Cardiology Department, Onassis Cardiac Surgery Center, 50 Esperou St, 175-61, P. Faliro, Athens, Greece.
Cardiovascular Department, Cardiac MR Center of the CHUV, Centre Hospitalier Universitaire Vaudois-CHUV, Lausanne, Switzerland.
Semin Arthritis Rheum. 2017 Aug;47(1):79-85. doi: 10.1016/j.semarthrit.2017.03.020. Epub 2017 Mar 31.
Systemic sclerosis (SSc) is an autoimmune disease characterized by vascular dysfunction and excessive fibrosis, involving internal organs including the heart. The estimated prevalence of cardiac involvement in SSc is high and remains subclinical until the late stages. It is either primary, related to myocardial inflammation and fibrosis, or secondary, due to pulmonary arterial hypertension (SSc-PAH) or systemic hypertension, in those patients with renal involvement. Cardiovascular magnetic resonance (CMR) is a useful tool for the early assessment of cardiac involvement in SSc. It is the gold standard technique to assess ventricular volumes,ejection fraction, and in particular is very useful to reliably and non-invasively detect myocardial inflammation, early perfusion defects, and myocardial fibrosis. However, the CMR evaluation in SSc may be problematic, because of cardiac and respiratory artefacts, commonly found in these patients. Therefore, a high level of expertise is necessary for both acquisition and interpretation of CMR images in SSc.
系统性硬化症（SSc）是一种自身免疫性疾病，其特征为血管功能障碍和过度纤维化，涉及包括心脏在内的内脏器官。SSc 患者的心脏受累估计患病率较高，且在晚期前一直处于亚临床状态。其心脏受累为原发性，与心肌炎症和纤维化有关，或继发性，与肺动脉高压（SSc-PAH）或系统性高血压有关，这些患者伴有肾脏受累。心血管磁共振（CMR）是评估 SSc 患者心脏受累的一种有用工具。它是评估心室容积、射血分数的金标准技术，特别是非常有助于可靠且无创地检测心肌炎症、早期灌注缺陷和心肌纤维化。然而，由于这些患者中常见的心脏和呼吸伪影，SSc 的 CMR 评估可能存在问题。因此，在 SSc 中进行 CMR 图像的获取和解读都需要高水平的专业知识。
