Anaesthesia for the child with a univentricular heart: a practical approach

Abstract

Congenital heart disease (CHD) represents a major challenge for anaesthetists. This is due to the complexities in pathophysiology and the impact of surgery and anaesthesia, which predisposes to significant perioperative morbidity and mortality.¹ Univentricular heart (UH) consists of a large variety of rare and complex congenital cardiac malformations. There are rare cases of children having only a single functional ventricle from birth. More commonly, children may receive surgical palliation for complex cardiac defects by creating a univentricular heart when biventricular repair is not feasible. About 30% of patients with univentricular physiology have complications when undergoing non-cardiac surgery.²

During this review, we aim to provide a practical understanding of the pathophysiology of UH and outline a systematic approach to the perioperative management of these patients presenting for non-cardiac surgery. We conclude with an evaluation of the use of near-infrared spectroscopy, transoesophageal echocardiography and ventricular assist devices, as technologies with increasing application in the management of this challenging patient population.