van der Velde Nikki, Poleij Anne, Lenzen Mattie J, Budde Ricardo P J, Brabander Tessa, Miedema Jelle R, Schinkel Arend F L, Michels Michelle, Hirsch Alexander
Department of Cardiology, Thorax Centre, Cardiovascular Institute, Erasmus Medical Centre, Rotterdam, The Netherlands.
Department of Radiology and Nuclear Medicine, Erasmus Medical Centre, Rotterdam, The Netherlands.
Neth Heart J. 2025 Feb;33(2):55-64. doi: 10.1007/s12471-024-01925-0. Epub 2025 Jan 9.
Cardiac sarcoidosis (CS) is associated with poor prognosis, making early diagnosis and treatment important. This study evaluated the results of a diagnostic approach in patients with known sarcoidosis and suspected cardiac involvement in a tertiary centre and their long-term outcomes.
We included 180 patients with sarcoidosis and a clinical suspicion of CS. In addition to an electrocardiogram (ECG)/transthoracic echocardiogram (TTE), cardiovascular magnetic resonance imaging (CMR) and positron emission tomography (PET) were performed in 66% and 37% of the patients, respectively. The diagnosis of CS was based on the Heart Rhythm Society criteria. Follow-up was performed, and a composite endpoint of sustained ventricular tachycardia, ventricular fibrillation, aborted sudden cardiac death, heart failure hospitalisation, heart transplantation or cardiac death was used for the survival analysis.
Symptoms were present in 87% of the patients, and ECG/TTE abnormalities were found in 92/180 patients (51%). Using CMR and/or PET, 31/92 patients (34%) were diagnosed with CS. In 15 patients, an alternative diagnosis was found. CS was diagnosed in 11/88 patients (13%) without ECG/TTE abnormalities. During a median follow-up time of 4.4 years (interquartile range: 2.3-6.8), 11 composite endpoints occurred, more frequently in CS patients than in sarcoidosis patients without cardiac involvement (p < 0.001). Patients with ECG/TTE abnormalities at baseline had worse outcomes than those without abnormalities (p = 0.019).
CS was diagnosed in 23% of the referred sarcoidosis patients. ECG/TTE were of limited diagnostic value for screening for CS but seemed to have important prognostic value as patients with normal ECG/TTE results who did meet the diagnostic CS criteria had a very good prognosis. CMR/PET provided a good diagnostic yield and identified other cardiac diseases.
心脏结节病（CS）与不良预后相关，因此早期诊断和治疗至关重要。本研究评估了在三级医疗中心对已知结节病且怀疑有心脏受累患者的诊断方法结果及其长期预后。
我们纳入了180例结节病且临床怀疑患有CS的患者。除心电图（ECG）/经胸超声心动图（TTE）外，分别有66%和37%的患者进行了心血管磁共振成像（CMR）和正电子发射断层扫描（PET）。CS的诊断基于心律学会标准。进行了随访，并将持续性室性心动过速、心室颤动、心脏性猝死未遂、心力衰竭住院、心脏移植或心源性死亡的复合终点用于生存分析。
87%的患者有症状，180例患者中有92例（51%）发现ECG/TTE异常。使用CMR和/或PET，31/92例患者（34%）被诊断为CS。在15例患者中发现了其他诊断。11/88例（13%）无ECG/TTE异常的患者被诊断为CS。在中位随访时间4.4年（四分位间距：2.3 - 6.8年）期间，发生了11个复合终点事件，CS患者比无心脏受累的结节病患者更频繁（p < 0.001）。基线时ECG/TTE异常的患者比无异常的患者预后更差（p = 0.019）。
在转诊的结节病患者中，23%被诊断为CS。ECG/TTE对CS筛查的诊断价值有限，但似乎具有重要的预后价值，因为符合CS诊断标准但ECG/TTE结果正常的患者预后非常好。CMR/PET提供了良好的诊断率并识别了其他心脏疾病。
