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Systemic RV in ccTGA — EECC MCQ

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ModerateCongenital Heart Disease (Adult)Systemic RV in ccTGAEECC

A 22-year-old woman with congenitally corrected transposition of the great arteries (ccTGA) presents with progressive breathlessness. Her systemic ventricle is the morphological right ventricle (RV), which is dilated with moderate systolic dysfunction. What is the fundamental problem and prognosis?

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Correct answer: C — In ccTGA, the morphological RV serves as the systemic ventricle; it is not designed for systemic pressure work and develops progressive dysfunction and failure, often by the 4th-5th decade

Congenitally corrected TGA (L-TGA, ccTGA) has atrioventricular and ventriculo-arterial discordance: blood flows RA→LV→PA and LA→RV→aorta. The morphological RV serves as the systemic ventricle (pumping against systemic afterload). The RV is designed for low-pressure pulmonary circulation and progressively fails under chronic systemic pressure, typically developing dysfunction by age 40-50. The systemic AV valve (morphological tricuspid valve, in the systemic position) often develops significant regurgitation, further accelerating RV failure. The 2020 ESC ACHD Guidelines recommend: (1) monitoring with serial echo/CMR of systemic RV function; (2) standard HF therapy (ACEi, beta-blockers — though evidence is extrapolated from HFrEF trials); (3) cardiac transplant consideration when RV failure is refractory.

Reference: ESC (2020): Guidelines for Adult Congenital Heart Disease