Background Single ventricle physiology (SVP) is one of the most severe forms of complex congenital heart disease (CHD). Patients undergo multiple surgical interventions including the Fontan procedure (caval venous return is routed directly to the pulmonary arteries). Despite the apparent success of the procedure, it is unclear why some patients develop ‘failing Fontan physiology’ while others r...

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...

A lthough the dramatically improved survival of children with single-ventricle heart disease with Fontan circulation in the recent era is a tremendous accomplishment in pediatric cardiology and cardiac surgery, it has been accompanied by a growing realization that Fontan circulation is largely a state of compensated heart failure at risk for decompensation. Fontan failure occurs in numerous way...

BACKGROUND The Fontan circulation, a result of a palliative procedure in patients with single systemic ventricles, is defined by chronically elevated pulmonary vascular resistance. When traditional heart failure therapies fail, pharmacological agents that reduce pulmonary artery pressures may be used. These include endothelial-receptor antagonists, prostanoids and phosphodiesterase type 5 inhib...

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...

BACKGROUND The Fontan procedure is a successful palliation for children with single-ventricle physiology; however, many will eventually require heart transplantation. The purpose of this study was to determine risk factors for death awaiting transplantation and to examine results after transplantation in Fontan patients. METHODS AND RESULTS A retrospective, multi-institutional review was perf...

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...

Background—The Fontan procedure is a successful palliation for children with single-ventricle physiology; however, many will eventually require heart transplantation. The purpose of this study was to determine risk factors for death awaiting transplantation and to examine results after transplantation in Fontan patients. Methods and Results—A retrospective, multi-institutional review was perfor...

BACKGROUND The Fontan procedure has been modified several times since it was introduced into practice in 1968. As many patients now survive to adulthood, attention is directed towards their clinical status and late morbidity. We report our surgical experience of 30 years in Fontan procedures. METHODS From January 1985 to January 2015, 80 patients underwent Fontan surgery. Twenty-one patients ...