The past decade has seen a substantial improvement in the outcome following surgical palliation for hypoplastic left heart syndrome. This has been attributed to modifications in the surgical as well as postoperative management strategies. One such modification is the reemergence of the right ventricle to pulmonary artery (RV-PA) shunt as an alternative to the modified Blalock-Taussig (mBT) shunt as the source of pulmonary blood flow. The RV-PA shunt has been shown to improve the immediate surgical outcome compared with the classic Norwood procedure with an mBT shunt. Despite the several reported advantages, the impact of the RV-PA shunt on growth of the pulmonary arteries and incidence of late development of central PA stenosis remains unclear. This systematic review evaluates the current best available evidence to address this issue and concludes that the evidence from retrospective studies and only available randomized controlled trial (RCT) is conflicting. The retrospective studies predominantly suggest that the Norwood procedure with RV-PA shunt may have favorable effects on the development of the pulmonary arteries due to even distribution of pulmonary blood flow with greater distal left pulmonary artery growth, resulting in more balanced distal branch pulmonary artery size albeit with a greater degree of central pulmonary artery hypoplasia needing surgical attention. On the contrary, the RCT reports that the overall size of the pulmonary artery on angiography before the stage II procedure was smaller in the RV-PA shunt group than in the mBT shunt group, with no information available on incidence of central pulmonary hypoplasia.
Keywords: RV-PA shunt); congenital heart disease; congenital heart surgery; hypoplastic left heart syndrome; shunts (modified Blalock-Taussig shunt.