Perioperative Considerations for Children with Right Ventricular Dysfunction and Failing Fontan

The survival of patients with congenital heart diseases (CHD) has increased in the past decades, resulting in the identification of new characteristics of chronic comorbidities observed in pediatric and adults with CHD. Patients with CHD can present with a broad clinical spectrum of manifestations of congestive heart failure (CHF) at any point throughout their lives that may be related to anatomical or surgical variables. This article focuses on the perioperative assessment of patients with CHD and CHF, with an emphasis on pathophysiologic, diagnostic, and therapeutic alternatives in patients with right ventricular failure and failing Fontan circulation. We also provide descriptions of the effects of sedatives and anesthetics commonly used in this population in diagnostic or invasive procedures.