It has been 50 years since Francis Fontan pioneered the operation that today bears his name. Initially designed for patients with tricuspid atresia, this procedure is now offered for a vast array of congenital cardiac lesions when a circulation with 2 ventricles cannot be achieved. As a result of technical advances and improvements in patient selection and perioperative management, survival has steadily increased, and it is estimated that patients operated on today may hope for a 30-year survival of >80%. Up to 70 000 patients may be alive worldwide today with Fontan circulation, and this population is expected to double in the next 20 years. In the absence of a subpulmonary ventricle, Fontan circulation is characterized by chronically elevated systemic venous pressures and decreased cardiac output. The addition of this acquired abnormal circulation to innate abnormalities associated with single-ventricle congenital heart disease exposes these patients to a variety of complications. Circulatory failure, ventricular dysfunction, atrioventricular valve regurgitation, arrhythmia, protein-losing enteropathy, and plastic bronchitis are potential complications of the Fontan circulation. Abnormalities in body composition, bone structure, and growth have been detected. Liver fibrosis and renal dysfunction are common and may progress over time. Cognitive, neuropsychological, and behavioral deficits are highly prevalent. As a testimony to the success of the current strategy of care, the proportion of adults with Fontan circulation is increasing. Healthcare providers are ill-prepared to tackle these challenges, as well as specific needs such as contraception and pregnancy in female patients. The role of therapies such as cardiovascular drugs to prevent and treat complications, heart transplantation, and mechanical circulatory support remains undetermined. There is a clear need for consensus on how best to follow up patients with Fontan circulation and to treat their complications. This American Heart Association statement summarizes the current state of knowledge on the Fontan circulation and its consequences. A proposed surveillance testing toolkit provides recommendations for a range of acceptable approaches to follow-up care for the patient with Fontan circulation. Gaps in knowledge and areas for future focus of investigation are highlighted, with the objective of laying the groundwork for creating a normal quality and duration of life for these unique individuals.

I n 1616, Sir William Harvey was the first to describe the importance of right ventricular (RV) function in his seminal treatise, De Motu Cordis: "Thus the right ventricle may be said to be made for the sake of transmitting blood through the lungs, not for nourishing them." 1,2 For many years that followed, emphasis in cardiology was placed on left ventricular (LV) physiology, overshadowing the study of the RV. In the first half of the 20th century, the study of RV function was limited to a small group of investigators who were intrigued by the hypothesis that human circulation could function adequately without RV contractile function. 3 Their studies, however, were based on an open pericardial dog model, which failed to take into account the complex nature of ventricular interaction. In the early 1950s through the 1970s, cardiac surgeons recognized the importance of right-sided function as they evaluated procedures to palliate right-heart hypoplasia. Since then, the importance of RV function has been recognized in heart failure, RV myocardial infarction, congenital heart disease and pulmonary hypertension. More recently, advances in echocardiography and magnetic resonance imaging have created new opportunities for the study of RV anatomy and physiology.The goal of the present review is to offer a clinical perspective on RV structure and function. In the first part, we discuss the anatomy, physiology, aging, and assessment of the RV. In the second part, we discuss the pathophysiology, clinical importance, and management of RV failure. Anatomy of the RV Macroscopic Anatomy of the RVIn the normal heart, the RV is the most anteriorly situated cardiac chamber and lies immediately behind the sternum. In the absence of transposition of great arteries, the RV is delimited by the annulus of the tricuspid valve and by the pulmonary valve. As suggested by Goor and Lillehi, 4 the RV can be described in terms of 3 components: (1) the inlet, which consists of the tricuspid valve, chordae tendineae, and papillary muscles; (2) the trabeculated apical myocardium; and (3) the infundibulum, or conus, which corresponds to the smooth myocardial outflow region 4,5 (Figure 1). In the study of congenital heart disease, this division seems to be more practical than the traditional division of the RV into sinus and conus components. 5 Additionally, the RV can also be divided into anterior, lateral, and inferior walls, as well as basal, mid, and apical sections. 6 Three prominent muscular bands are present in the RV: the parietal band, the septomarginal band, and the moderator band. The parietal band and the infundibular septum make up the crista supraventricularis. 7 The septomarginal band extends inferiorly and becomes continuous with the moderator band, which attaches to the anterior papillary muscle. 7 When abnormally formed or hypertrophied, the septomarginal band can divide the ventricle into 2 chambers (double-chambered RV). 5 Another important characteristic of the RV is the presence of a ventriculoinfundibular fold that separate...

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Our trial showed that survival rates were significantly higher with the ventricular assist device than with ECMO. Serious adverse events, including infection, stroke, and bleeding, occurred in a majority of study participants. (Funded by Berlin Heart and the Food and Drug Administration Office of Orphan Product Development; ClinicalTrials.gov number, NCT00583661.).