Pediatric cardiology
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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.
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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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Pediatric cardiology · Nov 2007
Clinical TrialResults of 100 consecutive extracardiac conduit Fontan operations.
Throughout the years, the experience with Fontan's operation has increased and has opened the way to a much wider application of this principle. A number of major risk factors have been identified and managed by several modifications of the original Fontan procedure. In the past 15 years, operative and postoperative risks have been controlled to a major extent by the application of intermediate surgical procedures. ⋯ At follow-up, 87 patients (89%) were in New York Heart Association (NYHA) functional class I, 4 in class II, and 3 in class III due to moderate to severe atrioventricular valve regurgitation. Use of the following guidelines can result in the achievement of orthoterminal repair with complete separation of pulmonary and systemic circulation, with negligible early mortality and a long-term NYHA class I: (1) Not all patients with univentricular heart (UVH) should undergo the extracardiac Fontan procedure; (2) in UVH with favorable streaming, a Q (p)/Q (s) ratio of approximately 1 to 1, and mild cyanosis, natural history might be preferable to surgical history; (3) any form of fenestration is contradictory to orthodox application of Fontan's principle. Children in whom a planned fenestration seems necessary because of suboptimal conditions should instead undergo a combination of BCPA and associated forward pulmonary blood flow; and (4) negligible mortality should be considered mandatory in UVH, normal pulmonary arteries, and absent cardiomegaly after appropriated and correct staging.