Management of neonates with ventricular septal defects and aortic arch anomalies

Objective: Complete neonatal repair of aortic arch anomalies (AAA) combined with ventricular septal defects (VSD) avoiding palliative procedures remains controversial. The aim of the study was to analyze the strict policy of primary early correction of AAA+VSD introduced in 2006.

Methods: Between 2006 and 4/2014 a total of 31 consecutive patients with AAA and VSD underwent primary repair in neonatal age. AAA included simple coarctation (CoA), hypoplasia of the aortic arch or interrupted aortic arch with or without aortic atresia. All VSD types except of multiple muscular VSD's were included in the observation. The median age at surgery was 10 days (2-26 d) and median weight was 3 kg (2-4 kg).

Results: There was no early or late death during a mean follow up (FU) of 30 months. All but 3 patients with restrictive muscular VSD's were completely corrected. No palliative procedures were needed. The median in hospital stay was 23 days (2-96 d). There were no clinically apparent neurological problems and all patients are doing well showing adequate growth. There were 3 (9,6%) postoperative transcatheteral interventions on the aortic arch at 2, 6 and 21 months, respectively.

Conclusions: Primary complete repair of aortic arch anomalies combined with VSD's in neonatal period, provides excellent mid-term outcome with no mortality and low morbidity. Staged approach with the risk of pulmonary banding related pulmonary artery distortion, and subsequent pulmonary artery underdevelopment should be reserved for carefully selected high risk patients with multiple VSD's or associated complex malformations.