Long-term survival of a patient with multiple myeloma-associated severe cardiac AL amyloidosis after implantation of a cardioverter-defibrillator

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  • 重症AL型心アミロイドーシスによる致死性不整脈に植え込み型除細動器を装着後に長期生存している多発性骨髄腫
  • 症例報告 重症AL型心アミロイドーシスによる致死性不整脈に植え込み型除細動器を装着後に長期生存している多発性骨髄腫
  • ショウレイ ホウコク ジュウショウ ALガタシン アミロイドーシス ニ ヨル チシセイ フセイミャク ニ ウエコミ ガタジョサイドウキ オ ソウチャク ゴ ニ チョウキ セイゾン シテ イル タハツセイ コツズイシュ

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Description

Cardiac involvement is by far the most relevant factor impacting poor outcomes of patients with systemic light-chain (AL) amyloidosis. Median survival of patients with symptomatic cardiac AL amyloidosis is less than 6 months. Approximately two-thirds of these patients die suddenly due to ventricular arrhythmias and electromechanical dissociation. We report a 56-year-old female with very severe cardiac AL amyloidosis (NT-proBNP 13,355 ng/l, troponin T 0.16 μg/l, and systolic blood pressure 100 mmHg), who was successfully treated with diuretics and an implantable cardioverter-defibrillator (ICD) and has survived for more than 4 years, to date. During the 4-year period after receiving the ICD, she experienced several episodes of sustained ventricular tachycardia and ventricular fibrillation, all successfully terminated by anti-tachycardia pacing or electrical shock. The benefit of ICD for cardiac AL amyloidosis is unclear since there have been only a few reports of successful use of this therapy for patients with cardiac AL amyloidosis. Recently, new treatment options for AL amyloidosis, such as bortezomib and lenalidomide, have shown high response rates and improved outcomes. It is important to identify those cardiac amyloidosis patients who might be more likely to benefit from ICD implantation.

Journal

  • Rinsho Ketsueki

    Rinsho Ketsueki 55 (4), 450-455, 2014

    The Japanese Society of Hematology

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