Pediatric Cardiologist, Assistant professor in Pediatrics Oklahoma Children's Hospital Oklahoma City, Oklahoma, United States
Abstract: Our patient is a 3-year-old with history of Alagille syndrome and Tetralogy of Fallot with pulmonary atresia with discontinuous branch pulmonary arteries which were severely hypoplastic supplied by bilateral ducti. He underwent bilateral shunts with the right subclavian artery to the right pulmonary artery (RPA) “right sided classic BT shunt” and a central shunt to the left pulmonary artery (LPA) at 1 week of life for PA growth. Hilum to hilum patch angioplasty with unifocalization and a RV to PA conduit using a 6mm shunt with Brawn modification was done at 8months of age. He had chronic respiratory failure due to severe tracheobronchomalacia requiring tracheostomy 3 months after surgery.
A CT done after his second surgery showed significant tracheal and bronchial compression (figure 1) caused by the aneurysmal ascending aorta, which measured around 26 mm Z score of +8, and the resulting narrow AP window between the ascending and descending aorta. He underwent replacement of the aneurysmal ascending aorta with a 16mm Hemashield graft in an effort to improve the aortopulmonary window. In addition, he underwent replacement of the RV to PA conduit with reposition of the conduit from the right to the left of the aortic arch.
A repeat CT scan done 7 months after surgery showed increased anterior posterior dimension between the ascending and descending aorta, (21.5mm compared to 12.6mm on the CT scan done before surgery, Figure 2), improved caliber of the distal main trachea with only mild narrowing of the distal trachea in the anterior posterior plane and no residual compression of the right and left bronchus since last surgery (Figure 3)
Patient tolerated trach decannulation 11months after the last heart surgery. He is currently 4-years-old and is doing well.
