特纳综合征中的部分性肺静脉异位回流。
Partial anomalous pulmonary venous return in Turner syndrome.
机构信息
Department of Congenital Cardiology, Erasmus MC, Rotterdam, the Netherlands; Department of Radiology, Erasmus MC, Rotterdam, the Netherlands.
Department of Cardiology, UMC Radboud University Medical Center, Nijmegen, the Netherlands.
出版信息
Eur J Radiol. 2017 Oct;95:141-146. doi: 10.1016/j.ejrad.2017.07.024. Epub 2017 Aug 4.
PURPOSE
The aim of this study is to describe the prevalence, anatomy, associations and clinical impact of partial anomalous pulmonary venous return in patients with Turner syndrome.
METHODS AND RESULTS
All Turner patients who presented at our Turner clinic, between January 2007 and October 2015 were included in this study and underwent ECG, echocardiography and advanced imaging such as cardiac magnetic resonance or computed tomography as part of their regular clinical workup. All imaging was re-evaluated and detailed anatomy was described. Partial anomalous pulmonary venous return was diagnosed in 24 (25%) out of 96 Turner patients included and 14 (58%) of these 24 partial anomalous pulmonary venous return had not been reported previously. Right atrial or ventricular dilatation was present in 11 (46%) of 24 partial anomalous pulmonary venous return patients.
CONCLUSION
When studied with advanced imaging modalities and looked for with specific attention, PAPVR is found in 1 out of 4 Turner patients. Half of these patients had right atrial and/or ventricular dilatation. Evaluation of pulmonary venous return should be included in the standard protocol in all Turner patients.
目的
本研究旨在描述 Turner 综合征患者部分肺静脉异常回流的发生率、解剖结构、相关性及临床影响。
方法与结果
本研究纳入了 2007 年 1 月至 2015 年 10 月期间在我们 Turner 诊所就诊的所有 Turner 综合征患者，这些患者均接受了心电图、超声心动图及心脏磁共振或计算机断层扫描等高级影像学检查，作为其常规临床评估的一部分。所有影像学检查结果均重新评估，并详细描述了解剖结构。在 96 例 Turner 综合征患者中，有 24 例（25%）被诊断为部分肺静脉异常回流，其中 14 例（58%）此前未被报道过。在 24 例部分肺静脉异常回流患者中，有 11 例（46%）存在右心房或右心室扩张。
结论
通过使用先进的影像学检查手段，并特别注意观察，约 1/4 的 Turner 综合征患者存在肺静脉异常回流。其中一半患者存在右心房和/或右心室扩张。在所有 Turner 综合征患者中，应将肺静脉回流的评估纳入标准方案中。
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