Duplicated left pulmonary artery: an unknown disease? Three case reports and review of the literature

Abstract

We report three cases of an abnormal finding of duplicated left pulmonary artery: two of these occurring in children with Kabuki syndrome and configuring the setting of a pseudo-pulmonary sling without any clinical or cardiac cross-sectional evidence of tracheal compression. The other case instead represents duplicated left pulmonary artery with pulmonary sling caused by the retro-tracheal course of the lower left pulmonary artery associated with "Christmas Tree" arrangement of the tracheo-bronchial system. In both patients with pseudo-pulmonary sling and Kabuki syndrome, the abnormal finding was incidental during echocardiographic examination and neither of the patients required surgical repair for the condition. To the best of our knowledge, they represent the third and fourth cases in which such an anomaly of the pulmonary artery branches not forming a sling is seen in association with Kabuki syndrome. Another case represents our second experience and the second case reported in literature with duplicated left pulmonary artery in the setting of a complex tracheal anatomy. In this symptomatic patient, surgical repair of atrial septal defect and relief of the vascular ring were indicated, and the surgical repair was performed successfully at the age of 3 years.

Partial Anomalous Left Pulmonary Artery Sling in an Adult

“…In all of the few prior reports, this abnormality has only been described in infants or young children with other congenital anomalies, especially congenital heart disease. [4][5][6][7] To the best of our knowledge, the case we report is the first instance of this anomaly presenting as an incidental finding in an adult patient.…”

“…is variant is associated with compression of the distal trachea. [5,7] e embryologic origin of the pulmonary artery sling is uncertain, but is generally believed to occur from malformation of the left sixth aortic arch. e bilateral sixth aortic arches supply blood to the developing lung buds and ultimately differentiate into the right and left pulmonary arteries.…”

Clinical and imaging features of pulmonary artery sling: The experience in one major medical center

“…The embryology is still unknown. It was proposed that PAS occurred when the left lung bud fails to connect with the left sixth arch, and a secondary connection was acquired to the right sixth branchial arch through the embryonic peritracheal primitive mesenchymal vessels …”

“…It was proposed that PAS occurred when the left lung bud fails to connect with the left sixth arch, and a secondary connection was acquired to the right sixth branchial arch through the embryonic peritracheal primitive mesenchymal vessels. 5 Pulmonary artery sling is usually associated with congenital cardiac defects, such as ventricular septal defect, atrial septal defect, patent ductus arteriosus, double-outlet right ventricle, aortopulmonary window with interrupted aortic arch, and so on, suggesting an association with a more complex process of dysmorphogenesis and a genetic background. [6][7][8] This is reinforced by the presence of associated congenital anomalies in our patients.…”

An unusual presentation of Kabuki syndrome with orbital cysts, microphthalmia, and cholestasis with bile duct paucity

“…Other than aortic coarctation, which is known to be a frequent arteriovascular malformation in KS, venous or arterial vascular malformations of the cardiopulmonary system, such as anomalous pulmonary venous drainage or double aortic arch, are rare, reported only in six patients [Dyamenahalli et al, ; Shahdadpuri et al, ; Moral et al, ; Bhat et al, ; Oswal et al, ; Giudici et al, ]. Vascular malformations of the central nervous system or the eyes are even rarer.…”