Sarquella-Brugada Georgia, Prada Fredy, Mortera Carlos
Department of Cardiology, Hospital Sant Joan de Déu, Avinguda de Sant Joan de Déu 2, Esplugues de Llobregat, Spain.
Cardiol Young. 2011 Jun;21(3):357-60. doi: 10.1017/S1047951111000096. Epub 2011 Feb 4.
Congenital venolobular or scimitar syndrome is a rare congenital cardiopulmonary anomaly consisting in a partial anomalous pulmonary venous drainage, lung hypoplasia, and anomalous systemic arterial supply to the lung. It can associate with other congenital disorders which will confer the clinical presentation and prognosis of these patients. In most of the cases, the therapeutic approach is partial, as anatomy allows only aberrant arterial embolisation. We present a 6-year-old girl with recurrent pulmonary infections, diagnosed as scimitar syndrome with double collector drainage to the inferior caval vein and left atrium, undergoing interventional catheterisation for complete correction of her disorder. The anomalous systemic artery supply was embolised and the anomalous venous drainage was occluded. The patient was asymptomatic during follow-up, which supports the interventional catheterisation approach as a valid therapeutic option in cases of scimitar syndrome with double venous drainage.
先天性肺静脉叶综合征或弯刀综合征是一种罕见的先天性心肺异常，包括部分肺静脉异位引流、肺发育不全以及肺的异常体循环动脉供血。它可与其他先天性疾病相关联，这将影响这些患者的临床表现和预后。在大多数情况下，治疗方法是局部性的，因为解剖结构仅允许对异常动脉进行栓塞。我们报告一名6岁反复肺部感染的女孩，诊断为弯刀综合征，有双支收集静脉向下腔静脉和左心房引流，接受介入导管治疗以完全纠正其病症。异常的体循环动脉供血被栓塞，异常的静脉引流被闭塞。患者在随访期间无症状，这支持了介入导管治疗方法作为双静脉引流型弯刀综合征病例的有效治疗选择。
