90 mm Hg in all. Electrocardiograms, recorded in the last 6 months of life in 40 patients, were abnormal in each: low voltage in 35 (63%); “myocardial infarction pattern” in 33 (83%); abnormal QRS axis in 29 (73%); arrhythmias in 29 (73% ); first, second, or third degree heart block in 28 (45%); and complete bundle branch block in 7 (18%). In 30 patients, the QRS amplitude in all 12 leads was measured: in the 15 men it ranged from 60 to 197 mm (mean 99) (10 mm = 1 mV) and in the 15 women from 58 to 199 mm (mean 109). Diagnosis of amyloidosis was established by biopsy of noncardiac organs or tissues during life in only 18 (33%) patients. During life the condition simulated hypertrophic cardiomyopathy in 5 patients, constrictive pericardial disease in 3, and coronary heart disease (because of angina pectoris) in 4.At necropsy, the hearts ranged in weight from 300 to 900 g (mean 554), and all but 1 had a “rubbery,” noncompliant consistency. In addition to their presence in myocardial interstitium (53 patients) and in intramural coronary arteries (54 patients), amyloid deposits were present grossly in mural endocardium in all 54 patients and in valvular endocardium in 46 (85% ). The cardiac ventricles were not dilated in 43 patients (80%), but both atria were dilated in all 54 patients. Intracardiac thrombi were present in 14 patients (26% ). Cardiac amyloidosis must be considered in any elderly patient with chronic CHF unassociated with chest pain when blood pressure is normal and the electrocardiogram discloses low voltage and a pattern of “healed myocardial infarction.”