Department of Neurology, Autonomic Laboratory, HELIOS Klinikum Wuppertal, University of Witten/Herdecke, Heusnerstr 40, D-42283 Wuppertal, Germany.
J Neurol Neurosurg Psychiatry. 2010 Mar;81(3):339-43. doi: 10.1136/jnnp.2008.168484. Epub 2009 Aug 16.
Postural orthostatic tachycardia syndrome (POTS) is a disorder of orthostatic intolerance characterised by excessive tachycardia of unknown aetiology. Whether this condition involves abnormal cardiac sympathetic innervation or function remains elusive. Meta-iodobenzylguanidine (MIBG) resembles guanethidine and is a pharmacologically inactive analogue of norepinephrine, which is similarly metabolised in noradrenergic neurons. MIBG myocardial scintigraphy is used clinically to estimate local myocardial sympathetic nerve damage in some forms of heart disease and autonomic neuropathy. The objective of this study was to evaluate cardiac sympathetic innervation in patients with POTS.
20 patients with POTS were studied using (123)I-MIBG-single photon emission computed tomography, standardised autonomic testing, assessment of catecholamine plasma levels and sympathetic skin response.
In four POTS patients (20.0%), myocardial MIBG uptake was markedly decreased. The mean heart to mediastinum ratio was reduced to 1.22+/-0.08 compared with the normal range of >1.7. No correlation was found between myocardial MIBG uptake and degree of postural tachycardia, baroreflex sensitivity, catecholamine plasma levels or other autonomic parameters. Sympathetic skin responses were normal in all patients.
These findings suggest that POTS may be, in part, a manifestation of autonomic cardiac neuropathy. MIBG myocardial scintigraphy may be helpful to distinguish patients with neuropathic POTS from patients with orthostatic intolerance of other origin.
体位性心动过速综合征(POTS)是一种直立不耐受疾病,其特征为原因不明的心动过速。这种情况是否涉及异常心脏交感神经支配或功能仍不清楚。间位碘苄胍(MIBG)类似于胍乙啶,是去甲肾上腺素的药理学上无活性类似物,在去甲肾上腺素能神经元中也被类似代谢。MIBG 心肌闪烁显像术用于临床评估某些形式心脏病和自主神经病中局部心肌交感神经损伤。本研究的目的是评估 POTS 患者的心脏交感神经支配。
使用 (123)I-MIBG-单光子发射计算机断层扫描、标准化自主测试、儿茶酚胺血浆水平评估和交感皮肤反应对 20 例 POTS 患者进行研究。
在 4 例 POTS 患者(20.0%)中,心肌 MIBG 摄取明显减少。心脏与纵隔的比值降至 1.22±0.08,而正常范围>1.7。心肌 MIBG 摄取与体位性心动过速程度、压力反射敏感性、儿茶酚胺血浆水平或其他自主参数之间无相关性。所有患者的交感皮肤反应均正常。
这些发现表明 POTS 可能部分是自主心脏神经病的表现。MIBG 心肌闪烁显像术有助于将神经源性 POTS 患者与其他来源直立不耐受患者区分开来。