81
4.4.5. Fontan Palliation of Single-Ventricle Physiology
COR LOE
Recommendations
erapeutic (cont'd)
2a C-EO
20. In adults with Fontan circulation and hemodynamically
significant stenosis of the Fontan or PAs, catheter-based
stenting of the stenosis to reduce Fontan pressure and
improve flow is reasonable to improve hemodynamics.
2a C-EO
21. In symptomatic adults with Fontan circulation, or those
with evidence of systemic ventricular volume overload,
closure of aortopulmonary collaterals to improve symptoms
and reduce the likelihood of pathologic ventricular
remodeling can be useful.
2b
B-R
22. In adults with Fontan circulation, treatment with pulmonary
vasodilators may be considered to improve exercise capacity.
2b C-LD
23. In adults with an atriopulmonary Fontan, preserved
ventricular function, and acceptable surgical risk with
atrial arrhythmias refractory to medical and catheter-based
therapies, Fontan conversion surgery may be considered to
reduce arrhythmia recurrence.
2b C-EO
24. In adults with Fontan circulation and symptoms or Fontan
circulatory failure attributable to significant intracardiac
structural or anatomic abnormalities, reoperation may be
considered to improve symptoms or reduce the likelihood of
progressive Fontan circulatory failure.
(cont'd)
Table 42. Fontan Palliation: Routine Follow-Up and Testing
Intervals
Type of Follow-Up
or Testing
Physiological
Stage A* (mo)
Physiological
Stage B* (mo)
Physiological
Stage C* (mo)
Physiological
Stage D* (mo)
Outpatient ACHD
cardiologist
N/A 12
6–12
3–6
Electrocardiogram N/A 12
6–12 6–12
Transthoracic
echocardiogram
N/A 12
12 6–12
For recommendations about timing of CMR imaging, see Section 4.4.5 supportive text for
recommendations #6 and #7.
For recommendations about timing of liver imaging, see Section 4.4.5 supportive text for
recommendation #2.
Modified with permission from Stout et al. Copyright © 2018 American Heart Association, Inc.
and American College of Cardiolog y Foundation.
* See Section 2.2 for details on the ACHD anatomic and physiological classification system.
ACHD indicates adult congenital heart disease; CMR, cardiovascular magnetic resonance;
and N/A, not applicable.
