72
Specific Conditions
4.4.1.1. Patients With dextro-Transposition of the Great
Arteries and Atrial Switch
COR LOE
Recommendations
Diagnostic
1 B-NR
1. Adults with dextro-transposition of the great arteries
(d-TGA) and atrial switch who have progressive exercise
intolerance or heart failure should undergo imaging with
transthoracic echocardiogram and CMR imaging to assess
ventricular, valve, baffle, and pathway function to detect and
identify treatable hemodynamic lesions.
1 B-NR
2. Adults with d-TGA and atrial switch who present with
progressive exercise intolerance or heart failure should
undergo CPET to assess for potential intervention targets,
including arrhythmia and desaturation.
1 B-NR
3. Adults with d-TGA and atrial switch with progressive
exercise intolerance, heart failure, pulmonary hypertension,
worsening atrial or ventricular tachyarrhythmia, pathway
obstruction, or baffle leak should undergo invasive
hemodynamic assessment to identify targets for medical,
percutaneous, or surgical intervention.
1 C-LD
4. Adults with d-TGA and atrial switch who require permanent
pacemaker or an ICD should undergo preprocedural
imaging, in addition to intraprocedural contrast venography,
to detect baffle stenosis and residual baffle leaks.
2a B-NR
5. For adults with d-TGA and atrial switch, biomarkers and
validated disease-specific risk scores can be useful to identify
patients at high risk for adverse cardiac events, prompting
closer follow-up and referral to specialized heart failure
transplant centers.
2a B-NR
6. In adults with d-TGA and atrial switch with new or
progressive symptoms of heart failure, it is reasonable to
obtain a resting electrocardiogram and outpatient ambulatory
rhythm monitoring, to ensure timely diagnosis and treatment
of tachyarrhythmia or bradyarrhythmia.
2b B-NR
7. In adults with d-TGA and atrial switch, it may be reasonable
to apply disease-specific scores to identify patients at
increased risk for sustained ventricular tachyarrhythmia and/
or SCD, in order to determine eligibility for a primary-
prevention ICD.
4.4. Complex Lesions
4.4.1. Dextro-Transposition of the Great Arteries
