Contemporary Management and Future Directions of Adults Living With a Fontan Circulation

Figures

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  Figure 1. Red Flags and Considerations for Fontan Circulatory Failure Clinical presentation red flags/changes from baseline that should prompt further evaluation are outlined. The multiple pathophysiologic factors and underlying mechanisms are highlighted and shown to demonstrate that clinical changes can be attributed to 1 or more pathophysiologic factors and underlying mechanisms. CPET = cardiopulmonary exercise test; CT = computed tomography; EP = electrophysiology; MR = magnetic resonance; MRI = magnetic resonance imaging.

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  Figure 2. Fontan Care–Focused Multidisciplinary Team Persons with lived experience should remain at the center of care delivery, considering their perspectives to optimize outcomes. It is essential that the core team members identify and are supported by other care providers (green circles) who also have expertise in delivering Fontan care. In the background, it is critical that the multidisciplinary care is supported by family and other social supports in addition to infrastructure that provides resources to facilitate care delivery as outlined. ACHD = adult congenital heart disease.

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  Figure 3. Approach to Single Ventricle Assessment Single ventricle function assessment is challenging and can require multiple modalities for an accurate understanding of the contribution of systolic/diastolic function at rest and exertion on clinical status and overall Fontan circulation function. The use of most of these modalities, in particular diastolic function assessment, is extrapolated from the biventricular population and not validated in the single ventricle population. AV = atrioventricular; BNP = B-type natriuretic peptide; CMR = cardiac magnetic resonance; 2D = 2-dimensional; 3D = 3-dimensional; NT-proBNP = N-terminal pro–B-type natriuretic peptide; 2D-PC = 2 dimensional phase contrast; S/D = pulmonary vein systolic/diastolic ratio; other abbreviations as in Figure 1.

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  Figure 4. Comprehensive Management of the Adult with a Fontan Circulation Holistic approach is critical to addressing noncardiac concerns and complications while encouraging lifestyle changes, preventative strategies, understanding the need for lifelong surveillance while minimizing barriers to care. It is paramount that the person with lived experience is supported to gain the skills necessary to receive the required care.

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  Central Illustration. Fontan Circulatory Function Grading Classification Grading along a spectrum ranging from compensated or at risk for dysfunction to overt circulatory failure.1 The stage is defined by the most advanced criterion recognizing that any given patient will have criteria in more than 1 stage. Criteria cutoffs across the stages were determined from previously published consensus definitions1,4 with the proposed grading classification achieved through expert consensus. ∗Fontan specific VO2 for age, sex, and weight.81#Fontan pathway obstruction = anatomical or functional narrowing detected by catheterization or cross-sectional imaging anywhere in the cavopulmonary pathways. May or may not manifest with flow discrepancy on cardiac MRI or hemodynamic perturbation with a gradient ≥1 mm Hg at cardiac catheterization.1 ˆVentricular systolic function as determined by echocardiography and/or MRI imaging.☨atrial or ventricular tachy/bradyarrhythmias. ALP = alkaline phosphatase; ALT = alanine aminotransferase; AST = aspartate aminotransferase; BNP = B-type natriuretic peptide; EF = ejection fraction; eGFR = estimated glomerular filtration rate; FALD = Fontan-associated liver disease; GGT =…