Blankstein Ron, Achenbach Stephan
Department of Medicine (Cardiovascular Division), Brigham and Women's Hospital, Boston, MA, USA.
Department of Medicine (Cardiovascular Division), Harvard Medical School, Boston, MA, USA.
Herz. 2023 Oct;48(5):366-371. doi: 10.1007/s00059-023-05208-z. Epub 2023 Sep 8.
Cardiac involvement is clinically apparent in approximately 5% of all patients with systemic sarcoidosis, whereas evidence of cardiac involvement by imaging studies can be found in approximately 20% of cases. Occasionally, isolated cardiac sarcoidosis is encountered and is the only sign of the disease. The most frequent cardiac manifestations of the multifocal granulomatous inflammation include atrioventricular (AV) blocks and other conduction disorders, ventricular arrhythmias, sudden cardiac death and left and right ventricular wall disorders. Accordingly, symptoms that should raise suspicion include palpitations, lightheadedness and syncope. The diagnostic approach to cardiac sarcoidosis is not straightforward. Typical echocardiographic findings include regional thinning and contraction abnormalities particularly in basal, septal and lateral locations. Infrequently, myocardial hypertrophy may be present; however, the sensitivity of echocardiography is low and cardiac sarcoidosis can be present even when an echocardiogram is unrevealing. Cardiac magnetic resonance imaging (MRI) frequently shows late gadolinium enhancement (LGE) in a multifocal pattern often involving the basal septum and lateral walls. The sensitivity and specificity of MRI for detecting cardiac sarcoidosis are high. Fluorodeoxyglucose positron emission tomography (FDG-PET) plays an important role in the diagnostic algorithm due to its ability to visualize focal inflammatory activity both in the myocardium and in extracardiac locations. This may help target the optimal location for biopsy in order to obtain histologic proof of sarcoidosis and can also be used to follow the response to anti-inflammatory treatment. Notably, the sensitivity of endomyocardial biopsy is poor due to the patchy nature of myocardial involvement. In clinical practice, either histologic evidence of noncaseating granulomas from the myocardium or evidence from extracardiac tissue in combination with typical cardiac imaging findings are required to establish the diagnosis.
在所有系统性结节病患者中,约5%有明显的心脏受累临床表现,而影像学研究发现心脏受累证据的病例约占20%。偶尔会遇到孤立性心脏结节病,且是该病的唯一表现。多灶性肉芽肿性炎症最常见的心脏表现包括房室传导阻滞和其他传导障碍、室性心律失常、心源性猝死以及左右心室壁病变。因此,应引起怀疑的症状包括心悸、头晕和晕厥。心脏结节病的诊断方法并不简单。典型的超声心动图表现包括局部变薄和收缩异常,特别是在心底、室间隔和侧壁部位。心肌肥厚较少见;然而,超声心动图的敏感性较低,即使超声心动图未显示异常,也可能存在心脏结节病。心脏磁共振成像(MRI)常显示钆延迟强化(LGE)呈多灶性,常累及基底室间隔和侧壁。MRI检测心脏结节病的敏感性和特异性都很高。氟脱氧葡萄糖正电子发射断层扫描(FDG-PET)在诊断流程中发挥重要作用,因为它能够显示心肌和心脏外部位的局灶性炎症活动。这有助于确定活检的最佳部位以获得结节病的组织学证据,也可用于观察抗炎治疗的反应。值得注意的是,由于心肌受累呈斑片状,心内膜活检的敏感性较差。在临床实践中,确诊需要心肌非干酪样肉芽肿的组织学证据或心脏外组织的证据以及典型的心脏影像学表现。