Scully Michael S, Wessman Dylan E, McKee James M, Francisco Gregory M, Nayak Keshav R, Kobashigawa Jon A
Department of Cardiology, Naval Medical Center San Diego, 34730 Bob Wilson Drive, Suite 303, San Diego, CA 92134-3303.
Department of Radiology, Naval Medical Center San Diego, 34730 Bob Wilson Drive, Suite 303, San Diego, CA 92134-3303.
Mil Med. 2017 Mar;182(3):e1858-e1860. doi: 10.7205/MILMED-D-16-00122.
Cardiac involvement by light-chain (AL) amyloid occurs in up to 50% of patients with primary AL amyloidosis. The prognosis of amyloid heart disease is poor with 1-year survival rates of 35 to 40%. Historically, heart transplantation was considered controversial for patients with AL amyloid cardiomyopathy (CM) given the systemic nature of the disease and poor survival. We present a case report of an active duty service member diagnosed with advanced cardiac amyloid who underwent total artificial heart transplant as a bridge to heart transplant and eventual autologous stem cell transplant.
A 47-year-old active duty male initially evaluated for atypical chest pain was found to have severe concentric left ventricular hypertrophy on echocardiogram but normal voltage on electrocardiogram. Cardiac magnetic resonance imaging, laboratory studies, and bone marrow biopsy established the diagnosis of cardiac amyloidosis. At the time of diagnosis, the patient's prognosis was very poor with a median survival of 5 months on the basis of the Mayo Clinic revised prognostic staging system for amyloidosis. The patient developed rapidly progressive left ventricular dysfunction and heart failure leading to cardiac arrest. The patient received a total artificial heart as a bridge to orthotopic heart and kidney transplantation and eventual stem cell transplant. He continues to be in remission and has a fair functional capacity without restriction in activities of daily living or moderate exercise.
Amyloid CM is a rare and devastating disease. The natural course of the disease has made heart transplant in these patients controversial. Modern advancements in chemotherapies and advanced heart failure treatments have improved outcomes for select patients with AL amyloid CM undergoing heart transplantation. There is ongoing research seeking improvement in treatment options and outcomes for patients with this deadly disease.
轻链（AL）淀粉样变性累及心脏的情况在原发性AL淀粉样变性患者中发生率高达50%。淀粉样心脏病的预后较差，1年生存率为35%至40%。从历史上看，鉴于疾病的全身性和较差的生存率，心脏移植对于AL淀粉样心肌病（CM）患者一直存在争议。我们报告一例现役军人病例，该患者被诊断为晚期心脏淀粉样变性，接受了全人工心脏移植作为心脏移植和最终自体干细胞移植的过渡。
一名47岁现役男性因非典型胸痛接受初步评估，超声心动图显示严重的同心性左心室肥厚，但心电图电压正常。心脏磁共振成像、实验室检查和骨髓活检确诊为心脏淀粉样变性。根据梅奥诊所修订的淀粉样变性预后分期系统，在诊断时，患者的预后非常差，中位生存期为5个月。患者出现快速进展的左心室功能障碍和心力衰竭，导致心脏骤停。患者接受了全人工心脏作为原位心脏和肾脏移植以及最终干细胞移植的过渡。他持续缓解，功能能力良好，日常生活活动或适度运动不受限制。
淀粉样CM是一种罕见且具有破坏性的疾病。该疾病的自然病程使得这些患者的心脏移植存在争议。化疗和晚期心力衰竭治疗的现代进展改善了部分接受心脏移植的AL淀粉样CM患者的预后。目前正在进行研究，以寻求改善这种致命疾病患者的治疗选择和预后。
