Outpatient Sarcoidosis Clinic, General Hospital of Chest Diseases "Sotiria," Athens, Greece; Interstitial Lung Disease Unit, Royal Brompton Hospital, London, United Kingdom.
Department of Pathophysiology, Laiko Hospital, University of Athens Medical School, Athens, Greece.
JACC Cardiovasc Imaging. 2017 Dec;10(12):1437-1447. doi: 10.1016/j.jcmg.2016.11.019. Epub 2017 Mar 15.
The goal of this study was to assess the independent and collective diagnostic value of various modalities in cardiac sarcoidosis, delineate the role of cardiac magnetic resonance (CMR), and identify patients at risk.
Cardiac sarcoidosis is associated with increased morbidity and mortality. CMR is a key modality in the evaluation of patients with cardiac symptoms, but the complementary role of CMR to conventional tests for the diagnosis of cardiac sarcoidosis is not fully defined.
Patients (N = 321) with biopsy-proven sarcoidosis underwent conventional cardiac testing and CMR with late gadolinium enhancement (LGE) and were followed up for primary (composite of all-cause mortality, sustained ventricular tachycardia [VT] episodes, or hospitalization for heart failure) and secondary (nonsustained VT episodes) endpoints.
Cardiac sarcoidosis was diagnosed in 29.9% of patients according to the Heart Rhythm Society consensus criteria. CMR was the most sensitive and specific test (area under the curve: 0.984); it detected 44 patients with cardiac symptoms and/or electrocardiogram (ECG) abnormalities but normal echocardiogram, as well as 15 asymptomatic patients with normal baseline testing. Echocardiography added to cardiac history and ECG did not change sensitivity of the initial screening strategy (68.8% vs. 72.9%). Despite a high positive predictive value (83.9%), echocardiography had a low sensitivity (27.1%). During follow-up, 7.2% of patients reached the primary endpoint and another 3.4% reached the secondary endpoint. LGE was and independent predictor of primary endpoints (hazard ratio: 5.68; 95% CI: 1.74 to 18.49; p = 0.004). LGE, age, and baseline nonsustained VT were independent predictors of all events. In patients with cardiac symptoms and/or an abnormal ECG, CMR increased diagnostic accuracy and independently predicted primary endpoints (hazard ratio: 12.71; 95% confidence interval: 1.48 to 109.35; p = 0.021).
Of all cardiac tests, CMR was the most valuable in the diagnosis and prognosis of cardiac sarcoidosis in a general sarcoidosis population. Echocardiography had an overall limited diagnostic value as a screening test, and an abnormal study, despite a high positive predictive value, may still need confirmation with CMR.
本研究旨在评估心脏结节病中各种检查方法的独立和综合诊断价值，阐述心脏磁共振（CMR）的作用，并确定高危患者。
心脏结节病与发病率和死亡率增加相关。CMR 是评估有心脏症状患者的关键手段，但 CMR 对心脏结节病诊断的常规检查的补充作用尚未完全明确。
经活检证实患有结节病的 321 例患者接受了常规心脏检查和 CMR 检查，包括晚期钆增强（LGE），并进行了主要终点（全因死亡率、持续性室性心动过速[VT]发作或心力衰竭住院的复合终点）和次要终点（非持续性 VT 发作）随访。
根据心律学会共识标准，29.9%的患者被诊断为心脏结节病。CMR 是最敏感和特异的检查（曲线下面积：0.984）；它检测到 44 例有心脏症状和/或心电图（ECG）异常但超声心动图正常的患者，以及 15 例无症状但基线检查正常的患者。在初始筛查策略中，超声心动图联合心脏病史和心电图并未改变其敏感性（68.8%比 72.9%）。尽管阳性预测值较高（83.9%），但超声心动图的敏感性较低（27.1%）。随访期间，7.2%的患者达到主要终点，另有 3.4%的患者达到次要终点。LGE 是主要终点的独立预测因素（风险比：5.68；95%置信区间：1.74 至 18.49；p=0.004）。LGE、年龄和基线非持续性 VT 是所有事件的独立预测因素。在有心脏症状和/或心电图异常的患者中，CMR 提高了诊断准确性，并独立预测主要终点（风险比：12.71；95%置信区间：1.48 至 109.35；p=0.021）。
在一般结节病人群中，在心脏结节病的诊断和预后方面，所有心脏检查中，CMR 最有价值。超声心动图作为筛查试验总体上具有有限的诊断价值，尽管阳性预测值较高，但异常检查结果仍可能需要 CMR 确认。
