ABSTRACT
Enhanced techniques for single-ventricle palliation (SVP), in which the Fontan procedure is the final phase, have improved the survival and quality of life in patients not eligible for biventricular repair (BiVR). However, Fontan physiology also is associated with predictable long-term consequences that ultimately affect quality of life and freedom from adverse events. Given this harsh reality, the advances in ventricular rehabilitation strategies and the conversion from SVP increasingly are being used to achieve biventricular circulation in patients with left-heart hypoplasia who previously were considered to be marginal or unacceptable candidates for BiVR. The techniques for ventricular recruitment include restriction of the atrial septal defect (ASD), atrioventricular valve repair, repair of left ventricular outflow obstruction, endocardial fibroelastosis resection, an augmented pulmonary blood flow, and catheter-based interventions. In addition, reverse double-switch surgery has been used as an option for patients with a left ventricle of insufficient size and function to support the systemic circulation, who would otherwise require a Fontan surgery. Despite improved management and surgical technique, the outcomes after biventricular staging repair and conversion remain variable, and the complications ultimately may exclude patients from future single-ventricle palliation or transplant. The anesthesiologist should have a basic understanding of the anatomy and physiology of this growing patient population in order to develop adequate perioperative management options.
