Prenatal diagnosis of left pulmonary artery sling and review of literature

Abstract

Left pulmonary artery sling (LPAS) is a very rare cause of large airway compression. In LPAS, the left pulmonary artery (LPA) arises from the proximal right pulmonary artery, coursing over the right mainstem bronchus, posterior to the trachea and anterior to the esophagus prior to reaching the left hilum. The aberrant course of the LPA results in anatomical obstruction of the right mainstem bronchus, the trachea, or both. Thirty percent of affected patients have major associated structural heart disease 1 which can importantly impact clinical outcome. 2 The clinical manifestation of LPAS after birth usually relates to the severity of airway obstruction and the associated cardiac pathology. Only a few reports present the prenatal features of LPAS. In the present report, we present the prenatal diagnosis of a case of LPAS in one of a set of identical twins in which the only feature was that of an abnormal course of the LPA on 3-vessel tracheal view. | C A S E PRE S ENTATI ONA 30-year-old primigravida woman at 27 weeks' gestation was referred to our maternal fetal medicine unit given the presence of a set of monochorionic, diamniotic spontaneous twins. A full anatomical ultrasound examination performed in both fetuses demonstrated no pathology, and fetal echocardiography demonstrated no major structural heart disease in one twin. In the other twin, fetal echocardiography revealed on a 3-vessel tracheal view that the LPA originated from the right pulmonary artery, originating initially to the right side of the trachea, and turning sharply to course between the trachea and esophagus prior to joining the left hilum (Figure 1, Movie S1). The examination was performed transabdominally using a Voluson E 6 (GE healthcare Ultrasound, Milwaukee, WI, USA) ultrasound machine equipped with a RAB 6D (2-7 MHz) probe. With these findings, pediatric cardiology counseling was offered and LPAS was suspected. The remainder of the cardiac anatomy was Left pulmonary artery sling (LPAS) is a very rare cause of large airway compression.In LPAS, the left pulmonary artery (LPA) arises from the proximal right pulmonary artery, coursing over the right mainstem bronchus, posterior to the trachea and anterior to the esophagus prior to reaching the left hilum. The aberrant course of the LPA results in anatomical obstruction of the right mainstem bronchus, the trachea, or both. Only a few reports present the prenatal features of LPAS. In this report, we present the prenatal diagnosis of a case of LPAS in one of a set of identical twins in which the only feature was that of an abnormal course of the LPA on 3-vessel tracheal view. The cross-sectional view at the level of three vessels which includes both pulmonary artery branches is useful to detect this abnormality. Color and power Doppler may be helpful as well. K E Y W O R D Sfetal echocardiography, left pulmonary artery, vascular imaging

Novel TrueVue series of 3D echocardiography: Revealing the pathological morphology of congenital heart disease

“…Rare pulmonary artery sling imaging has certain difficulties in 2D and traditional 3DE, usually need time-consuming and laborious to confirm the diagnosis through the 3D reconstruction of enhanced computed tomography (CT), and the children under examination are not easy to cooperate well ( Sezer et al, 2019 ; Xu et al, 2020 ). TrueVue Glass has an obvious advantage in the diagnosis of pulmonary artery sling, and it is the first time that an innovative 3D ultrasound technology has been used to diagnose such rare cardiovascular malformations.…”

Two fetuses in one family of arterial tortuosity syndrome: prenatal ultrasound diagnosis

“…And also differentiated from pulmonary artery sling antenatally, the cross-sectional view at the level of three vessels which includes both PA branches is useful to detect and color and power doppler may be helpful as well. The difference is that the ATS is only PA tortuous and does not bypass the trachea, but the PA sling is the LPA originated from the RPA, originating initially to the right side of the trachea, and turning sharply to course between the trachea and esophagus prior to joining the left hilum [ 19 ]. In conclusion, the cases that both parents were carriers, each sib has a 25% chance of being affected, a 50% chance of being an asymptomatic carrier, and a 25% chance of being unaffected and not a carrier.…”

The Role of Lung Function Testing in Newborn Infants With Congenital Thoracic Arterial Anomalies

“…CTAA may encircle the trachea and/or the esophagus resulting in an obstruction and determining respiratory symptoms such as respiratory distress, recurrent respiratory tract infections, stridor, and/or dysphagia ( 1 ). Depending on the severity of obstruction, this may determine a mild to severe respiratory distress during infancy and may require surgical treatment ( 1 , 2 ). Anomalies of the aortic arch and branches are known as vascular rings (VRs).…”