A 6-week-old infant has been in cardiac failure since birth. Echocardiography shows an L-transposition of the great arteries (congenitally corrected TGA, cc-TGA). The anatomical right ventricle is serving as the systemic ventricle. Which long-term complication is MOST characteristic of cc-TGA even without associated defects?
- A Progressive systemic (morphological right) ventricular failure and complete heart block ✓
- B Supravalvar pulmonary stenosis
- C Eisenmenger syndrome due to elevated pulmonary vascular resistance
- D Coarctation of the aorta developing in the second decade of life
Explanation
In congenitally corrected TGA (cc-TGA), the morphological right ventricle supports the systemic circulation. The right ventricle is structurally designed for low-pressure pulmonary work and progressively fails when chronically exposed to systemic afterload — failure typically manifests by the fourth to fifth decade. Additionally, cc-TGA has a high intrinsic risk of spontaneous complete heart block (~2% per year) due to the anomalous course of the AV bundle around the non-coronary aortic cusp. Both right ventricular failure and heart block occur even in isolated cc-TGA without associated defects.
Reference: Ghai Essential Pediatrics, 10th ed.
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Written and medically reviewed by the StethoPrep medical team.