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Systemic Right Ventricle - Mustard Repair — EECC MCQ

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HardCongenital Heart Disease (Adult)Systemic Right Ventricle - Mustard RepairEECC

A 45-year-old man with a Mustard repair for D-transposition of the great arteries (D-TGA) presents with breathlessness. Echocardiography shows a systemic right ventricle with RVEF 35% and moderate systemic AV valve (tricuspid) regurgitation. What is the key structural concern in this patient?

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Correct answer: A — Systemic RV failure, as the morphological right ventricle supports the systemic circulation and is prone to progressive dysfunction

In patients with atrial switch operations (Mustard or Senning) for D-TGA, the morphological right ventricle serves as the systemic ventricle. The RV was not designed for long-term systemic pressure loading, and progressive systemic RV dysfunction is the most important long-term complication, occurring in the majority of patients by middle age. Associated systemic AV valve (tricuspid) regurgitation accelerates RV failure. Management includes standard HF therapies (though evidence is extrapolated from LV failure), and cardiac transplantation may ultimately be required. The 2020 ESC ACHD Guidelines recommend regular surveillance of systemic RV function.

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