BACKGROUND Previous studies of patients after the Fontan operation have reported IQ scores lower than population norms. In the past decade, changes have occurred both in surgical methods used and in the patient population undergoing Fontan palliation. The present study examined the impact of these changes on neurodevelopmental outcomes after Fontan. METHODS AND RESULTS Neuropsychological test...

BACKGROUND The growing population with Fontan operation surviving into adulthood has significant morbidity and mortality rates from recurrent atrial tachyarrhythmias. We hypothesized that the structural characteristics and electrical behavior of atria may differ in these patients compared with those without arrhythmias. METHODS AND RESULTS We studied 33 consecutive patients (age, 25.4+/-9.5 y...

BACKGROUND Significant pleural effusions after the Fontan operation prolong hospital stay, may increase the risk of infection, and may necessitate a pleurodesis procedure. METHODS AND RESULTS From February 1991 to April 2000, 98 consecutive patients under the age of 18 years underwent the fenestrated Fontan procedure at Children's Hospital of Wisconsin. Ninety-four patients who survived at le...

PURPOSE To investigate how blood lactate concentration changes in the early postoperative course after the Fontan procedure, and whether such a change is associated with postoperative hemodynamics. MATERIALS AND METHODS Eight pediatric patients who underwent the Fontan procedure for congenital heart disease were included. Enrollment criteria were body weight >10 kg and staged Fontan procedure...

Coronary artery embolism is an uncommon cause of acute myocardial infarction (MI). We present a patient with pulmonary atresia and severe right heart hypoplasia who underwent a lateral tunnel Fontan procedure in childhood and presented with an acute ST-segment elevation MI at 19 years of age. In addition to the known risk of thrombotic complications associated with a Fontan circulation, potenti...

Nearly 50 years after the pioneering procedure performed by Francis Fontan and Eugene Baudet, which has saved the lives of thousands of children, there are still more questions than answers regarding therapeutic management. The complex pathophysiology of Fontan circulation, the lack of clear guidelines, and the shift in the care of such patients from pediatric cardiological and cardiac surgical...

Impaired exercise capacity is common after the Fontan procedure and is attributed to cardiovascular limits. The Fontan circulation, however, is also distinctively vulnerable to unfavorable lung mechanics. This study aimed to define the prevalence and physiological relevance of pulmonary dysfunction in patients with Fontan physiology. We analyzed data from the Pediatric Heart Network Fontan Cros...

Conversion of the atriopulmonary Fontan to a total cavopulmonary extracardiac connection with concomitant arrhythmia surgery and pacemaker placement is a safe and efficacious procedure for this patient population. From 1994 to 2007 a total of 118 patients have undergone this procedure with one (0.8%) early and nine (7.6%) late deaths. During the course of our experience with Fontan conversion o...

Tricuspid atresia (TAt) is a complex congenital heart defect (CHD) characterized by the absence of the tricuspid valve and right ventricular hypoplasia requiring surgery in childhood, the Fontan procedure. We present a case of a 21-year-old male with TAt and cystic fibrosis (CF), who underwent a Fontan procedure in childhood, presenting to an adult CF clinic with severe deterioration in his res...

OBJECTIVE To determine outcomes of patients undergoing a bidirectional cavo-pulmonary shunt (BCPS) in the Fontan era. METHODS From 1990 to 2000, 212 patients underwent a BCPS in a single institution at a mean age of 2.6+/-5 years. RESULTS Hospital mortality was 6% (13 patients). Five patients were lost to follow-up (3%). After a mean of 9+/-4 years, 15 patients had not yet been referred for...