Echocardiographic diagnosis of a pulmonary artery sling: what kept it hidden for seven years?

Xi-jun Zhu, Wen-juan Qiu

Abstract

Pulmonary artery sling (PAS) is a rare cardiovascular anomaly, occurring in approximately 1 in 17,000 individuals, often leading to severe tracheal compression and respiratory distress. This case report details the diagnosis, treatment, and follow-up of a seven-year-old female patient with PAS, a condition that remained undiagnosed for years despite multiple hospitalisations. Initially admitted at 4 weeks old for neonatal pneumonia, hypoxic encephalopathy, and patent ductus arteriosus (PDA), the patient underwent various treatments and misdiagnoses, including bronchopneumonia and congenital heart defects. It wasn’t until she was seven years old, after presenting with fever, cough, and respiratory distress, that a third echocardiographic examination suggested PAS. This was later confirmed through Pulmonary Artery Computed Tomography Angiography (CTA) and Multi-Slice Spiral Computed Tomography (MSCT) three-dimensional (3D) reconstruction of the airway, revealing distal bronchus stenosis. The patient underwent successful end-to-side anastomosis of the left pulmonary artery to the main pulmonary artery, significantly improving her respiratory function and reducing hospitalisations for pneumonia. This case underscores the importance of considering PAS in differential diagnosis for children with unexplained respiratory symptoms and the role of repeated echocardiography in avoiding delayed diagnosis and treatment. Early detection and prompt surgical intervention are crucial in reducing mortality and morbidity associated with PAS.