Case Report
A 62-year-old female with history of Alpha-1 Antitrypsin deficiency and congenital pulmonic valve (PV) stenosis presented with symptoms of palpitations, lightheadedness, chest pressure, and bradycardia. A comprehensive cardiac evaluation was performed including cardiac catheterization which did not demonstrate coronary disease, however, was concerning for PV stenosis. Follow-up MRI demonstrated mild PV stenosis, but incidentally noted a significant post-stenotic pulmonary artery (PA) aneurysm measuring 6 cm with extension into the left PA. [Figure 1] She continued to have symptoms of palpitations and chest pressure. Given the size of the aneurysm, she was offered surgical repair due to the increased risk of PA rupture, similar to guidelines for aortic aneurysms.
She was taken to the operating room and underwent a median sternotomy, was placed on cardiopulmonary bypass, and the heart was arrested. The aortopulmonary groove was dissected free and the main PA was encircled. Dissection was carried down the left PA until it exited the pericardium, where the size was dilated, but had tapered to within a normal range. The PA bifurcation was isolated and dissected free, and the right PA was dissected free under the aorta and to the exit of the pericardium. The right PA was of normal size distally. With full exposure, the PA was transected about 1.0cm distal to the PV and carried up toward the left PA. The bifurcation was preserved as a potential reimplantation button. The left PA was transected distally leaving only a small sewing cuff along the pericardium. A 26mm Gelweave graft was used to reconstruct the main to left PA segment as an interposition graft. The right PA was felt to be dilated at the bifurcation and an 18mm Gelweave graft was used to reconstruct the right PA segment underlying the aorta as an interposition graft to main Gelweave graft. [Figure 2] TEE at the conclusion of the operation demonstrated no significant PV stenosis and good luminal flow through both PA branches of the reconstruction.
The patient tolerated the procedure without any difficulties and was discharged on POD 4 after an unremarkable course. She was doing well at 2- and 4-week follow-up and completed cardiopulmonary rehabilitation. She has continued to have aggressive pulmonary management due to her Alpha-1 Antitrypsin deficiency but has recovered completely from her surgical intervention.