Phenoxybenzamine in the treatment of hypoplastic left heart syndrome: a core review

Abstract

Perioperative management of neonates after the Norwood procedure is extremely complex. Limited reserve of the neonatal single ventricle and the parallel arrangement of the pulmonary and systemic circuits result in a tenuous balance between pulmonary and systemic blood flows. Precise manipulations of both pulmonary and systemic vascular resistance are necessary to prevent excessive pulmonary blood flow at the expense of systemic oxygen delivery. An emerging treatment strategy aimed at improving early mortality is the intraoperative administration of phenoxybenzamine, a profound systemic vasodilator. Maximum systemic vasodilation is thought to reduce afterload of the single ventricle and produce a more stable parallel circulation by ameliorating the postoperative fluctuations in systemic vascular resistance. Although this strategy has gained popularity at many centers, it is not without scrutiny. The following review provides an overview of the pharmacology of phenoxybenzamine, the surgical and physiologic implications of the Norwood procedure, and a discussion of the pros and cons of phenoxybenzamine administration.