Congenital disconnection of the pulmonary arteries

Disconnected pulmonary artery (PA) is a rare anomaly that can be isolated or associated with complex intracardiac malformations. Early reimplantation of the disconnected PA is recommended to allow growth and satisfactory pulmonary perfusion while preventing collateral artery development. The aim of this study was to describe the characteristics of patients with disconnected PA and, for those who had surgical reimplantation, to determine the incidence, delay and predictive factors of reintervention for reconnected PA stenosis.

We include patients with the diagnosis of congenitally disconnected PA and surgical repair at our institution.

Retrospective observational study of 55 patients with a disconnected PA. Fifty-one underwent surgical correction and were followed up at our institution between 2000 and 2022. Disconnected PAs were observed in isolation in 31% of the cases. The most frequent form was left PA originating from the arterial duct (58%). The reimplantation was done at the median age of 12 days. Anastomotic stenosis was observed during follow-up in 71% of the patients with 75% of them requiring reintervention (55% of the population). The median delay to reintervention was 3.2 years after reimplantation, and >25% of reinterventions on the reimplanted PA occurred within the first postoperative year. We found more reintervention if associated cardiac defect, without significant statistic difference. Weight at re-confluence, presence of ductal tissue at the origin of the PA and prior shunt placement on the disconnected PA were not found to be risk factors for reintervention.

After surgical reimplantation of PA, >50% of patients required reintervention for PA stenosis. Technical improvements should be sought to reduce the incidence of this complication.