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Title: [Clinical analysis of scimitar syndrome in 6 pediatric patients].
Author: Yang J, Ding WW, Wang R, Li XF, Fan XM, Jin M.
Journal: Zhonghua Er Ke Za Zhi; 2019 Sep 02; 57(9):705-709. PubMed ID: 31530357.
Abstract:
Objective: To characterize the clinical features and outcomes of scimitar syndrome (SS) to aid the understanding of this syndrome. Methods: This retrospective study included 6 children who were diagnosed with SS at the pediatric cardiovascular center of Beijing Anzhen Hospital from January 2012 to September 2018. SS was diagnosed by echocardiography and confirmed by cardiac computed tomography angiography(CTA) or surgery. All data were collected to analyze the clinical and imaging characteristics and prognosis. Results: Among the 6 SS children (aged 2 months to 15 years; 5 males) weighed 5.6-17.1 kg. Three cases were infant type, the clinical manifestations were recurrent respiratory tract infection with growth retardation, including 2 cases with severe pulmonary hypertension, while 3 cases with adult type, were asymptomatic. Cardiac CTA imaging showed that the right single or all pulmonary veins descended through the diaphragm and converged into the inferior vena cava. One case was isolated infracardiac partial anomalous pulmonary venous connection (PAPVC) without other malformations. The remaining 5 cases complicated with atrial septal defect, different vascular and trachea malformations as well as spinal malformations. Vascular malformations included pulmonary veins stenosis, abnormal origin of pulmonary artery branches, collateral branches of systemic artery supplying local lung tissue, and persistent left superior vena cava. The treatment varied according to the specific location of anomalous pulmonary venous connection, the degree of pulmonary hypertension and the severity of clinical symptoms. Four cases underwent one-stage radical surgery, one case accepted intervention to occlude the collateral artery which was supplying the right lower lung and received stage Ⅱ radical surgery half a year later, and the remaining one case died from pulmonary hypertension crisis preoperation. Conclusions: Isolated SS can easily miss diagnosis due to mild clinical symptoms. Patients with complicated malformations can benefit from combination therapy. SS associated with severe pulmonary hypertension can lead to early death. Therefore, early diagnosis and appropriate treatment can improve the prognosis of patients. 目的： 探讨镰刀综合征（SS）的特点并分析其临床转归，旨在提高对该综合征的认识。 方法： 收集2012年1月至2018年9月就诊于首都医科大学附属北京安贞医院儿童心脏中心，经超声心动图诊断为SS，并经外科手术或心脏血管造影断层扫描成像（CTA）证实的患儿共6例，分析其临床及影像学特点、预后转归。 结果： 6例SS患儿中男5例、女1例，年龄2月龄~15岁，体重5.6~17.1 kg。婴儿型3例，临床表现为反复呼吸道感染，生长发育受限，其中2例合并重度肺动脉高压。成人型3例，无明显临床症状，心脏CTA影像表现为右侧单支或全部肺静脉穿越膈肌下行，汇入下腔静脉。其中1例为单纯心下型部分性肺静脉异位引流（PAPVC），不合并其他畸形。余5例除合并房间隔缺损外，分别合并不同的血管、气管畸形以及脊柱畸形。合并的血管畸形包括肺静脉狭窄，肺动脉分支起源异常，体动脉异常侧支供应局部肺组织，永存左上腔静脉。6例患儿中治疗方法依肺静脉异位引流的具体部位、肺动脉高压的程度及临床症状的严重程度而不同。本组6例中4例行Ⅰ期外科手术根治；1例Ⅰ期行介入手术封堵供应右下肺的粗大体动脉侧支血管，半年后行外科根治手术；另1例因肺动脉高压危象于术前死亡。 结论： 单纯SS临床症状轻，极易漏诊；合并复杂畸形时可采取内外科综合治疗；重度肺动脉高压时可导致患儿早期死亡。因此，早期明确诊断有助患儿及时接受适当治疗并改善预后。.

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