BACKGROUND Patients with a single ventricle have multiple risk factors for central nervous system injury, both before and after the Fontan procedure. METHODS AND RESULTS A geographically selected cohort was invited to undergo standardized testing, including age-appropriate measures of intelligence quotient (IQ) and achievement tests. Historical information was obtained by chart review and pat...

OBJECTIVES Results of Fontan's procedure have improved considerably, but perioperative mortality still occurs, attributed to ventricular dysfunction, stroke, arrhythmia, thromboembolism, and multi-organ dysfunction. Our protocols of operative and intensive care unit management address these potential issues, and have been associated with zero mortality, even with many high-risk candidates. ME...

Between August 1999 and December 2007, 72 consecutive patients with single ventricle physiology underwent a modified Fontan procedure after a bidirectional Glenn shunt using an extracardiac polytetrafluoroethylene conduit without fenestration. Nitric oxide gas inhalation was commenced just after cardiopulmonary bypass together with intravenous phosphodiesterase III inhibitor administration. Aft...

OBJECTIVE To examine cardiopulmonary performance in 52 adult patients with a Fontan circulation. DESIGN Retrospective cohort study. Values of maximum oxygen uptake (VO(2)max), maximum heart rate (HRmax), forced vital capacity (FVC), and forced expiratory volume in one second (FEV(1)) were compared with predictive values for different age groups. Patients were further subdivided into those wit...

BACKGROUND Late complications after Fontan procedure may be due to the absence of pump and pulsatile pulmonary blood flow in this type of palliation. Our aim was to quantify the degree of pulsation by echocardiographic method in patients with extracardiac total cavopulmonary connection (ECTCPC) in comparison with biventricular circulation and few cases of pulsatile Fontan. METHODS In a case s...

OBJECTIVE Early circuit separation enhances the long-term success of Fontan haemodynamics. To test this hypothesis, we analysed the postoperative cardiopulmonary capacity in children and adults. PATIENTS Spiroergometry was performed at least twice in 43 patients with a median age of 14 (range: 7-43) years, with a median time interval of 4.6 (1.1-10.4) years between early and late testing. Twe...