Right ventricle-to-pulmonary artery shunt related complications after Norwood procedure

The right ventricle-to-pulmonary artery (RV-PA) shunt in the Norwood procedure (NP) for children with hypoplastic left-heart syndrome (HLHS) provides stable early hemodynamics and improves survival in many centers. However, lower pulmonary-to-systemic flow ratio causes early cyanosis and may require earlier second-stage procedure. The aim of the study was to present shunt-related results after NP with RV-PA shunt and our technique of RV-PA shunt construction.

Between June 2001 and August 2010, 236 children with HLHS and variants underwent NP with RV-PA shunt, and were operated on by the same surgeon. The medical records were retrospectively reviewed.

To date, 180 children at a mean age of 7.0 ± 1.6 months with a mean weight of 6.4 ± 0.9 kg underwent second-stage procedure. The mean systemic oxygen saturation before stage 2 operation was 74.8 ± 6.6% and mean arterial partial oxygen pressure was 32.8 ± 6.7 mm Hg. These two parameters were significantly lower than after NP (p = 0.029, p < 0.001, respectively). Between stage 1 and 2 operation, three children (1.3%) died due to the shunt obstruction. Four children (1.7%) underwent re-operations due to shunt problems (one of them died), and the other four (1.7%), stent implantation in RV-PA shunt. Two infants (1.1%) developed aneurysm of the right ventricle infundibulum, which was resected during stage 2 without complications. One child required early (before fifth month of age) second-stage procedure due to the shunt obstruction. The patients with right-sided to the neo-aorta course of the RV-PA shunt had significantly more frequent delayed sternal closure after NP than children with left-sided shunt (35.5% vs 14.1%; p = 0.008).

The RV-PA shunt can be a safe and efficient technique in providing optimal pulmonary blood flow in the children with HLHS after Norwood procedure, performed with minimal rate of complications. In our experience, the use of RV-PA shunt in NP does not require earlier second-stage procedure.