Abstract：
BACKGROUND: Scimitar syndrome is a rare form of congenital heart disease (CHD) characterized by anomalous pulmonary venous drainage of the right lung to the inferior vena cava. We describe the presentation, diagnosis, therapeutic management and long-term follow-up of 10 pediatric patients with Scimitar Syndrome.
METHODS: We performed a retrospective observational study of all pediatric patients from our institution with scimitar syndrome (March 1996-July 2023). Patients underwent systematic evaluation including medical and family history, chest x-ray, 12-lead electrocardiogram, echocardiogram, angiography and/or computed tomography; or magnetic resonance angiography.
RESULTS: Ten patients with scimitar syndrome were included. The median age at diagnosis was 10.4 [0.1-150.2] months and the median follow-up time was 7.7 [1.3-15.3] years. Eight patients presented with aortopulmonary collateral arteries which were embolized. Two patients had dual connections to the inferior vena cava and left atrium; embolization of the inferior vena cava connection was only feasible in one of them. No patients underwent surgery of the scimitar vein. Three patients had surgical correction of CHDs. There were no deaths related to scimitar syndrome during follow-up.
CONCLUSIONS: All patients with scimitar syndrome need prompt cardiovascular evaluation and follow-up. Our study demonstrates that a conservative approach with aortopulmonary collateral artery embolization, scimitar vein embolization when dual drainage to the left atrium is identified, along with correction of concomitant CHDs might have good results in patients with scimitar syndrome in order to postpone surgical correction of the anomalous pulmonary venous return to an older age when clinically or hemodynamically indicated. Further studies with longer-term follow-up and a larger sample size are needed to more effectively determine treatment strategy.
METHODS: We performed a retrospective observational study of all pediatric patients from our institution with scimitar syndrome (March 1996-July 2023). Patients underwent systematic evaluation including medical and family history, chest x-ray, 12-lead electrocardiogram, echocardiogram, angiography and/or computed tomography; or magnetic resonance angiography.
RESULTS: Ten patients with scimitar syndrome were included. The median age at diagnosis was 10.4 [0.1-150.2] months and the median follow-up time was 7.7 [1.3-15.3] years. Eight patients presented with aortopulmonary collateral arteries which were embolized. Two patients had dual connections to the inferior vena cava and left atrium; embolization of the inferior vena cava connection was only feasible in one of them. No patients underwent surgery of the scimitar vein. Three patients had surgical correction of CHDs. There were no deaths related to scimitar syndrome during follow-up.
CONCLUSIONS: All patients with scimitar syndrome need prompt cardiovascular evaluation and follow-up. Our study demonstrates that a conservative approach with aortopulmonary collateral artery embolization, scimitar vein embolization when dual drainage to the left atrium is identified, along with correction of concomitant CHDs might have good results in patients with scimitar syndrome in order to postpone surgical correction of the anomalous pulmonary venous return to an older age when clinically or hemodynamically indicated. Further studies with longer-term follow-up and a larger sample size are needed to more effectively determine treatment strategy.
摘要：
背景：弯刀综合征是一种罕见的先天性心脏病（CHD），其特征是右肺异常肺静脉引流到下腔静脉。我们描述了演示文稿，诊断,10例尖刀综合征患儿的治疗管理和长期随访。
方法：我们对来自我们机构的所有患有弯刀综合征（1996年3月至2023年7月）的儿科患者进行了回顾性观察性研究。患者接受系统评估，包括病史和家族史，胸部X光,12导联心电图,超声心动图,血管造影和/或计算机断层扫描；或磁共振血管造影。
结果：包括10例弯刀综合征患者。诊断时的中位年龄为10.4[0.1-150.2]个月，中位随访时间为7.7[1.3-15.3]年。八名患者出现主动脉肺侧支动脉栓塞。两名患者与下腔静脉和左心房有双重连接；下腔静脉连接的栓塞仅在其中之一中可行。没有患者接受弯刀静脉手术。三名患者接受了CHD的手术矫正。随访期间无与弯刀综合征相关的死亡病例。
结论：所有患有弯刀综合征的患者都需要及时的心血管评估和随访。我们的研究表明，保守的方法与主肺侧支动脉栓塞，当发现左心房双重引流时，弯刀静脉栓塞，在有弯刀综合征的患者中,伴随合并CHD的矫正可能取得良好的效果,以便在有临床或血液动力学指示的情况下,将肺静脉异常回流的手术矫正推迟到年龄较大的患者.需要进行更长期随访和更大样本量的进一步研究，以更有效地确定治疗策略。
方法：我们对来自我们机构的所有患有弯刀综合征（1996年3月至2023年7月）的儿科患者进行了回顾性观察性研究。患者接受系统评估，包括病史和家族史，胸部X光,12导联心电图,超声心动图,血管造影和/或计算机断层扫描；或磁共振血管造影。
结果：包括10例弯刀综合征患者。诊断时的中位年龄为10.4[0.1-150.2]个月，中位随访时间为7.7[1.3-15.3]年。八名患者出现主动脉肺侧支动脉栓塞。两名患者与下腔静脉和左心房有双重连接；下腔静脉连接的栓塞仅在其中之一中可行。没有患者接受弯刀静脉手术。三名患者接受了CHD的手术矫正。随访期间无与弯刀综合征相关的死亡病例。
结论：所有患有弯刀综合征的患者都需要及时的心血管评估和随访。我们的研究表明，保守的方法与主肺侧支动脉栓塞，当发现左心房双重引流时，弯刀静脉栓塞，在有弯刀综合征的患者中,伴随合并CHD的矫正可能取得良好的效果,以便在有临床或血液动力学指示的情况下,将肺静脉异常回流的手术矫正推迟到年龄较大的患者.需要进行更长期随访和更大样本量的进一步研究，以更有效地确定治疗策略。
