Wright Ben L C, Grace Andrew A, Goodman Hugh J B
Papworth Hospital, Papworth Everard.
Nat Clin Pract Cardiovasc Med. 2006 Feb;3(2):110-4; quiz 115. doi: 10.1038/ncpcardio0461.
A 49-year-old woman presented with chest pain of 10 days' duration. Initial physical examinations and laboratory investigations were normal. The patient received symptomatic treatment with beta-blockers, which continued following normal findings on coronary angiogram. About 7 months later the patient developed ventricular arrhythmias, with clinical evidence of left ventricular heart failure. Her arrhythmia symptoms persisted despite pharmacological therapy with atenolol, carvedilol and amiodarone.
Physical examination, electrocardiography, laboratory testing, serologic testing, exercise-tolerance testing, coronary angiography, chest radiography, cardiac MRI, tongue biopsy, bone-marrow biopsy, CT scan, iodine-123-labeled serum-amyloid-P-component scintigraphy.
Systemic primary amyloidosis (AL amyloidosis), with predominant cardiac involvement.
Pharmacological antiarrhythmic therapy and cardioverter-defibrillator implantation. Chemotherapy was planned but, despite intervention, the patient died before this treatment could begin.
一名49岁女性出现持续10天的胸痛。初始体格检查和实验室检查均正常。患者接受了β受体阻滞剂的对症治疗，冠状动脉造影结果正常后仍继续使用。约7个月后，患者出现室性心律失常，并伴有左心室心力衰竭的临床证据。尽管使用阿替洛尔、卡维地洛和胺碘酮进行药物治疗，但其心律失常症状仍持续存在。
体格检查、心电图、实验室检查、血清学检查、运动耐量测试、冠状动脉造影、胸部X线检查、心脏磁共振成像、舌活检、骨髓活检、CT扫描、碘-123标记血清淀粉样蛋白P成分闪烁扫描。
系统性原发性淀粉样变性（AL淀粉样变性），以心脏受累为主。
抗心律失常药物治疗和植入心脏复律除颤器。计划进行化疗，但尽管进行了干预，患者在该治疗开始前死亡。
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