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.