77
4.4.2. Management of CCTGA
COR LOE
Recommendations
erapeutic
1 B-NR
4. In symptomatic adults with CCTGA, severe systemic
tricuspid regurgitation, and normal or mildly impaired RV
systolic function, surgical tricuspid valve replacement is
recommended to relieve symptoms and preserve RV function.
2a B-NR
5. In asymptomatic adults with CCTGA, severe systemic
tricuspid regurgitation, and normal or mildly impaired RV
systolic function, surgical tricuspid valve replacement is
reasonable to preserve RV systolic function.
2a B-NR
6. In symptomatic adults with CCTGA and high-grade
atrioventricular block, physiological pacing (CRT or
conduction system pacing ) is reasonable to relieve symptoms.
2b B-NR
7. In adults with CCTGA and heart failure with RV
dysfunction, the use of GDMT may be reasonable to improve
ventricular function and functional capacity.
2b B-NR
8. In symptomatic adults with CCTGA and severe LVOT
stenosis, surgical or transcatheter therapy to relieve LVOT
obstruction may be considered to improve symptoms and
preserve LV systolic function while accounting for the potential
risk of worsening the severity of tricuspid regurgitation.
* See Table 39 for details on the periodicity of testing.
(cont'd)
Table 39. Congenitally Corrected Transposition of the Great
Arteries: 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
12
6
Electrocardiogram N/A 12
12 12
Transthoracic
echocardiogram
N/A 12
12 12
For recommendations regarding CMR and cardiac CT, see supportive text for recommendation #2.
For recommendations regarding ambulatory rhythm monitoring, see supportive text for
recommendation #3.
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;
CT, computed tomography; and N/A, not applicable.
