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Repaired Tetralogy of Fallot — EECC MCQ

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ModerateCongenital Heart Disease (Adult)Repaired Tetralogy of FallotEECC

A 22-year-old man with repaired tetralogy of Fallot is seen in clinic. MRI shows severe pulmonary regurgitation with an RV end-diastolic volume index of 170 mL/m2, RVEF 42%, and QRS duration of 170 ms. He reports reduced exercise tolerance. What intervention should be discussed?

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Correct answer: E — Pulmonary valve replacement

Explanation lettering: E = shown as B · B = shown as E

This patient with repaired TOF has severe pulmonary regurgitation causing progressive RV dilatation and dysfunction. Per the 2020 ESC ACHD Guidelines, pulmonary valve replacement (surgical or transcatheter) is recommended for symptomatic patients with severe PR and RV dilatation/dysfunction (Class I). The RV EDVi of 170 mL/m2 exceeds the commonly used threshold. Tricuspid valve replacement (A) is not the primary issue. ICD (C) does not address the haemodynamic problem. Medical therapy (D) does not reverse PR-related RV overload.

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