The perinatal transition and early neonatal period in hypoplastic left heart syndrome is associated with reduced systemic and cerebral perfusion.

Abstract Background The impact of the striking perinatal circulatory changes on blood flow distribution have not to date been well-examined in hypoplastic left heart syndrome (HLHS). This study aims to document perinatal redistribution of cardiac output in HLHS compared to healthy controls, to further understand the impact of perinatal transition on cerebral and systemic blood flow. Methods Prospectively recruited HLHS cases (n=31) and healthy controls (n=19) underwent serial echocardiography from late fetal stages to 96 hours after birth. Combined cardiac output (CCO), systemic, pulmonary, cerebrovascular and splanchnic flow data were compared between HLHS neonates and controls, and the impact of vasoactive support and positive pressure ventilation (PPV) in HLHS patients examined. Results In late gestation, CCO was similar between HLHS and controls, whereas middle cerebral artery (MCA) pulsatility index (PI) in HLHS was consistent with low cerebral vascular resistance. In the 96 hours after birth, CCO and pulmonary blood flow progressively increased in HLHS compared to controls (p Conclusion We provide quantitative echocardiographic evidence associating impaired cerebral and systemic blood flow with perinatal hemodynamic changes in the preoperative HLHS neonate.