[Left pulmonary artery sling associated with right pulmonary hypoplasia and ventricular septal defect]

The pulmonary artery sling is a congenital malformation where the left pulmonary artery forms a vascular sling that bends around the right border of the lower trachea. Its clinical manifestations are signs of tracheal compression in the first months of life. The diagnosis of this rare anomaly is suspected on radiological and echocardiographic grounds and confirmed by oesophagography, tracheoscopy and angiocardiography. Diagnostic problems may be encountered when the condition is associated with tracheobronchial (50% of the cases) or cardiovascular malformations. In the case reported here respiratory symptoms and heart failure were present in a 1-month hypotrophic infant who also had ventricular septal defect and dextrocardia due to right lung hypoplasia. The pulmonary artery sling was diagnosed by angiography. The severity of the clinical signs precluded all attempts at surgical repair of the cardiovascular anomalies. The child died at the age of 2 1/2 months.