Andersons pediatric cardiology 1920

Management of the “Well” Fontan Routine Surveillance and Testing: the Role of Practice Guidelines The Fontan population is heterogenous with a wide spectrum of functional performance The morbidity profile is highly variable depending on a number of factors including underlying anatomy and ventricular morphology, time from the Fontan procedure, and the era in which Fontan surgery was performed Hence practice guidelines must be adaptable to these features and must also take into account the life stage of the patient For example, the pediatric population has care priorities that differ to those of adults Moreover, the transitions between life stages, lifestyles, and physiologic and psychological states are important milestones that need to be anticipated, and discussed and planned in advance.71,246 There is a paucity of published practice guidelines that adequately traverse the lifetime care of a Fontan patient.247 This is largely due to the lack of a demonstrable advantage of any particular treatment options or management strategies Consequently, there is significant practice variation.248,249 There are few practice guidelines for pediatric care, especially for the “well” Fontan,11 in part due to a general perception that these patients have a low resource requirement Moreover, there is no evidence base to assess the impact of a more structured approach to ongoing surveillance on outcome in this group The majority of published guidelines are for adult patients, often with a focus on the failing Fontan.250–253 Nevertheless, a structured approach to surveillance in childhood is likely to lead to lower resource use and a better understanding as to which investigations are productive at a given time In addition, specific timing of discussion and counseling in relation to exercise participation, teenage risk taking, contraception, and transition to adult care is likely to improve outcomes in these areas The latter is especially important given the mortality and morbidity risk associated with drop out from cardiology surveillance at the time of transition.254–257 The Fontan patient requires regular surveillance over his or her lifetime (Fig 73.23) The core requirement is a regular clinical review with a pediatric cardiologist during childhood and an adult congenital cardiologist thereafter The frequency of review in pediatric practice is debatable; however, American and European guidelines for adult congenital heart disease recommend annual review.250,253 Echocardiography forms the mainstay of imaging given its relative ease of access and noninvasive properties Useful information primarily relates to the function of the ventricle and the assessment of valve regurgitation and the outflow tracts, although additional information including the quantification of the gradient between the Fontan pathway and the pulmonary venous atrium through Doppler interrogation of a patent fenestration or the diagnosis of thrombosis can be useful The primary limitations of echocardiography include a reliance on geometric indices to assess ventricle size and function, which is often problematic given the heterogeneous ventricular morphology encountered in the Fontan population and the increasingly challenging acoustic properties in older patients Nevertheless, it continues to have a place in surveillance for older patients who are MRI incompatible Its usefulness would increase if nongeometric indices of diastolic and systolic performance were able to predict outcome.48,49,258–263 Small studies have shown inconsistent performance to date in this regard, although deformation assessment appears to hold the most promise.49,260,264–266 Cardiac MRI is superior to echocardiography in the assessment of Fontan flow dynamics and the size and systolic function of the systemic ventricle, especially when it is a right ventricle It is a useful adjunct to surveillance particularly in the adult with a Fontan circulation,251,260,267–269 although current guidelines leave its inclusion in regular surveillance to individual assessment.250,253 The ability to perform exercise MRI may lead to its inclusion in future surveillance algorithms.270 The utility of cardiac MRI is limited in the pediatric population given the frequent need for general anesthesia Cardiac CT and cardiac catheterization are useful where there are specific questions not answered by echocardiography or MRI FIG 73.23 Lifelong practice guidelines for the care of the Fontan patient Practice Guidelines can be broken down into Universal Recommendations, Suggested Serial Additional Surveillance, and the Individualized Component The largest part of any Fontan practice guideline is the Individualized Component due to the spectrum of the population and the change in needs over time *Under universal guidelines “regular” follow-up has been defined as at least yearly (From Baumgartner H, Bonhoeffer P, De Groot NM, et al ESC Guidelines for the management of grown-up congenital heart disease [new version 2010] Eur Heart J 2010;31[23]:2915–2957; and Warnes CA, Williams RG, Bashore TM, et al ACC/AHA 2008 Guidelines for the Management of Adults with Congenital Heart Disease: Executive Summary: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines [writing committee to develop guidelines for the management of adults with congenital heart disease] Circulation 2008;118[23]:2395–451.) ... individual assessment.250,253 The ability to perform exercise MRI may lead to its inclusion in future surveillance algorithms.270 The utility of cardiac MRI is limited in the pediatric population given the frequent need for general anesthesia Cardiac CT and cardiac catheterization are useful where there are specific... ACC/AHA 2008 Guidelines for the Management of Adults with Congenital Heart Disease: Executive Summary: a report of the American College of Cardiology/ American Heart Association Task Force on Practice Guidelines [writing committee to develop guidelines for the management of adults with