Van Praagh Classification for D-TGA and L-TGA
D-Transposition of the Great Arteries (D-TGA)
- D-TGA is defined by each great artery arising from the wrong ventricle, with atrioventricular concordance but ventriculoarterial discordance 1, 2
- In D-TGA, the aorta arises rightward and anterior to the pulmonary artery from the systemic right ventricle 1, 3
- Ventricular septal defect (VSD) occurs in up to 45% of D-TGA cases 1, 3
- Left ventricular outflow tract obstruction is present in approximately 25% of cases 1, 2
- Coarctation of the aorta is found in approximately 5% of cases 1, 3
- Coronary artery anomalies are common and require clear delineation for surgical planning 1, 3
L-Transposition of the Great Arteries (L-TGA)
- Heart failure develops by age 45 years in 67% of L-TGA patients with associated lesions (VSD, tricuspid insufficiency, pulmonary stenosis) and in 25% of those without associated lesions 4, 5
- The morphological right ventricle functions as the systemic ventricle in L-TGA, which can lead to long-term ventricular dysfunction 4
Surgical Considerations
- Older adults with D-TGA often have had Mustard or Senning procedures (atrial baffle repairs) that redirect blood flow at the atrial level 1, 6
- Common complications after atrial switch include baffle obstruction, baffle leaks, arrhythmias, and systemic right ventricular dysfunction 1, 2
Imaging and Follow-up
- Annual follow-up with a cardiologist experienced in adult congenital heart disease is recommended for all patients with repaired D-TGA 1, 3
- Comprehensive echocardiographic imaging should be performed at a regional ACHD center to evaluate anatomy and hemodynamics 1, 2
- Additional imaging with TEE, CT, or MRI should be performed to evaluate the great arteries, veins, and ventricular function 1, 2
- Assessment of systemic right ventricular function is challenging and may require specialized techniques 1, 3