LAUSR

In 1971, Francis Fontan published his work on the surgical treatment of tricuspid atresia, with the premise that systemic venous return is routed directly into the pulmonary circuit and effectively pulled through the pulmonary capillary bed by residual energy from the systemic ventricle. Although the establishment of Fontan‐type circulation can extend the survival of patients with congenital heart defects, this palliative correction cannot be maintained indefinitely and these patients will require additional forms of treatment. In 2019, the American Heart Association (AHA) published a statement entitled “Evaluation and Management of the Child and Adult with Fontan Circulation,” adding an important contribution (among other factors) to present the various sequelae experienced by palliated single ventricle patients. Potential complications involve not only the heart but also multiple organ systems, including the liver, lungs, brain, bones, and lymphatic system. An important example of the involvement of other systems can be represented on the neurological and neurocognitive functions, genetic abnormalities, and prolonged cyanosis affecting neurodevelopment, learning, and behavioral difficulties are frequently encountered. Rates of anxiety and depression are extremely high in adolescent and adult Fontan patients. As the AHA Statement discusses, in The Failing Fontan, systolic and diastolic ventricular dysfunction are frequently seen, these patients often have the manifestations of chronic low cardiac output, including exercise intolerance, poor somatic growth, and fatigue. Systolic ventricular dysfunction is present in 40%–60% of patients with Fontan circulation undergoing evaluation for heart transplantation. Although the potential need for transplantation is always present, given a continued shortage of donor organs, viable forms of mechanical circulatory support (MCS) for the Fontan patient is critical. Unfortunately, MCS device performance needs to be discussed and adapted to the unique physiological needs of the Fontan circulation: left‐sided failure may be best served with a systemic ventricular assist device; right‐sided failure (preserved systolic function, diastolic dysfunction, sequelae of systemic venous hypertension) may be best served with a cavopulmonary assist device and in some instances, both forms of support may be needed. The statement was necessary and justified due to the greater number of patients, submitted to Fontan circulation, who are reaching adulthood. Reading of this statement is mandatory, it describes the fundamental cardiovascular and extracardiac physiological challenges faced by the patient with Fontan circulation, provides a better understanding of the end‐organ consequences, describes the best diagnostic and therapeutic practices that will benefit this population, and highlights the need to more investigative research.