Huang Shao-Chi, Wang Chao-Jan, Su Wen-Jen, Chu Jaw-Ji, Hwang Mao-Sheng
Department of Pediatrics, Chang-Gung Children's Hospital, Chang-Gung University College of Medicine, Taoyuan, Taiwan.
Cardiology. 2008;111(1):16-20. doi: 10.1159/000113421. Epub 2008 Feb 1.
Truncus arteriosus, a double aortic arch, and a cervical aortic arch are all rare cardiovascular anomalies. We experienced a unique female newborn with the rare combination of truncus arteriosus with a cervical double aortic arch, which probably resulted from abnormal persistence of the bilateral 2nd or 3rd rather than the 4th embryonic aortic arches and failure of regression of the right 8th somitic segment of the right dorsal aorta. She presented with respiratory distress soon after birth, which was initially attributed to the vascular ring and hypertensive pulmonary arteries. Our inability to relieve her respiratory compromise by surgical division of the vascular ring and main pulmonary artery banding prompted the diagnosis of left main bronchial compression caused by a posteriorly displaced dilated ascending aorta that compressed the right pulmonary artery and left main bronchus against the descending aorta. The patient then underwent successful left main bronchus stent implantation. We speculate the cervical double aortic arch is redundant in nature and is a loose ring that may not cause tracheal compression. Nevertheless, a posteriorly displaced dilated ascending aorta in patients with truncus arteriosus may compress the right pulmonary artery and the main bronchus on the side of the aortic arch against the descending aorta.
动脉干、双主动脉弓和颈主动脉弓均为罕见的心血管畸形。我们接诊了一名独特的女婴，她患有动脉干合并颈双主动脉弓这种罕见的组合畸形，这可能是由于双侧第2或第3而非第4对胚胎主动脉弓异常持续存在，以及右背主动脉第8体节段未退化所致。她出生后不久即出现呼吸窘迫，最初认为是血管环和高血压性肺动脉所致。我们通过手术切断血管环和主肺动脉束带无法缓解她的呼吸功能不全，这促使我们诊断为由后移位扩张的升主动脉压迫右肺动脉和左主支气管使其紧贴降主动脉导致的左主支气管受压。该患者随后成功接受了左主支气管支架植入术。我们推测颈双主动脉弓本质上是多余的，是一个松弛的环，可能不会导致气管受压。然而，动脉干患者中后移位扩张的升主动脉可能会将右肺动脉和主动脉弓一侧的主支气管压向降主动脉。
