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. 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. 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. Supplementary video available online: http://www.sajaa.co.za/index.php/sajaa/article/view/2572