Management of the failing Fontan circulation
- 1Division of Cardiology, Children's Memorial Hospital, Feinberg School of Medicine, Northwestern University, Chicago, Illinois, USA
- 2Department of Pediatric and Congenital Heart Surgery, Center for Pediatric and Congenital Heart Disease, Cleveland Clinic Foundation, Cleveland, Ohio, USA
- Correspondence to Dr Barbara J Deal, Division of Cardiology, Children's Memorial Hospital, 2300 Children's Plaza, mc 21, Chicago, IL 60614, USA;
Contributors Both authors contributed to the concept and writing of this article in terms of writing the text, literature review, and approval of the content, analysing the possible risk factors that may contribute to a patient's failing Fontan circulation. Their objective was to educate cardiologists and others who care for patients with single ventricular physiology of the warning signs that can lead to a failed Fontan circulation. Dr Deal is responsible for the overall content as guarantor of this article.
The ‘Fontan circulation' has evolved to include a variety of surgical procedures designed to overcome the absence of two distinct ventricular chambers.1 w1–w3 Inherent to this circulation is chronic elevation of right atrial and vena caval pressure, and absence of a dedicated power source to serve the pulmonary circulation, making low pulmonary vascular resistance and optimal systemic ventricular function the essential ingredients of a successful Fontan circulation.2 Originally designed for the single left ventricle, modifications to the original atriopulmonary connections extended repairs to complex ventricular anatomy, and are now most commonly performed for single right ventricular anatomy associated with hypoplastic left heart syndrome. Together with improved perioperative management, creation of the Fontan circulation in two stages (superior cavopulmonary anastomosis followed by later Fontan completionw4), and performance of Fontan procedures at a younger age, have led to reduced operative mortality associated with the Fontan procedure of ≤5% (compared with 15–30% in earlier decades); survival at 20 years is presently 85%.3 w5
Over the last two decades, the initial survivors of the atriopulmonary Fontan repairs have reached adulthood, bringing a multiplicity of haemodynamic complications and sequelae of their abnormal circulatory status. The atriopulmonary connection is now obsolete as a surgical option, and the current surviving adults with this circulation do not reflect contemporary Fontan outcomes. Nonetheless, their attendant compendium …