OBJECTIVE The modified Fontan procedure represents the final stage for the palliation of hearts with single-ventricle physiology. Different opinions exist regarding the optimal timing of the operation, with most centres advocating early intervention. By contrast, over the past decade, we have progressively increased the age at Fontan operation with the aim to potentially delay the onset of late...

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 imp...

OBJECTIVE Evaluation of incremental risk factors for early mortality in children undergoing orthotopic heart transplantation (OHT) for congenital heart disease. METHODS Between 1988 and 2002, 43 patients (mean age 9.1+/-7.2 years) underwent 44 OHT for complex TGA (6), DORV (4), single ventricle (21), and other end-stage structural heart disease (11). Two discernible ventricular chambers were ...

The original Fontan procedure was first introduced in the 1970s. The concept behind this surgical technique was revolutionary. It has subsequently transformed the lives of children born with complex congenital heart disease which was once thought to be inoperable and resulted in early death. The procedure itself has had several modifications over the decades, with subsequent improvements in lon...

Results Two patients underwent the first Fontan palliations while participating in a development program for congenital heart surgery. Both presented at 32-33 months of age with baseline oxygen saturations of 82-84%. Patient 1 underwent an extracardiac Fontan with a GoreTex conduit larger than the IVC due to limited supplies. Postoperatively, the patient decompensated to a low cardiac output st...

INTRODUCTION A Fontan operation is performed to provide palliation for patients with many forms of highly complex congenital heart disease that cannot support a biventricular circulation. Increasing numbers of women who have undergone these connections in childhood are now reaching their childbearing years, and some are becoming pregnant. The low flow and fixed cardiac output of a Fontan circul...

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 Previous studies suggested a high incidence of congestive heart failure in patients with single and/or systemic right ventricles. The corresponding risk in an adult population is unknown. METHODS AND RESULTS A cohort of 188 consecutive adult patients with single or systemic right ventricles was prospectively assessed with gated radionuclide angiography (n=135) or 2D echocardiograph...