Fondazione Toscana 'Gabriele Monasterio', CNR, Regione Toscana, Pisa, Italy Institute of Life Sciences, Scuola Superiore 'Sant'Anna', Pisa, Italy.
Clinical Physiology Institute, National Research Council, CNR, Pisa, Italy.
Eur Heart J Cardiovasc Imaging. 2015 Jan;16(1):74-80. doi: 10.1093/ehjci/jeu167. Epub 2014 Sep 4.
Systemic sclerosis (SSc) may induce cardiac fibrosis and systo-diastolic dysfunction. Cardiovascular magnetic resonance (CMR) can detect replacement myocardial fibrosis with late gadolinium enhancement (LGE) and interstitial myocardial fibrosis with T1 mapping techniques. The aim of the study was to detect subclinical cardiac involvement with CMR in paucisymptomatic SSc patients with no previous history of myocardial disease, comparing it with skeletal muscle remodelling.
Thirty consecutive SSc patients (mean age: 51 ± 12 years, all women) and 10 healthy controls (mean age: 48 ± 15 years, all women) underwent clinical, biohumoral assessment, and CMR. Extracellular volume fraction (ECV) was calculated from pre- and post-contrast T1 values in the myocardium and skeletal muscle. Seventeen patients (57%) were asymptomatic, 13 (43%) paucisymptomatic (effort dyspnoea). All patients had normal biventricular volumes and systolic function, while LGE was present in seven patients (23%). Myocardial ECV was significantly increased in patients with SSc (30 ± 4%) than controls (28 ± 4%, P = 0.03), as was skeletal muscle ECV (23 ± 6% vs. 18 ± 4%, P < 0.01). Myocardial ECV did not differ between patients with and without LGE (P = NS) and showed no significant correlations with clinical data, biventricular volumes, systolic, or diastolic function. Overall, myocardial ECV showed a significant correlation with skeletal muscle ECV (R = 0.58, P < 0.001).
SSc is associated not only with myocardial replacement fibrosis, as detected by LGE, but also with interstitial remodelling of the myocardium and skeletal muscles, as detected by an increased ECV also in patients with normal biventricular function, with potential diagnostic, prognostic, and therapeutic clinical implications.
系统性硬化症（SSc）可引起心脏纤维化和舒缩功能障碍。心血管磁共振（CMR）可以通过钆延迟增强（LGE）检测到替换性心肌纤维化，通过 T1 映射技术检测到间质心肌纤维化。本研究的目的是在无先前心肌疾病史的少症状 SSc 患者中使用 CMR 检测亚临床心脏受累情况，并与骨骼肌重塑进行比较。
连续纳入 30 例 SSc 患者（平均年龄：51 ± 12 岁，均为女性）和 10 例健康对照者（平均年龄：48 ± 15 岁，均为女性）进行临床、生物化学评估和 CMR 检查。通过心肌和骨骼肌的对比 T1 值计算细胞外容积分数（ECV）。17 例患者（57%）无症状，13 例患者（43%）少症状（运动性呼吸困难）。所有患者双心室容积和收缩功能均正常，7 例患者（23%）存在 LGE。SSc 患者的心肌 ECV 显著高于对照组（30 ± 4% vs. 28 ± 4%，P = 0.03），骨骼肌 ECV 也显著高于对照组（23 ± 6% vs. 18 ± 4%，P < 0.01）。LGE 患者与无 LGE 患者的心肌 ECV 无差异（P = NS），且与临床数据、双心室容积、收缩或舒张功能均无显著相关性。总体而言，心肌 ECV 与骨骼肌 ECV 呈显著相关性（R = 0.58，P < 0.001）。
SSc 不仅与 LGE 检测到的心肌替换性纤维化有关，还与心肌和骨骼肌的间质重塑有关，即使在双心室功能正常的患者中，ECV 增加也可检测到，这具有潜在的诊断、预后和治疗临床意义。
