Congenital disconnection of the pulmonary arteries

Introduction

Disconnected pulmonary artery is a rare anomaly that can be isolated or associated with complex intracardiac malformations. Early re-implantation of the disconnected pulmonary artery is recommended to allow growth and satisfactory pulmonary perfusion while preventing collateral arteries development.

Objective

To describe the characteristics of patients with disconnected pulmonary artery and, for those who had surgical re-implantation, to determine the incidence, delay and predictive factors of re-intervention for reconnected pulmonary artery stenosis.

Methods

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

Results/Expected results

Retrospective observational study of 55 patients with a disconnected pulmonary artery. Fifty-one underwent surgical correction and were followed up at our institution between 2000 and 2022.

Disconnected pulmonary arteries were observed in isolation in 31% of the cases. The most frequent form was left pulmonary artery originating from the arterial duct (58%). The re-implantation was done at median age of 12 days. Anastomotic stenosis was observed during follow-up in 71% of the patients with 75% of them requiring re-intervention (55% of the population). The median delay to re-intervention was 3.2 years after re-implantation, and more than 25% of re-interventions on the re-implanted pulmonary artery occurred within the first postoperative year. We found more re-intervention if associated cardiac defect, without significant statistic difference. Weight at re-confluence, presence of ductal tissue at the origin of the pulmonary artery, and prior shunt placement on the disconnected pulmonary artery were not found to be risk factors for re-intervention (Figure 1).

Conclusion/Perspectives

After surgical re-implantation of pulmonary artery, more than 50% of patients required re-intervention for pulmonary artery stenosis. Technical improvements should be sought to reduce incidence of this complication.