Dorniak Karolina, Gogulska Zuzanna, Viti Alessandro, Glińska Anna, Kulawiak-Gałąska Dorota, Fijałkowska Jadwiga, Wojteczek Anna, Wojtowicz Dagmara, Sienkiewicz Katarzyna, Hellmann Marcin, Smoleńska Żaneta
Department of Cardiac Diagnostics, Faculty of Medicine, Medical University of Gdańsk, 80-210 Gdańsk, Poland.
Department of Internal Medicine, Connective Tissue Diseases & Geriatrics, Medical University of Gdańsk, 80-210 Gdańsk, Poland.
Diagnostics (Basel). 2025 Feb 6;15(3):393. doi: 10.3390/diagnostics15030393.
: Cardiac involvement (CI) in systemic sclerosis (SSc) is frequently subclinical and it can be identified in up to 80% of autopsied hearts. If present, symptoms are related to adverse prognosis, and CI represents one of the predominant causes of SSc-related mortality. : A total of 20 patients with a diagnosis of SSc were included and followed up, and 37 volunteers were included and subsequently scanned on a 1.5T MR system. : Overall, thirteen (65%) patients had one or more abnormal cardiac findings in CMR (defined as CI[+]), of which in seven (35%), baseline ECGs and standard echocardiograms were normal or unspecific. Compared to healthy volunteers, SSc patients had a lower LVEF% (56.6% vs. 61.6%; = 0.0131), longer T1 (1028.3 ms vs. 993.1 ms; = 0.0049) and T2 relaxation times (48.24 ms vs. 43 ms = 0.0011), and higher extracellular volume (ECV, 27.9% vs. 26.0%; = 0.0112). However, no difference in CMR-derived, feature-tracking GLS values between patients and healthy controls was found (-15.5[2,8] vs. -16.3[1,1], respectively, = 0.11). Over 3.4 (1.9-5.5) years, three patients (15%) died, and two others (10%) sustained major cardiac complications. : Cardiac magnetic resonance with modern quantitative techniques reveals subtle morpho-functional alterations and thus allows for early diagnosis of myocardial involvement in systemic sclerosis. Our findings emphasize the need for extended diagnostic workup in these patients and demonstrate the ability of cardiac MR to select patients requiring closer follow-up and/or treatment decisions.
系统性硬化症(SSc)中的心脏受累(CI)通常为亚临床状态,在高达80%的尸检心脏中可被发现。若存在心脏受累,症状与不良预后相关,且CI是SSc相关死亡的主要原因之一。共有20例诊断为SSc的患者被纳入并进行随访,37名志愿者被纳入并随后在1.5T磁共振系统上进行扫描。总体而言,13例(65%)患者在心脏磁共振成像(CMR)中有一项或多项心脏异常表现(定义为CI阳性),其中7例(35%)患者的基线心电图和标准超声心动图正常或无特异性。与健康志愿者相比,SSc患者的左心室射血分数(LVEF)百分比更低(56.6%对61.6%;P = 0.0131),T1时间更长(1028.3毫秒对993.1毫秒;P = 0.0049)以及T2弛豫时间更长(48.24毫秒对43毫秒,P = 0.0011),细胞外容积(ECV)更高(27.9%对26.0%;P = 0.0112)。然而,患者与健康对照之间在CMR衍生的特征追踪全心纵向应变(GLS)值方面未发现差异(分别为-15.5[2,8]对-16.3[1,1],P = 0.11)。在3.4(1.9 - 5.5)年期间,3例患者(15%)死亡,另外2例(10%)发生了严重心脏并发症。采用现代定量技术的心脏磁共振成像可揭示细微的形态功能改变,从而能够早期诊断系统性硬化症的心肌受累情况。我们的研究结果强调了对这些患者进行全面诊断检查的必要性,并证明了心脏磁共振成像在筛选需要密切随访和/或治疗决策的患者方面的能力。