Ortigado Matamala A, García García A, Galicia Poblet G, Jiménez Bustos J M, De Juan Sánchez C
Hospital General Universitario Guadalajara, Universidad de Alcalá de Henares, Madrid, España.
An Pediatr (Barc). 2010 Mar;72(3):205-9. doi: 10.1016/j.anpedi.2009.10.019. Epub 2010 Feb 8.
The anomalous origin of the left pulmonary branch known as pulmonary artery sling (PAS) is a rare form of vascular ring, a congenital heart disease in which the left pulmonary artery originates from the right pulmonary artery and runs posterior to the trachea, encircling it, and goes leftwards between the trachea and oesophagus to reach the left pulmonary hilum. The clinical outcome depends on the associated tracheal lesions and cardiac anomalies. The majority of patients (90%) have respiratory symptoms in the first year of life, due to tracheal stenosis (chronic stridor and wheezing), and with a high mortality rate if there is no surgical intervention. Other major congenital heart diseases are present in up to 50% of patients with this rare vascular ring. We present a case of this rare disease in an asymptomatic 3-year-old girl, without any associated congenital heart disease and the findings in the echocardiography and tomography that made the non-invasive diagnosis possible.
左肺动脉分支异常起源,即所谓的肺动脉吊带(PAS),是一种罕见的血管环形式,属于先天性心脏病,其中左肺动脉起源于右肺动脉,走行于气管后方并环绕气管,然后在气管和食管之间向左走行,到达左肺门。临床结果取决于相关的气管病变和心脏异常。大多数患者(90%)在出生后第一年出现呼吸道症状,原因是气管狭窄(慢性喘鸣和喘息),如果不进行手术干预,死亡率很高。高达50%患有这种罕见血管环的患者还存在其他主要先天性心脏病。我们报告一例3岁无症状女童患这种罕见疾病的病例,该女童无任何相关先天性心脏病,以及通过超声心动图和断层扫描做出无创诊断的结果。