Abnormal origin of the pulmonary artery

Abnormal origin of the pulmonary artery (also known as anomalous origin of the pulmonary artery) refers to a group of rare congenital cardiovascular malformations in which one or both pulmonary arteries arise from an abnormal location. The most well-recognized form is anomalous origin of the left pulmonary artery from the right pulmonary artery, commonly called pulmonary artery sling, where the left pulmonary artery courses between the trachea and esophagus to reach the left lung. Another variant involves anomalous origin of a pulmonary artery from the aorta (hemitruncus). These malformations primarily affect the cardiovascular and respiratory systems. Clinical presentation typically occurs in infancy or early childhood and may include respiratory distress, stridor, wheezing, recurrent respiratory infections, and feeding difficulties. In pulmonary artery sling, compression of the trachea and main bronchi can lead to significant airway obstruction. Associated anomalies such as complete tracheal rings, other congenital heart defects (ventricular septal defect, patent ductus arteriosus, tetralogy of Fallot), and tracheobronchial anomalies are frequently observed. If a pulmonary artery arises from the aorta, pulmonary hypertension may develop rapidly due to exposure of the pulmonary vascular bed to systemic pressures. Diagnosis is established through echocardiography, CT angiography, or MRI, and cardiac catheterization may be performed for hemodynamic assessment. Treatment is surgical and typically involves reimplantation of the anomalous pulmonary artery to its correct anatomical position. In cases of pulmonary artery sling with associated tracheal stenosis from complete tracheal rings, simultaneous tracheoplasty may be required. Early surgical intervention is important to prevent irreversible pulmonary vascular disease and to relieve airway compression. Outcomes have improved significantly with advances in pediatric cardiac surgery, though long-term follow-up is necessary to monitor for potential complications such as pulmonary artery stenosis at the reimplantation site.

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