Abstract：
BACKGROUND: In systemic light-chain (AL) amyloidosis, cardiac involvement portends poor outcomes.
OBJECTIVE: The authors\' objectives were to detect early myocardial alterations, to analyze longitudinal changes with therapy, and to predict major adverse cardiac events (MACE) in participants with AL amyloidosis using cardiac magnetic resonance imaging (MRI).
METHODS: Recently diagnosed participants were prospectively enrolled. AL amyloidosis with and without cardiomyopathy (AL-CMP, AL-non-CMP) were defined based on abnormal cardiac biomarkers and wall thickness. MRI was performed at baseline, 6 months in all participants, and 12 months in participants with AL-CMP. MACE were defined as all-cause death, heart failure hospitalization, and cardiac transplantation. Mayo stage was based on troponin T, N-terminal pro-B-type natriuretic peptide, and difference in free light chains.
RESULTS: This study included 80 participants (median age 62 years, 58% men). Extracellular volume (ECV) was abnormal (>32%) in all participants with AL-CMP and in 47% of those with AL-non-CMP. ECV tended to increase at 6 months (median +2%; AL-CMP P = 0.120; AL-non-CMP P = 0.018) and returned to baseline values at 12 months in participants with AL-CMP. Global longitudinal strain (GLS) improved at 6 months (median -0.6%; P = 0.048) and 12 months (median -1.2%; P < 0.001) in participants with AL-CMP. ECV and GLS were strongly associated with MACE (P < 0.001) and improved the prognostic value when added to Mayo stage (P ≤ 0.002). No participant with ECV ≤32% had MACE, while 74% of those with ECV >48% had MACE.
CONCLUSIONS: In patients with systemic AL amyloidosis, ECV detects subclinical myocardial alterations. With therapy, ECV tends to increase at 6 months and returns to values unchanged from baseline at 12 months, whereas GLS improves at 6 and 12 months in participants with AL-CMP. ECV and GLS offer additional prognostic performance over Mayo stage. (Molecular Imaging of Primary Amyloid Cardiomyopathy [MICA]; NCT02641145).
OBJECTIVE: The authors\' objectives were to detect early myocardial alterations, to analyze longitudinal changes with therapy, and to predict major adverse cardiac events (MACE) in participants with AL amyloidosis using cardiac magnetic resonance imaging (MRI).
METHODS: Recently diagnosed participants were prospectively enrolled. AL amyloidosis with and without cardiomyopathy (AL-CMP, AL-non-CMP) were defined based on abnormal cardiac biomarkers and wall thickness. MRI was performed at baseline, 6 months in all participants, and 12 months in participants with AL-CMP. MACE were defined as all-cause death, heart failure hospitalization, and cardiac transplantation. Mayo stage was based on troponin T, N-terminal pro-B-type natriuretic peptide, and difference in free light chains.
RESULTS: This study included 80 participants (median age 62 years, 58% men). Extracellular volume (ECV) was abnormal (>32%) in all participants with AL-CMP and in 47% of those with AL-non-CMP. ECV tended to increase at 6 months (median +2%; AL-CMP P = 0.120; AL-non-CMP P = 0.018) and returned to baseline values at 12 months in participants with AL-CMP. Global longitudinal strain (GLS) improved at 6 months (median -0.6%; P = 0.048) and 12 months (median -1.2%; P < 0.001) in participants with AL-CMP. ECV and GLS were strongly associated with MACE (P < 0.001) and improved the prognostic value when added to Mayo stage (P ≤ 0.002). No participant with ECV ≤32% had MACE, while 74% of those with ECV >48% had MACE.
CONCLUSIONS: In patients with systemic AL amyloidosis, ECV detects subclinical myocardial alterations. With therapy, ECV tends to increase at 6 months and returns to values unchanged from baseline at 12 months, whereas GLS improves at 6 and 12 months in participants with AL-CMP. ECV and GLS offer additional prognostic performance over Mayo stage. (Molecular Imaging of Primary Amyloid Cardiomyopathy [MICA]; NCT02641145).
