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I collaborate internationally with colleagues from medicine, ecology, landscape architecture, engineering, industry and other social scientists. I was co-author on a highly cited interdisciplinary paper on biodiversity and wellbeing Fuller, Irvine et al which was recommended by Faculty Review and has spawned a new area of research into the potential wellbeing benefits of biodiverse settings.
Line thickness reflects output; color reflects oxygen saturation. Because patients with Fontan circulation are surviving into adulthood in greater s, their management is affected by a relative scarcity of medical knowledge of the pathophysiological origin and optimal care for their unique condition. Whereas multiple reports exist characterizing the condition of these patients, there is an important unmet need in the medical community for a more complete understanding of the pathophysiology, origins, and mechanisms of the development of end-organ consequences.
The evolution of the surgical techniques to achieve this unique circulation began with animal studies in the s and culminated in the successful treatment of 2 patients with tricuspid atresia reported in by Francis Fontan and colleagues 9 and the pioneering work by Guillermo Kreutzer and colleagues 2 reported in Since then, the Fontan procedure has undergone numerous refinements.
The initial use of homograft valves was abandoned because of early calcification and pathway obstruction. The role of therapies such as cardiovascular drugs to prevent and treat complications, heart transplantation, and mechanical circulatory support remains undetermined. Investigations have now demonstrated that the routine use of an to mm conduit for extracardiac TCPC with the avoidance of flow competition between the superior and inferior venous pathways can lead to the lowest power loss; thus, this conduit is a favorable construct.
In low-risk patients with acceptable ventricular and atrioventricular valve function and absence of residual lesions such as aortic or pulmonary arterial obstruction, some centers now proceed with a Fontan procedure on the basis of an assessment with echocardiography and cardiac magnetic resonance only.
The early experience with the Fontan procedure was characterized by evolution of the cavopulmonary connection type. Appropriate medical care for these patients requires coordinated input from pediatric and adult congenital cardiologists, as well as multiple subspecialists, many of whom are not familiar with the physiology or the end-organ consequences of the Fontan circulation.
A focused effort to increase our knowledge about patients surviving single-ventricle palliative surgeries is therefore critically important at this time. In a synthesis of the best current evidence and expertise into a single unified source, this American Heart Association AHA statement outlines the fundamental cardiovascular and extracardiac physiological challenges faced by the patient with Fontan circulation, defines our current understanding of the end-organ consequences, highlights knowledge gaps in need of further investigational research, and provides a rationale to support diagnostic and therapeutic best practices that will benefit this population as it continues to increase in and age over the coming years.
Scheme of normal cardiovascular circulation and Fontan circulation at early and late stages. 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. Figure 1. The hallmark of the Fontan circulation is a sustained, abnormally elevated central venous pressure combined with decreased cardiac output, especially during periods of increased demands, resulting in a cascade of physiological consequences.
The caval veins are directly connected to the pulmonary artery PA ; systemic venous pressures are markedly elevated. Large variation has been observed across centers worldwide in terms of access to heart transplantation. Various techniques of the Fontan procedure. Long-term outcomes for complex biventricular repairs will need to be compared with those for Fontan circulation, as outlined in this statement.
Healthcare providers are ill-prepared to tackle these challenges, as well as specific needs such as contraception and pregnancy in female patients. Potential complications involve not only the heart but also multiple organ systems, including the liver, lungs, brain, bones, and lymphatic system.
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A proposed surveillance testing toolkit provides recommendations for a range of acceptable approaches to follow-up care for the patient with Fontan circulation. AAtriopulmonary connection. As a testimony to the success of the current strategy of care, the proportion of adults with Fontan circulation is increasing. AAll patients and B conditional transplantation-free survival to 1 year.
Evaluation and management of the child and adult with fontan circulation: a scientific statement from the american heart association
These 2 features, elevated systemic venous pressure and chronically decreased output, are the root cause of the majority of the pathological impairments of the Fontan circulation. Considerable investigative efforts have been devoted to discovering ways to further improve surgical technique and to optimize the circulation. Having a single right ventricle supporting the Fontan circulation and, in particular, having hypoplastic left heart syndrome, does not adversely affect early survival to this stage, although patients with a hypoplastic left heart seem to be at higher risk for more complications.
Figure 3. Because fenestration augments ventricular preload and partially offlo systemic venous hypertension, some centers have routinely adopted fenestration for all Fontan procedures, whereas others have used it only in high-risk patients.
Impact of surgical era on transplantation-free survival after the Fontan operation. While there is no standardized age or weight for proceeding from a superior cavopulmonary circulation to Fontan completion, most centers currently proceed when a patient is 2 to 4 years of age. As ever-greater s of patients with single-ventricle types of congenital heart disease CHD survive, this unique population will require continued lifelong medical care, with important personal, societal, and financial impact. In the absence of a subpulmonary ventricle, Fontan circulation is characterized by chronically elevated systemic venous pressures and decreased cardiac output.
