Incidental finding of pulmonary arterial sling during patent ductus arteriosus surgery in a patient with Mowat-Wilson syndrome

Mowat-Wilson syndrome is a genetic condition due to a mutation in the ZEB2 gene; it affects many systems including the cardiovascular system. The pulmonary arterial sling originates from a failure of development of the proximal portion of the left sixth aortic arch, resulting in an anomalous left pulmonary artery origin from the posterior wall of the right pulmonary artery and the left pulmonary artery crossing to the left lung between the trachea and the oesophagus. We present a 4-month-old infant with Mowat-Wilson syndrome and left pulmonary arterial sling, and discuss the association of these two rare conditions. Pulmonary arterial sling is significantly more frequent in patients with Mowat-Wilson syndrome than in the general population. © Cambridge University Press 2018.

• Fecha de publicación: 2018
• Institución: Universidad del Rosario
• Repositorio: Repositorio EdocUR - U. Rosario
• Idioma: eng
• OAI Identifier: oai:repository.urosario.edu.co:10336/23640
• Acceso en línea: https://doi.org/10.1017/S1047951118000689; https://repository.urosario.edu.co/handle/10336/23640
• Palabra clave: Anamnesis; Artery ligation; Article; Bronchoscopy; Case report; Clinical article; Echocardiography; Failure to thrive; Gastroesophageal reflux; Gene; Genetic disorder; Heart dilatation; Human; Image reconstruction; Incidental finding; Infant; Intensive care unit; Male; Mowat wilson syndrome; Patent ductus arteriosus; Physical examination; Pulmonary arterial sling; Pulmonary artery; Pulmonary artery malformation; Sequence analysis; Systolic heart murmur; Time to treatment; X-ray computed tomography; Zeb2 gene; Abnormalities; Complication; Diagnostic imaging; Facies; Genetics; Hirschsprung disease; Incidental finding; Intellectual impairment; Microcephaly; Patent ductus arteriosus; Three dimensional imaging; Vascular surgery; Facies; Hirschsprung disease; Humans; Incidental findings; Infant; Intellectual disability; Male; Microcephaly; Pulmonary artery; Vascular surgical procedures; 6th aortic arch; General cardiology; Mowat-wilson syndrome; Pulmonary arterial sling; Zeb2; x-ray computed; patent; three-dimensional; Ductus arteriosus; Imaging; Tomography
• License: Abierto (Texto Completo)