Interventional Treatment of a Total Coarctation of the Left Pulmonary Artery in a Newborn with Complete Atrioventricular Septal Defect Combined with a Tetralogy of Fallot

All articles of this category (opens in new window) Background: Coarctatio pulmonalis is observed in approximately 2 to 3% of patients with pulmonary valve atresia and ventricular septal defect or Tetralogy of Fallot. In these congenital heart defects, both the origin and entry point of the arterial duct may be anatomically displaced. Notably, lateral displacement of the ductal entry into the pulmonary arteries can result in significant stenosis or even atresia of the left or right pulmonary artery. Methods: We report the case of a newborn diagnosed with Trisomy 21, complete atrioventricular septal defect, and Tetralogy of Fallot. Initial echocardiographic evaluation revealed a tortuous patent arterial duct. The patient maintained oxygen saturation levels between 88 and 92%. By the 6th day of life, the saturation dropped to 70%. Subsequent echocardiography demonstrated the closure of the arterial duct and absence of blood flow in the left pulmonary artery (LPA). Administration of prostaglandin E1 successfully reopened the duct, resulting in detectable perfusion of the LPA. Computed tomography revealed a stenotic origin of the LPA from the main pulmonary artery, with lateral displacement of the arterial duct entry into the LPA. Angiography confirmed the absence of antegrade flow from the main pulmonary artery into the LPA, indicating complete occlusion. The LPA was perfused solely via the reopened ductus arteriosus. After careful probing of the occluded LPA origin, a guidewire was successfully placed, allowing restoration of vessel continuity by implanting a 4×8 mm coronary stent, which was subsequently dilated to a diameter of 4.6 mm. Subsequently, the prostaglandin E1 infusion has been discontinued. This stent is capable of being over-dilated up to 6 mm and shortens accordingly to approximately 6 mm in length, facilitating future surgical correction by avoiding a long segmental gap. Conclusion: This case highlights the critical importance of early recognition of anomalous pulmonary artery anatomy, especially in the context of an abnormal entry of the ductus arteriosus. Percutaneous recanalization provides a valuable neonatal treatment option, potentially avoiding complex and high-risk surgical reconstruction of small-caliber pulmonary vessels in early infancy. Publication History Article published online: 17 February 2026 © 2026. Thieme. All rights reserved. Georg Thieme Verlag KG Oswald-Hesse-Straße 50, 70469 Stuttgart, Germany