Pediatric cardiology
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The Fontan procedure has been a significant contribution to the therapeutic armamentarium of congenital heart surgeons for the treatment of single ventricle anomalies. However, it has its limitations. Patients may have failure of the Fontan circulation acutely postoperatively or late during the long-term follow-up. ⋯ Late failure may be related to a number of different factors, many of which are amenable to reoperation. Every effort should be made to identify which hemodynamic factors are responsible for late failures. However, cardiac transplantation will likely be necessary for the majority of patients.
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The Fontan operation accomplishes complete separation of systemic venous blood from pulmonary venous circulation in patients with single ventricle anatomy. Operative survival since the first description of the Fontan operation is excellent in the current era through modifications in surgical techniques, identification of patient-specific risk factors, and advances in postoperative care. ⋯ Even after excellent early results, patients with single ventricle lesions remain at risk of progressive ventricular dysfunction, dysrhythmias, progressive hypoxemia, elevated pulmonary vascular resistance, and protein-losing enteropathy, which can result in morbidities including but not limited to, myocardial failure, thromboembolism, and stroke. Consequently, continued long-term survival of patients who undergo the Fontan operation is dependent upon preservation of single ventricle function, avoidance of late complications, and, in the patient with a failing Fontan, recognition and treatment of the underlying pathophysiologic process that has resulted in Fontan failure.