Ali Orgun, Birgül Varan, İlkay Erdoğan, N. Tokel, A. Gürsu, M. Özkan, Mehmet Sait Aşlamaci
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引用次数: 0

摘要

目的:弯刀综合征是一种罕见的先天性心脏异常,其肺静脉引流到下腔静脉(IVC)而不是左心房,通常伴有其他心脏和肺部异常。弯刀综合征具有广泛的解剖和临床变异。本文报道5例小儿弯刀综合征的不同临床特点。材料与方法:选取2000 ~ 2020年在我院确诊的5例弯刀综合征患者。评估患者的临床表现、血管造影和外科手术以及随访情况。结果:5例患儿年龄在1.5个月~ 10岁之间,诊断为弯刀综合征;5名患者中有2名是婴儿。所有患者均有症状。1例患者有心房三裂和肺静脉狭窄,1例患者有左心房异构体和下腔静脉缺失(奇异连续性),1例患者有主动脉缩窄。一名患者有双重引流:下腔静脉和左心房伴曲流肺静脉,我们确定为弯刀型。对1例婴儿肺动脉高压患者和2例肺与全身血流比(Qp/Qs) >1.5的患者行再吻合术治疗。2例患者使用血管栓、线圈和玛瑙进行了经导管肺动脉侧支闭塞术。结论:本文对5例小儿弯刀综合征的临床表现进行了详细的评价。由于弯刀综合征具有广泛的解剖和临床变异,因此治疗应单独计划。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Çocuk Hastalarda Farklı Özellikleriyle Scimitar Sendromu: Tek Merkez Deneyimi
Purpose: Scimitar syndrome is a rare congenital cardiac anomaly in which pulmonary veins are drained to the inferior vena cava (IVC) instead of the left atrium and it is often associated with additional cardiac and pulmonary anomalies. Scimitar syndrome has a wide range of anatomic and clinical variations. In this article, the different clinical features of five pediatric patients with Scimitar syndrome are reported. Material and methods: Five patients with scimitar syndrome were diagnosed in our hospital between 2000 and 2020. The clinical findings, angiographic and surgical procedures, and follow-up of the patients are evaluated. Results: Five pediatric patients aged between 1.5 months and 10 years were diagnosed as Scimitar syndrome; two of the five patients were infants. All patients were symptomatic. One patient had cor triatriatum and pulmonary venous stenosis, one patient had left atrial isomerism and absence of the IVC (azygous continuity), and another patient had coarctation of the aorta. One patient had dual drainage: the IVC and left atrium with meandering pulmonary veins, which we determined as a Scimitar variant. One infant patient with pulmonary hypertension and two patients with pulmonary to systemic flow ratio (Qp/Qs) >1.5 were treated surgically with the reanastomosis technique. Transcatheter occlusion of the aortopulmonary collaterals was performed in two patients using vascular plugs, coils, and onyx. Conclusion: In this article, the clinical findings of five pediatric patients with Scimitar syndrome were evaluated in detailed. Treatment should be planned individually in this syndrome due to Scimitar syndrome has a wide range of anatomic and clinical variations.
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