PDF(Pubmed)
Abstract：
Scimitar syndrome is a congenital disorder characterized by partial anomalous pulmonary venous return to the inferior vena cava (IVC). Clinical manifestation in adulthood is infrequent. The management approach has not been universally accepted and may be challenging. Individually tailored and multidisciplinary team-based decisions are often necessary. We present the case of a symptomatic patient diagnosed with complex congenital heart disease, including scimitar syndrome and atrial septal defect at the age of 50 years. Surgical repair, involving scimitar vein implantation in the left atrium using a pericardial patch, was performed. Despite surgical correction, dyspnea persisted, and hemoptysis developed. A diagnostic workup revealed a critical stenosis of the re-inserted vein. This was successfully treated by percutaneous intervention with stent implantation. The patient has remained asymptomatic since the procedure. Scimitar syndrome can be first diagnosed in adulthood, and clinical manifestations can vary. Diagnostic workup necessitates a CT angiogram, magnetic resonance scan, and catheterization in selected cases. Stenoses of re-implanted pulmonary veins (PVs) can develop years after surgical correction, and hemoptysis may serve as a warning symptom prompting further PV imaging. Percutaneous vascular intervention using a stent is warranted in symptomatic cases and can lead to long-term success.
摘要：
弯刀综合征是一种先天性疾病，其特征是部分肺静脉异常回流到下腔静脉（IVC）。成年后的临床表现很少见。管理方法尚未被普遍接受，可能具有挑战性。个性化和多学科的团队决策往往是必要的。我们介绍了一个诊断为复杂先天性心脏病的有症状患者的病例，包括50岁时的弯刀综合征和房间隔缺损。手术修复,涉及使用心包补片在左心房植入弯刀静脉，已执行。尽管进行了手术矫正,呼吸困难持续存在，出现咯血.诊断检查显示重新插入的静脉严重狭窄。通过经皮介入支架植入成功治疗。患者自手术以来一直无症状。弯刀综合征可以在成年期首次诊断，临床表现可能有所不同。诊断检查需要CT血管造影,磁共振扫描，和导管插入在选定的情况下。重新植入的肺静脉（PV）狭窄可以在手术矫正后数年发展，咯血可能作为提示进一步PV成像的警告症状。在有症状的情况下，使用支架进行经皮血管介入治疗是必要的，并且可以带来长期的成功。