This American Heart Association statement summarizes the current state of knowledge on the Fontan circulation and its consequences. The Fontan circulation is based on the premise that a subpulmonary ventricular pump is not compulsory for venous return to cross the pulmonary vascular bed.
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The primary clinical benefit of a fenestration appears to be in reducing the amount and duration of pleural drainage after the Fontan procedure, thus decreasing the duration of hospital stay. Many alterations in the general health of patients with Fontan circulation are related to the biophysics and physiology of their cardiac anatomy and surgery, in addition to psychosocial, neurocognitive, and mental health challenges, which are just being recognized. Figure 2. These variations can readily be explained by the impact of era, surgical techniques, heterogeneity of physiological features in different forms of a single ventricle, and indications for surgery.
A further landmark in the evolution of the Fontan procedure was the introduction of a fenestration between the systemic venous return and the pulmonary venous atrium, which creates a controlled right-to-left shunt.
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Other risk factors are now considered potentially modifiable. Conversely, because the spectrum of ventricular hypoplasia is wide, efforts are currently underway to de creative surgical options in select patients for whom a relatively high-risk biventricular repair may be possible. These more recent modifications eliminate massive atrial dilation and the associated energy losses and stasis.
CFontan circuit late superimposed on early Fontan circuit. Moderate to severe atrioventricular or semilunar valve regurgitation may require repair or replacement, and branch pulmonary artery stenosis or hypoplasia can be improved by arterioplasty or stenting. Consideration may be given to placement of steroid-eluting epicardial le at the time of the Fontan procedure in patients with preoperative atrial arrhythmias or underlying sinus or junctional bradycardia because the transvenous route for placement of pacemaker le can be problematic.
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It has been 50 years since Francis Fontan pioneered the operation that today bears his name. We are also more aware of factors affecting outcomes. However, with improved early surgical outcomes and better postoperative management, a more patient-specific algorithm has been adopted. The early atriopulmonary Fontan technique has been shown to be associated with worse long-term outcomes than the more recent versions of the operation, including the lateral tunnel and the extracardiac conduit.
BFontan circuit. Cardiac catheterization can be replaced with an internal jugular line to measure the superior vena cava pressure. Rather, pulmonary blood flow, and thus preload to the single ventricle, can be driven by moderately elevated central venous pressure when pulmonary vascular resistance is low enough to permit adequate forward flow under these hemodynamic circumstances.
Without clear evidence as to which is superior, both lateral tunnel and extracardiac TCPCs are performed by surgeons today according to personal and institutional preferences.
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The Fontan palliation was introduced in 1 and improved the survival of patients with all types of single-ventricle anatomy, including those with underdeveloped or absent right or left ventricles. Many of the so-called Ten Commandments of requisite anatomic and physiological criteria for Fontan suitability published in by Choussat et al 25 have been overturned or revised. Although technically TCPC can be completed as a single-stage operation, because of the survival benefits initially demonstrated with staged palliation in hypoplastic left heart syndrome, nearly all Fontan procedures are currently performed in planned stages with a transitional superior cavopulmonary connection either the bidirectional Glenn or hemi-Fontan connection.
Liver fibrosis and renal dysfunction are common and may progress over time.
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. An evaluation of survival of a population with Fontan circulation should also now include the impact of cardiac transplantation. Abnormalities in body composition, bone structure, and growth have been detected. There is a clear need for consensus on how best to follow up patients with Fontan circulation and to treat their complications.
Initially deed 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. The Fontan circulation is characterized by the absence of an adequate subpulmonary ventricle. Cognitive, neuropsychological, and behavioral deficits are highly prevalent. Search for more papers by this author. Some centers proceed with planned anticipatory fenestration closure by interventional catheterization during late follow-up.
Essentially, the caval veins are connected without a pumping ventricle to the pulmonary arteries. ANormal biventricular circulation. Reprinted from Gewillig and Brown. However, the optimal age at surgery is now being reconsidered in some centers.
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Clinical hazards faced by patients include progressive fatigue, heart failure, arrhythmias, and end-organ complications such as liver disease, in addition to anxiety and concern about their condition and future. The Fontan operation is approaching its fifth decade. Circulatory failure, ventricular dysfunction, atrioventricular valve regurgitation, arrhythmia, protein-losing enteropathy, and plastic bronchitis are potential complications of the Fontan circulation.
The end-organ consequences of the Fontan circulation are ubiquitous and usually progressive. The pulmonary circulation P is connected in series to the systemic circulation S. The right ventricle RV ensures that the right atrial pressure remains lower than the left atrial pressure and delivers the driving force to the blood to overcome pulmonary impedance.
Collaborating with engineers in Milan, Italy, Marc de Leval and coworkers 16 used computational fluid dynamics to conceptualize the most optimal cavopulmonary connections to limit flow disturbance and to minimize power loss. With time, a negative spiral ensues: Pulmonary resistance increases, resulting in a further increase in caval vein CV congestion but even more in reduced flow and ventricular stretch, which itself increases ventricular filling pressure.