PDF(Pubmed)
Abstract：
Cardiac amyloidosis (CA) is an infiltrative cardiomyopathy resulting from deposition of misfolded immunoglobulin light chains (AL-CA) or transthyretin (ATTR-CA) proteins in the myocardium. Survival varies between the different subtypes of amyloidosis and degree of cardiac involvement, but accurate diagnosis is essential to ensure initiation of therapeutic interventions that may slow or potentially prevent morbidity and mortality in these patients. As there are now effective treatment options for CA, identifying underlying disease pathogenesis is crucial and can be guided by multimodality imaging techniques such as echocardiography, magnetic resonance imaging, and nuclear scanning modalities. However, as use of cardiac imaging is becoming more widespread, understanding optimal applications and potential shortcomings is increasingly important. Additionally, certain imaging modalities can provide prognostic information and may affect treatment planning. In patients whom imaging remains non-diagnostic, tissue biopsy, specifically endomyocardial biopsy, continues to play an essential role and can facilitate accurate and timely diagnosis such that appropriate treatment can be started. In this review, we examine the multimodality imaging approach to the diagnosis of CA with particular emphasis on the prognostic utility and limitations of each imaging modality. We also discuss how imaging can guide the decision to pursue tissue biopsy for timely diagnosis of CA.
摘要：
心脏淀粉样变性（CA）是由错误折叠的免疫球蛋白轻链（AL-CA）或甲状腺素运载蛋白（ATTR-CA）蛋白在心肌中沉积引起的浸润性心肌病。不同亚型的淀粉样变性和心脏受累程度的生存率不同，但准确的诊断对于确保启动可能减缓或可能预防这些患者发病率和死亡率的治疗性干预措施至关重要.由于现在有有效的治疗方案，识别潜在的疾病发病机制是至关重要的，可以通过多模态成像技术，如超声心动图，磁共振成像，和核扫描模式。然而,随着心脏成像的使用越来越广泛，了解最佳应用和潜在的缺点越来越重要。此外,某些成像方式可以提供预后信息,并可能影响治疗计划.在影像学检查仍无法诊断的患者中，组织活检,特别是心内膜活检，继续发挥重要作用，可以促进准确和及时的诊断，从而可以开始适当的治疗。在这次审查中,我们研究了诊断CA的多模态成像方法，特别强调每种成像方法的预后效用和局限性。我们还讨论了影像学如何指导进行组织活检以及时诊断CA的决定。
