BACKGROUND The "fenestrated Fontan" (surgical baffle fenestration followed by transcatheter test occlusion and permanent closure after postoperative recovery) was adopted in an effort to reduce perioperative mortality and morbidity. This study assesses the effect of baffle fenestration on outcome. METHODS AND RESULTS Patients having a modified Fontan operation with a cavocaval baffle and cavo...

BACKGROUND A Fontan-type operation, i.e. a connection of the systemic veins and pulmonary arteries without subpulmonary ventricle, with different surgical techniques, is nowadays the only treatment option for patients with a functionally univentricular heart (UVH). Understanding the development of pulmonary arteries in patients who are considered for the Fontan procedure is important clinically...

T he Fontan procedure has been extensively used for palliation of patients with univentricular heart defects. Thrombi and thromboembolic complications have been frequently reported after this operation. Magnetic resonance imaging (MRI) has been shown to be very useful in patients with congenital heart disease and also in the detection of intracardiac masses and thrombosis. We describe for the f...

BACKGROUND The Fontan procedure is the definitive operation for palliation of complex congenital heart disease with single-ventricle physiology. Fenestration of the Fontan circuit allows for shunting of deoxygenated blood to the systemic circulation. This procedure improved the clinical outcomes of patients who are at high risk for poor Fontan results. However, it is controversial whether fenes...

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...

Management of adults with failing Fontan physiology poses many challenges, especially as transplantation offers the only realistic alternative to palliative care. We present the first combined heart and liver transplant performed in Europe, for a late survivor of single ventricle palliation with the Fontan circulation. In addition to the conventional medical and surgical challenges posed, we hi...

OBJECTIVE Our approach to the extracardiac conduit Fontan operation has evolved over time from full-pump, to partial-pump, to completely off-pump. This study is designed to report our overall experience with the extracardiac conduit Fontan operation and to evaluate the evolution in bypass technique on postoperative outcomes. METHODS From September 1992 to April 2005, 285 patients, median age ...

OBJECTIVE This study was undertaken to evaluate factors contributing to a decrease in early mortality and morbidity after the Fontan procedure between January 1, 1992, and December 31, 1999. METHODS Outcomes evaluated were early survival, duration of pleural effusions, and duration of hospitalization. Potential predictors evaluated included anatomic diagnosis, presence of a common atrioventri...

BACKGROUND Galectin-3 may play a role in cardiac and noncardiac fibrosis, and elevated circulating levels of this protein predict adverse outcomes in patients with heart failure who do not have congenital heart disease. We investigated galectin-3 in adults with single-ventricle Fontan circulation, patients who are prone to premature clinical deterioration in the context of extensive multiorgan ...

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...