Fitzgerald Benjamin Thomas, Bashford John, Newbigin Katrina, Scalia Gregory Malcolm
HeartCare Partners, Brisbane, Australia.
Wesley Hospital, Auchenflower, Brisbane, Australia.
Int J Cardiol Heart Vasc. 2017 Jan 12;14:53-57. doi: 10.1016/j.ijcha.2016.12.002. eCollection 2017 Mar.
AL amyloidosis and multiple myeloma result in extracellular deposition of insoluble fibrillary protein in tissue and organs. Untreated median survival is very poor, and even worse with cardiac involvement. Chemotherapy and peripheral blood stem cell transplantation (PBSCT) have been shown to dramatically improve survival, with hematologic remission documented. Regression of cardiac changes has previously been shown, as assessed by echocardiography (TTE) and cardiac magnetic resonance imaging (CMR). This study is a comparison of TTE and CMR in long-term survivors of cardiac amyloidosis with regression.
Four long-term survivors with cardiac amyloidosis and regression of cardiac features on TTE were identified. Mean age was 60 years and average survival was 139 months from the time of diagnosis of cardiac involvement. Statistically significant regression of the cardiac features of cardiac amyloidosis were demonstrated on TTE. In these survivors, post-PBSCT structural assessments were similar between TTE and CMR. Classical strain imaging features of cardiac amyloidosis were only present in 50%. All patients had diffuse, patchy gadolinium enhancement on CMR after PBSCT.
Treatment of cardiac amyloidosis with chemotherapy and PBSCT may result in regression of abnormalities on TTE with marked improvement in survival. Post treatment, TTE and CMR structural assessments appear similar. Gadolinium imaging suggests that microscopic residual infiltration persists despite macroscopic regression. Significant cardiac improvements with prolonged survival are seen nonetheless. Multimodality imaging has a vital role in the management of cardiac amyloidosis.
AL淀粉样变性和多发性骨髓瘤会导致组织和器官中不溶性纤维状蛋白的细胞外沉积。未经治疗的患者中位生存期很短，若伴有心脏受累则更差。化疗和外周血干细胞移植（PBSCT）已被证明可显著提高生存率，并记录到血液学缓解。先前已通过超声心动图（TTE）和心脏磁共振成像（CMR）评估显示心脏变化有所消退。本研究比较了TTE和CMR在心脏淀粉样变性长期存活且病情消退患者中的应用情况。
确定了4例心脏淀粉样变性且TTE显示心脏特征消退的长期存活者。平均年龄为60岁，自诊断心脏受累起平均生存期为139个月。TTE显示心脏淀粉样变性的心脏特征有统计学意义的消退。在这些存活者中，PBSCT后的结构评估在TTE和CMR之间相似。心脏淀粉样变性的经典应变成像特征仅在50%的患者中出现。所有患者在PBSCT后CMR上均有弥漫性、斑片状钆增强。
化疗和PBSCT治疗心脏淀粉样变性可能导致TTE上的异常消退，生存率显著提高。治疗后，TTE和CMR的结构评估似乎相似。钆成像表明，尽管宏观上病情消退，但微观上仍有残留浸润。尽管如此，仍可见心脏显著改善且生存期延长。多模态成像在心脏淀粉样变性的管理中起着至关重要的作用。