摘要：
背景：在系统性轻链（AL）淀粉样变性中，心脏受累预示着不良结局.
目的：作者的目的是检测早期心肌改变，为了分析治疗的纵向变化，并使用心脏磁共振成像（MRI）预测AL淀粉样变性参与者的主要不良心脏事件（MACE）。
方法：最近诊断的参与者被前瞻性纳入。有和没有心肌病的AL淀粉样变性(AL-CMP，AL-non-CMP）是根据异常的心脏生物标志物和壁厚定义的。MRI在基线时进行,在所有参与者中6个月，和12个月参加AL-CMP。MACE被定义为全因死亡，心力衰竭住院，和心脏移植。Mayo阶段是基于肌钙蛋白T,N末端B型利钠肽原,和自由轻链的差异。
结果：这项研究包括80名参与者（中位年龄62岁，58%男性）。所有AL-CMP参与者的细胞外体积（ECV）异常（>32％），而AL-非CMP参与者中有47％。ECV在6个月时趋于增加(中位数+2%;AL-CMPP=0.120;AL-non-CMPP=0.018),并在12个月时恢复到基线值。AL-CMP参与者的全球纵向应变（GLS）在6个月（中位数-0.6％；P=0.048）和12个月（中位数-1.2％；P<0.001）时有所改善。ECV和GLS与MACE密切相关（P<0.001），当加入Mayo分期（P≤0.002）时，可改善预后价值。ECV≤32%的参与者没有MACE，而74％的ECV>48％的人患有MACE。
结论：在系统性AL淀粉样变性患者中，ECV检测亚临床心肌改变。通过治疗，ECV倾向于在6个月时增加，并在12个月时恢复到基线值不变，而AL-CMP参与者的GLS在6个月和12个月时有所改善。ECV和GLS在Mayo阶段提供额外的预后表现。（原发性淀粉样心肌病的分子成像[MICA]；NCT02641145）。
目的：作者的目的是检测早期心肌改变，为了分析治疗的纵向变化，并使用心脏磁共振成像（MRI）预测AL淀粉样变性参与者的主要不良心脏事件（MACE）。
方法：最近诊断的参与者被前瞻性纳入。有和没有心肌病的AL淀粉样变性(AL-CMP，AL-non-CMP）是根据异常的心脏生物标志物和壁厚定义的。MRI在基线时进行,在所有参与者中6个月，和12个月参加AL-CMP。MACE被定义为全因死亡，心力衰竭住院，和心脏移植。Mayo阶段是基于肌钙蛋白T,N末端B型利钠肽原,和自由轻链的差异。
结果：这项研究包括80名参与者（中位年龄62岁，58%男性）。所有AL-CMP参与者的细胞外体积（ECV）异常（>32％），而AL-非CMP参与者中有47％。ECV在6个月时趋于增加(中位数+2%;AL-CMPP=0.120;AL-non-CMPP=0.018),并在12个月时恢复到基线值。AL-CMP参与者的全球纵向应变（GLS）在6个月（中位数-0.6％；P=0.048）和12个月（中位数-1.2％；P<0.001）时有所改善。ECV和GLS与MACE密切相关（P<0.001），当加入Mayo分期（P≤0.002）时，可改善预后价值。ECV≤32%的参与者没有MACE，而74％的ECV>48％的人患有MACE。
结论：在系统性AL淀粉样变性患者中，ECV检测亚临床心肌改变。通过治疗，ECV倾向于在6个月时增加，并在12个月时恢复到基线值不变，而AL-CMP参与者的GLS在6个月和12个月时有所改善。ECV和GLS在Mayo阶段提供额外的预后表现。（原发性淀粉样心肌病的分子成像[MICA]；NCT02641145）。
