Background: Pulmonary atresia with pulmonary coarctation may complicate diminished and unbalanced pulmonary development. The aim of this study is to assess the outcome of pulmonary arterial reconstruction with cardiopulmonary bypass in early infancy for sufficient and balanced pulmonary development.
Methods: We performed a retrospective review of 15 patients with pulmonary coarctation younger than 4 months of age who underwent pulmonary arterial reconstruction between 2001 and 2005. The mean age and weight were 42.2 days and 3.62 kg, respectively. The patient population included 5 biventricular repair candidates and 10 Fontan candidates. To evaluate the pulmonary arterial development, the preoperative and postoperative pulmonary arterial index and minimum diameter of the pulmonary artery were compared.
Results: No early or in-hospital deaths occurred, and there was no nonconfluent pulmonary artery development or segmental mal-development after a mean follow-up period of 14.9 months. Immediate pulmonary flow regulation was required in 2 patients because of excessive pulmonary flow. The mean pulmonary arterial index increased significantly from 103 mm2/m2 to 343 mm2/m2, and the mean minimum diameter of the pulmonary artery increased significantly from 2.02 mm to 4.45 mm. Four biventricular repair candidates completed definitive repair, and 2 required surgical reintervention in the pulmonary artery. Six Fontan candidates completed the Glenn procedure, and 1 completed the Fontan procedure. Three required surgical reintervention in the pulmonary artery. Two late deaths occurred after the Glenn procedure because of ventricular dysfunction and respiratory infection.
Conclusions: Pulmonary arterial reconstruction in early infancy provides sufficient and balanced pulmonary arterial development for pulmonary atresia with pulmonary coarctation.