The Fontan circulation was one of the “big ideas” in congenital heart disease in the 1970s. This completely novel type of circulation was at first the source of some astonishment but, gradually, understanding of its properties progressed and there is now an expanding cohort of patients followed up with stable Fontan circulations. In 1990, on the basis of an observational study of the survival a...

OBJECTIVE Evaluation of incremental risk factors for early mortality in children undergoing orthotopic heart transplantation (OHT) for failing single ventricle physiology. METHODS Between 1988 and 2002, 25 patients (mean age 9.3+/-7.1 years) underwent OHT for complex congenital heart disease (CHD) with a functional right (15 patients) or left (10 patients) single ventricle. Palliative staging...

BACKGROUND To better define determinants of mortality in patients with univentricular physiology, a database registry was created of patients born in 1985 or earlier with Fontan surgery who were followed up at Children's Hospital Boston. METHODS AND RESULTS A total of 261 patients, 121 of whom (46.4%) were women, had a first Fontan surgery at a median age of 7.9 years: right atrium-to-pulmona...

Persistent left superior vena cava (SVC) is a not uncommon finding in patients with congenital heart disease. This anatomical variant must be recognised before doing a Glenn anastomosis, bidirectional cavopulmonary connection or a Fontan-type procedure. Following these procedures, reopening of a left SVC leading to clinical cyanosis can occur. Five cases are described in whom persisting left SV...

We thank the Editor for giving us the opportunity to reply to the ‘Letter to the Editor’ describing the unguarded tricuspid orifice with pulmonary atresia [1]. We congratulate Kariya et al. for successful surgical treatment using a total right ventricular exclusion and total cavo-pulmonary connection (TCPC). Because of the high mortality of this rare anomaly, there are only a few successful rep...

The bidirectional Glenn operation may be particularly useful as an intermediate procedure before Fontan correction in high-risk patients. From October 1989 through February 1992, 50 patients 1 to 60 months old (median 12) have undergone a bidirectional Glenn operation. Diagnoses included hypoplastic left heart syndrome in 21 patients, pulmonary atresia with intact ventricular septum in 10, tric...

BACKGROUND In adult Fontan patients, ventricular or arterial dysfunction may impact homeostasis of the Fontan circulation and predispose to heart failure. We sought to characterize ventricular-arterial (VA) properties in adult Fontan patients. METHODS AND RESULTS Adult Fontan patients (n=170), including those with right (SRV, n=57) and left (SLV, n=113) dominant ventricular morphology, were c...

complexity of some congenital heart diseases sometimes necessitates a combination of interventional procedures and surgery, amongst which intraoperative stent implantation is one of the most common. we herein report a successful hybrid procedure in a cyanotic adult patient who had undergone no procedure in childhood. the patient was a 24-year-old cyanotic male (oxygen saturation in the room air...

The Fontan operation has been used to palliate patients with a functional single ventricle. In many such patients the operative risk for a Fontan procedure is high. The bidirectional cavopulmonary anastomosis (BCPA) is a useful intermediate palliative procedure before Fontan correction. It may reduce the deleterious sequelae of chronic hypoxaemia and long-term ventricular overload, thus yieldin...

OBJECTIVES The optimal timing of the Fontan staging for a univentricular heart and its impact on growth remains debateable. In a Fontan cohort, the influence of staged interventions and patient factors on somatic development was explored. METHODS We reviewed 64 total cavopulmonary connection (TCPC) patients treated since 1992. Serial anthropometric parameters recorded from birth to the latest...