Fontan operation after 3 decades: what we have learned.

T he Fontan operation was designed to provide blood flow in series to the pulmonary and systemic circulation without the requirement for a right ventricular pumping chamber. The operation allows systemic venous blood to flow directly into the pulmonary circulation on the basis of a single ventricular impetus through the arteries, capillaries, and systemic venous system. This arrangement has improved life expectancy for patients with single-ventricle and pulmonary-outflow obstruction compared with previous arterial shunts. The long-term effects of marked single-ventricle preload and inefficient oxygenation via an arterial shunt rarely allowed survival beyond the second or third decade of life. Remarkably , the Fontan operation has become the most common procedure performed for congenital heart disease after the age of 2 years. Over the past 3 decades, the early and intermediate prognoses for patients who have undergone this operation have been improving as a result of useful refinements in the surgical procedure that have been introduced since Fontan's original direct right atrium–to–pulmonary artery (RA-PA) connection. Furthermore, the indications for the operation have broadened considerably compared with the relatively few patients thought to be eligible in the late 1970s and 1980s. The ultimate test of any surgical procedure is the long-term status of the postoperative patients. The article by Khairy and associates 1 in this issue of Circulation provides insight into the survival of the earliest generation of Fontan patients and allows assessment of the impact of management and surgical variations that have evolved over the past 3 decades on mortality. However, the young adults reported in this study represent the survivors of the early operations and may not reflect the eventual status of today's infants and children who undergo the modern procedure. Beyond mortality, it is important to evaluate potential risk factors as they relate to the functional status and quality of life of patients with a Fontan circulation. A recent survey of young children and adolescents who have undergone the Fontan operation attempts to address this issue. 2,3 If the factors that lead to a failed Fontan in terms of mortality, current medical status, and quality of life can be understood, it is hoped that the next decade will allow further improvements in the management of patients with a single ventricle. Mortality Perioperative and early mortality after the Fontan operation have decreased markedly over the past 3 decades. Among early survivors, late mortality and modes of death can be assessed …