Primary pulmonary artery reconstruction for functional single ventricle with absent central pulmonary artery and bilateral patent ductus arteriosus.

Objective: This study aimed to evaluate and discuss the outcomes of creating a single systemic-pulmonary shunt and reconstruction of the pulmonary artery continuity in patients with a single functional ventricle, absent central pulmonary artery, and bilateral patent ductus arteriosus.

Methods: Six infants diagnosed with a functional single ventricle, absent central pulmonary artery, and bilateral patent ductus arteriosus were treated by creating a single systemic-pulmonary shunt and reconstructing the pulmonary artery continuity (primary operation) between January 2010 and September 2020. Pulmonary artery continuity was ensured using the remnant pulmonary artery and an autologous pericardial patch in five patients and a rolled autologous pericardium in one patient.

Results: All patients eventually underwent total cavopulmonary connection. Two patients underwent intrapulmonary artery septation before Glenn or total cavopulmonary connection procedure. The median follow-up period was 9.02 years (interquartile range, 3.90-9.53). No late deaths were observed.

Conclusions: Our strategy of establishing a single systemic-pulmonary shunt with reconstruction of the pulmonary artery continuity was useful for treating patients with a functional single ventricle with absent central pulmonary artery and bilateral patent ductus arteriosus. This procedure helped accomplish pulmonary artery growth and ensured an appropriate volume load after total cavopulmonary connection.