80
Specific Conditions
4.4.5. Fontan Palliation of Single-Ventricle Physiology
COR LOE
Recommendations
Diagnostic (cont'd)
2b C-LD
11. In adults with Fontan circulation undergoing cardiac
catheterization (diagnostic, interventional, or
electrophysiological), it may be reasonable to exclude
Fontan-related thrombus before catheter placement to avoid
pulmonary or systemic thromboembolism.
erapeutic
1
B-NR
12. In adults with Fontan circulation and a known or
suspected thrombus, atriopulmonary Fontan, a history of
thromboembolism, or a history of sustained atrial flutter or
fibrillation, anticoagulation is recommended to reduce the
likelihood of thromboembolic disease.
1
B-NR
13. In adults with Fontan circulation and the absence of
high-risk features (history of thromboembolism, sustained
atrial flutter/fibrillation, or atriopulmonary Fontan) or
bleeding contraindications, treatment with either aspirin or
anticoagulation is recommended to reduce the probability of
thromboembolic disease.
1
B-NR
14. In adults with Fontan circulation and sinus node dysfunction
requiring pacemaker placement, atrial-based pacing
with programming to minimize ventricular pacing is
recommended to improve patient symptoms and to avoid
pacing-induced cardiomyopathy.
1 C-LD
15. In adults with Fontan circulation with new-onset atrial flutter
or atrial fibrillation, timely cardioversion (pharmacological
or electrical) is recommended to prevent clinical
decompensation.
1 C-EO
16. Adults with Fontan circulation referred for cardiac
transplantation should undergo pretransplantation review by
a committee with broad multidisciplinary representation to
improve patient selection and posttransplant outcomes.
2a
B-NR
17. In adults with Fontan circulation and atrioventricular block
whose burden of ventricular pacing is likely to be high (ie,
>40%), apical site pacing is preferred over nonapical sites to
improve transplant-free survival.
2a
B-NR
18. In adults with Fontan circulation, formal exercise programs
and/or cardiac rehabilitation (appropriate to the patient's
ability) can be beneficial in improving functional capacity.
2a C-LD
19. In adults with Fontan circulation and recurrent
supraventricular tachycardia, catheter ablation performed
by an electrophysiologist with experience in ACHD is
reasonable to reduce arrhythmia recurrence and associated
morbidity.
(cont'd)
