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活检证实的系统性肉样瘤病患者心血管磁共振的预后价值。

Prognostic value of cardiovascular magnetic resonance in patients with biopsy-proven systemic sarcoidosis.

机构信息

Department of Radiology, University Hospitals Leuven, Leuven, Belgium.

Department of Nuclear Medicine and Molecular Imaging, University Hospitals Leuven, Leuven, Belgium.

出版信息

Eur Radiol. 2020 Jul;30(7):3702-3710. doi: 10.1007/s00330-020-06765-1. Epub 2020 Mar 12.

DOI:10.1007/s00330-020-06765-1
PMID:32166494
Abstract

OBJECTIVES

As prognosis in sarcoidosis is determined by cardiac involvement, the objective was to study the added value of cardiovascular magnetic resonance (CMR) in risk stratification.

METHODS

In 114 patients (48 ± 12 years/52% male) with biopsy-proven sarcoidosis, we studied the value of clinical and CMR-derived parameters to predict future events, using sustained ventricular tachycardia, ventricular fibrillation, aborted cardiac death, implantable cardioverter-defibrillator (ICD) placement with appropriate shocks, hospitalization for heart failure, and death as composite endpoint. Median follow-up after CMR was 3.1 years (1.1-5.7 years).

RESULTS

The ejection fraction (EF) was 58.2 ± 9.1% and 54.7 ± 10.8% for left ventricle (LV) and right ventricle (RV), respectively. LV late gadolinium enhancement (LGE) was present in 40 patients (35%) involving 5.1% of the LV mass (IQR, 3.0-12.0%), with concomitant RV involvement in 12 patients (11%). T2-weighting imaging and/or T2 mapping showed active disease in 14 patients. The composite endpoint was reached in 34 patients, with 7 deaths in the LGE-positive group (17.5%), versus two deaths in the LGE-negative group (2.7%) (p = 0.015). At univariate analysis, RVEF (p = 0.009), pulmonary arterial pressure (p = 0.002), and presence of LGE (p < 0.001) and LGE (% of LV) (p < 0.001) were significant. At multivariate analysis, only presence of LGE and LGE (% of LV) was significant (both p = 0.03). At Kaplan-Meier, presence of LGE and an LGE of 3% predicted event-free survival and patient survival. We found no difference in active versus inactive disease with regard to patient survival.

CONCLUSION

Myocardial enhancement at LGE-CMR adds independent prognostic value in risk stratification sarcoidosis patients. In contrast, clinical as well as functional cardiac parameters lack discriminative power.

KEY POINTS

• Sarcoidosis often affects the heart. • Comprehensive CMR, including T2 imaging and LGE enhancement CMR, allows to depict both active and inactive myocardial damage. • Patient prognosis in sarcoidosis is determined by the presence and severity of myocardial involvement at LGE CMR.

摘要

目的

由于预后取决于心脏受累情况,因此本研究旨在探讨心血管磁共振(CMR)在风险分层中的额外价值。

方法

在 114 名经活检证实患有结节病的患者(48 ± 12 岁/52%为男性)中,我们研究了临床和 CMR 衍生参数的价值,以预测未来事件,终点包括持续性室性心动过速、心室颤动、心脏性猝死、植入式心脏复律除颤器(ICD)放电、因心力衰竭住院和死亡的复合终点。CMR 后中位随访时间为 3.1 年(1.1-5.7 年)。

结果

左心室(LV)和右心室(RV)的射血分数(EF)分别为 58.2 ± 9.1%和 54.7 ± 10.8%。40 名患者(35%)存在 LV 晚期钆增强(LGE),LV 质量的 5.1%(IQR,3.0-12.0%)有 LGE 累及,同时 12 名患者(11%)RV 也有 LGE 累及。T2 加权成像和/或 T2 映射显示 14 名患者存在活动性疾病。34 名患者达到复合终点,LGE 阳性组有 7 例死亡(17.5%),而 LGE 阴性组有 2 例死亡(2.7%)(p = 0.015)。单因素分析显示,RVEF(p = 0.009)、肺动脉压(p = 0.002)、LGE 存在(p < 0.001)和 LGE(LV 的%)(p < 0.001)均有显著意义。多因素分析显示,只有 LGE 存在和 LGE(LV 的%)有显著意义(均 p = 0.03)。Kaplan-Meier 分析显示,存在 LGE 和 LGE (LV 的%)预测无事件生存率和患者生存率。我们发现,有或无活动病变的患者生存率无差异。

结论

LGE-CMR 心肌增强可在结节病患者的风险分层中提供独立的预后价值。相比之下,临床和功能心脏参数缺乏区分能力。

关键点

  1. 结节病常累及心脏。

  2. 全面的 CMR 包括 T2 成像和 LGE 增强 CMR,可显示活性和非活性心肌损伤。

  3. 结节病患者的预后取决于 LGE-CMR 检查中心肌受累的存在和严重程度。

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