Division of Cardiovascular Diseases, Mayo Clinic, Rochester, Minnesota 55905, USA.
JACC Cardiovasc Imaging. 2010 Feb;3(2):155-64. doi: 10.1016/j.jcmg.2009.09.023.
Our aim was to evaluate the role and mechanism of late gadolinium enhancement (LGE) cardiac magnetic resonance (CMR) in identifying cardiac amyloidosis (CA) and to investigate associations between LGE and clinical, morphologic, functional, and biochemical features.
CA can be challenging to diagnose by echocardiography. Recent studies have demonstrated an emerging role for LGE-CMR.
LGE-CMR was performed in 120 patients with amyloidosis. Cardiac histology was available in 35 patients. The remaining 85 patients were divided into those with and without echocardiographic evidence of CA.
Of the 35 patients with histologically verified CA, abnormal LGE was present in 34 (97%) patients and increased echocardiographic left ventricular wall thickness in 32 (91%) patients. Global transmural or subendocardial LGE (83%) was most common and was associated with greater interstitial amyloid deposition (p = 0.03). Suboptimal myocardial nulling (8%) and patchy focal LGE (6%) were also observed. LGE distribution matched the deposition pattern of interstitial amyloid. Among patients without cardiac histology, LGE was present in 86% of those with evidence of CA by echocardiography and in 47% of those without evidence of CA by echocardiography. In patients without echocardiographic evidence of CA, the presence of LGE was associated with worse clinical, electrocardiographic (ECG), and cardiac biomarker profiles. In all patients, LGE presence and pattern was associated with New York Heart Association functional class, ECG voltage, left ventricular mass index, right ventricular wall thickness, troponin-T, and B-type natriuretic peptide levels.
LGE is common in CA and detects interstitial expansion from amyloid deposition. Global transmural or subendocardial LGE is most common, but suboptimal myocardial nulling and focal patchy LGE are also observed. LGE-CMR may detect early cardiac abnormalities in patients with amyloidosis with normal left ventricular thickness. The presence and pattern of LGE is strongly associated with clinical, morphologic, functional, and biochemical markers of prognosis.
本研究旨在评估晚期钆增强（LGE）心脏磁共振（CMR）在识别心脏淀粉样变性（CA）中的作用和机制，并探讨 LGE 与临床、形态、功能和生化特征之间的相关性。
心脏淀粉样变性的超声心动图诊断具有挑战性。最近的研究表明，LGE-CMR 在该疾病的诊断中具有重要作用。
对 120 例淀粉样变性患者进行了 LGE-CMR 检查。35 例患者进行了心脏组织学检查。其余 85 例患者根据是否存在超声心动图提示的 CA 分为两组。
在 35 例经组织学证实的 CA 患者中，34 例（97%）患者存在异常 LGE，32 例（91%）患者出现左心室壁增厚。最常见的是弥漫性或心内膜下的 LGE（83%），与间质淀粉样沉积较多有关（p = 0.03）。也观察到次优心肌消除（8%）和局灶性斑片状 LGE（6%）。LGE 分布与间质淀粉样沉积模式相匹配。在没有心脏组织学的患者中，超声心动图证实有 CA 的患者中 86%存在 LGE，超声心动图无 CA 的患者中 47%存在 LGE。在没有超声心动图提示 CA 的患者中，LGE 的存在与更差的临床、心电图（ECG）和心脏生物标志物谱相关。在所有患者中，LGE 的存在和模式与纽约心脏协会功能分级、心电图电压、左心室质量指数、右心室壁厚度、肌钙蛋白-T 和 B 型利钠肽水平相关。
LGE 在 CA 中很常见，可检测到由淀粉样沉积引起的间质扩张。最常见的是弥漫性或心内膜下 LGE，但也观察到次优心肌消除和局灶性斑片状 LGE。LGE-CMR 可能检测到左心室厚度正常的淀粉样变性患者的早期心脏异常。LGE 的存在和模式与临床、形态、功能和生化预后标志物密切相关。
