Magnetic Resonance in Transthyretin Cardiac Amyloidosis
Magnetic Resonance in Transthyretin Cardiac Amyloidosis
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DOI：
10.1016/j.jacc.2017.05.053
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发表时间：
2017-07-25
影响因子：
24
通讯作者：
Fontana, Marianna
中科院分区：
文献类型：
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作者：
Martinez-Naharro, Ana;Treibel, Thomas A.;Fontana, Marianna
背景心脏甲状腺素运载蛋白淀粉样变性（ATTR）是一种越来越被认识到的心力衰竭的原因。心脏磁共振（CMR），晚期钆增强（LGE）和T1标测，方法263例经99 mTc-DPD证实为2 ~ 3级心脏ATTR的患者，采用CMR结合细胞外容积分数（ECV）测定，对ATTR的心脏受累与预后的关系进行了研究（99 mTc-3，3-二膦酰基-1，2-丙二羧酸）心脏摄取，17例疑似心脏ATTR（1级99 mTc-DPD），12例无症状个体淀粉样蛋白生成性甲状腺素运载蛋白（TTR）突变。50例心脏轻链（AL）淀粉样变性作为疾病comparators.Results不同的心脏AL淀粉样变性，不对称的间隔左室肥厚（LVH）存在于79%的ATTR患者（70%的乙状隔和30%的反向间隔轮廓），而对称的LVH存在于18%，3%没有LVH。在心脏淀粉样变性患者中，LGE的模式总是淀粉样变性的典型模式（29%内膜下，71%透壁），包括右心室LGE（96%）。在随访期间（19 +/- 14个月），65名患者死亡。ECV与死亡率独立相关，在校正年龄、N-末端B型利钠肽前体、射血分数、E/E '和左心室质量后，ECV仍独立相关（危害比：1.164; 95%置信区间：1.066 ~ 1.271;结论：不对称性心肌肥厚，传统上与肥厚型心肌病相关，是ATTR最常见的心室重构模式。LGE成像在所有心脏ATTR患者中均为典型。ECV与淀粉样蛋白负荷相关，是该队列患者生存的独立预后因素。(C)2017年美国心脏病学院基金会。
BACKGROUND Cardiac transthyretin amyloidosis (ATTR) is an increasingly recognized cause of heart failure. Cardiac magnetic resonance (CMR), with late gadolinium enhancement (LGE) and T1 mapping, is emerging as a reference standard for diagnosis and characterization of cardiac amyloidosis.OBJECTIVES The authors used CMR with extracellular volume fraction (ECV) measurement to characterize cardiac involvement in relation to outcome in ATTR.METHODS Subjects comprised 263 patients with cardiac ATTR corroborated by grade 2 to 3 99mTc-DPD (99mTc-3,3diphosphono-1,2-propanodicarboxylic acid) cardiac uptake, 17 with suspected cardiac ATTR (grade 1 99mTc-DPD), and 12 asymptomatic individuals with amyloidogenic transthyretin (TTR) mutations. Fifty patients with cardiac light-chain (AL) amyloidosis acted as disease comparators.RESULTS Unlike cardiac AL amyloidosis, asymmetrical septal left ventricular hypertrophy (LVH) was present in 79% of patients with ATTR (70% sigmoid septum and 30% reverse septal contour), whereas symmetrical LVH was present in 18%, and 3% had no LVH. In patients with cardiac amyloidosis, the pattern of LGE was always typical for amyloidosis (29% subendocardial, 71% transmural), including right ventricular LGE (96%). During follow-up (19 +/- 14 months), 65 patients died. ECV independently correlated with mortality and remained independent after adjustment for age, N-terminal pro-B-type natriuretic peptide, ejection fraction, E/E', and left ventricular mass (hazard ratio: 1.164; 95% confidence interval: 1.066 to 1.271; p < 0.01).CONCLUSIONS Asymmetrical hypertrophy, traditionally associated with hypertrophic cardiomyopathy, was the commonest pattern of ventricular remodeling in ATTR. LGE imaging was typical in all patients with cardiac ATTR. ECV correlated with amyloid burden and was an independent prognostic factor for survival in this cohort of patients. (C) 2017 by the American College of Cardiology Foundation.