Peripheral Pulmonary Artery Stenosis Is Not a Contraindication to a Single-ventricle Pathway

The presence of peripheral pulmonary artery stenosis (PPAS) involving lobar and/or segmental pulmonary artery (PA) branches might preclude proceeding to a single-ventricle pathway. We adopted a strict strategy for PA rehabilitation and surgical reconstruction in patients who are unable to progress to a single-ventricle pathway.

We conducted a retrospective review of 22 patients with single-ventricle physiology who underwent surgical reconstruction for PPAS from April 2008 to March 2020. Our surgical approach was single stage in 15 patients (68.2%) and 2 stage in 7 patients (31.8%) depending on the presence or absence of PA hypoplasia distal to the PPAS.

The PPAS was type 3 (lobar) in 19 patients (86.4%) and type 4 (segmental) in 3 patients (13.6%). The mean number of PA angioplasties performed was 8 ± 5.6 per patient. There was no mortality in this series with a median of 52 months (range, 8-143) of follow-up. Twenty patients (90.9%) were able to progress in the single-ventricle pathway with 12 patients (54.5%) undergoing a Fontan operation and 8 patients (36.4%) achieving bidirectional Glenn shunt and awaiting a Fontan operation. Two patients (9.1%) were not suitable to progress to the single-ventricle pathway because of elevated PA pressure. All patients who achieved cavopulmonary connection were alive and asymptomatic with no signs of elevation of PA pressure. No patient required further reinterventions for the PA.

PPAS is not a contraindication for single-ventricle palliation. With careful planning and aggressive early surgical reconstruction, most of these patients can successfully progress to cavopulmonary connections.