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轻链心脏淀粉样变性患者的长期心电图随访。

Long-Term Electrocardiographic Follow-Up of a Patient with Light-Chain Cardiac Amyloidosis.

机构信息

Department of General Medicine and Health Science, Nippon Medical School.

Department of Cardiovascular Medicine, Nippon Medical School.

出版信息

J Nippon Med Sch. 2022 Mar 11;89(1):119-125. doi: 10.1272/jnms.JNMS.2022_89-111. Epub 2021 Mar 9.

DOI:10.1272/jnms.JNMS.2022_89-111
PMID:33692307
Abstract

Amyloid light-chain (AL) cardiac amyloidosis can cause restrictive cardiomyopathy, which has a poor prognosis. Although electrocardiography (ECG) is useful for its diagnosis and management, there are few reports on the long-term follow-up of electrocardiographic changes in affected patients. The present patient was a 62-year-old woman who visited our hospital for assessment of palpitations and lower leg edema. A chest radiograph showed cardiac enlargement, and ECG revealed sinus rhythm, first-degree atrioventricular block, low QRS voltage in the limb leads and a pseudo-myocardial infarction pattern in the precordial leads. Echocardiography revealed left ventricular hypertrophy with systolic and diastolic dysfunction. Immunoelectrophoresis demonstrated M-protein (IgGλ), and bone marrow biopsy suggested IgGλ-type plasmacytoma. Myocardial biopsy findings were compatible with cardiac amyloidosis. On the basis of these findings, we diagnosed AL cardiac amyloidosis. Melphalan-prednisolone (MP) therapy was started in conjunction with treatment for non-sustained ventricular tachycardia and congestive heart failure. Two years and 4 months later, the sinus rhythm converted to atrial tachycardia. At a follow-up examination at 4 years and 8 months, right branch block appeared. After that, intraventricular conduction worsened, and the low voltage in the limb leads was not observed. Seven years after diagnosis, she was hospitalized for treatment of pneumonia and heart failure with tachycardia. On the seventh day of hospitalization, heart rhythm changed to atrial stand-still with escaped ventricular rhythm and she died of cardiac arrest. These ECG changes are valuable information regarding the pathophysiological changes that occur in AL cardiac amyloidosis.

摘要

轻链淀粉样变(AL)心脏淀粉样变性可导致限制型心肌病,预后不良。尽管心电图(ECG)对其诊断和治疗有用,但关于受影响患者心电图变化的长期随访的报道很少。本患者为 62 岁女性,因心悸和小腿水肿就诊。胸部 X 线片显示心脏扩大,心电图显示窦性心律、一度房室传导阻滞、肢体导联低 QRS 波电压和胸前导联假性心肌梗死样图形。超声心动图显示左心室肥厚伴收缩和舒张功能障碍。免疫电泳显示 M 蛋白(IgGλ),骨髓活检提示 IgGλ 型浆细胞瘤。心肌活检结果符合心脏淀粉样变性。根据这些发现,我们诊断为 AL 心脏淀粉样变性。开始用马法兰-泼尼松(MP)联合治疗非持续性室性心动过速和充血性心力衰竭。2 年零 4 个月后,窦性心律转为房性心动过速。4 年零 8 个月随访时出现右束支阻滞。此后,室内传导恶化,肢体导联低电压不再观察到。诊断 7 年后,因肺炎和心动过速性心力衰竭住院治疗。住院第 7 天,心律变为心房静止,伴有逸搏性室性节律,她因心脏骤停而死亡。这些心电图变化是关于 AL 心脏淀粉样变性发生的病理生理变化的有价值的信息。

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