Left pulmonary artery runs posterior to the trachea (arrowhead), and forms pulmonary artery sling. DAA, double aortic aorta dAo, descending aorta L, left pulmonary artery LAA, left aortic arch M, main pulmonary artery PAS, pulmonary artery sling R, right pulmonary artery RAA, right aortic arch T, trachea. In the pulmonary artery sling area, the trachea is pressed by left pulmonary artery immediately above the tracheal bifurcation. After the right upper lobe bronchus branches alone, the left and right bronchi branch and the trachea branching form takes on the shape of Wells classification Type IB. ( B) At the Th3 level, the right upper lobe bronchus branches alone. Right middle and lower pulmonary artery originate from right pulmonary artery (red arrow). Right upper pulmonary artery supplying S1 + S3 (A1 + A3: black arrow) originates from right pulmonary artery, and right upper pulmonary artery supplying S2 (A2: yellow arrow) originates from left pulmonary artery. ( A) Left pulmonary artery originates from right pulmonary artery, forming pulmonary artery sling and surrounding the trachea. Oesophagram at 18 days of age revealed the defect in the posterior wall of oesophagus at the level of DAA and the defect in the anterior wall at the level of PAS. Furthermore, the right A2, which is part of the right upper pulmonary artery (RUPA), showed abnormal branching of the pulmonary artery from LPA ( Figure Figure 3). Contrast-enhanced computed tomography (CT) at 16 days of age confirmed DAA ( Figure Figure 1) 1) with a concurrent PAS ( Figure Figure 2A) 2A) and anomalous Wells classification IB tracheal branching ( Figure Figure 2B). Surgery at 1 day of age corrected the duodenal occlusion. Ultrasound led to a diagnosis of VSD, DAA, coarctation of the left AA, and bilateral SVC. Clinical examination revealed that first heart sound and lung auscultation were normal. Blood pressure was mmHg heart rate was 110 b.p.m respiratory rate was 40 per min, and SpO 2 was 97%. The baby was born by normal delivery at 40 weeks and 4 days gestation. Ultrasound at 37 weeks and 6 days of gestation were suggestive of VSD, right aortic arch (AA), left patent ductus arteriosus (PDA), and bilateral superior vena cava (SVC). Ultrasound also revealed duodenal obstruction and ventricular septal defect (VSD). The patient was referred for treatment after polyhydramnios was detected on foetal ultrasound at 37 weeks and 4 days of gestation. The patient has persistent inspiratory stridor when affected by a common cold however, her progress is good with no achalasia. Ventricular septal defect patching, left AA resection distal to the root of the left subclavian artery, arterial ligament dissection, and LPA replacement were performed. Repeat CT examination revealed that the angle between the main pulmonary artery and LPA had decreased. The right A2, which is part of the right upper pulmonary artery, showed abnormal branching of the pulmonary artery from left pulmonary artery (LPA).Ĭardiac catheterization revealed Qp/Qs of 2.5, mean pulmonary artery pressure of 27 mmHg, and pulmonary vascular resistance of 1.5 WU⋅m 2. Echocardiography led to a diagnosis of VSD, double aortic arch (DAA), coarctation of the left AA, and bilateral SVC.Ĭomputed tomography (CT) revealed DAA with concurrent pulmonary artery sling (PAS) and anomalous branching of the trachea. Foetal echography revealed findings suggestive of ventricular septal defect (VSD), right aortic arch (AA), left patent ductus arteriosus, and bilateral superior vena cava (SVC).
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