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PVR Timing in Repaired TOF — EECC MCQ

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ModerateCongenital Heart Disease (Adult)PVR Timing in Repaired TOFEECC

A 22-year-old man with surgically repaired tetralogy of Fallot at age 1 presents with progressive exercise intolerance. CMR shows severe pulmonary regurgitation with RV dilatation (RVEDVi 170 mL/m²) and declining RV function (RVEF 40%). What intervention is indicated?

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Correct answer: E — Pulmonary valve replacement (surgical or transcatheter) — indicated for severe PR with RV dilatation (RVEDVi >160 mL/m²), declining RV function, or symptoms; timing before irreversible RV dysfunction is critical

Late pulmonary regurgitation (PR) after TOF repair is the most common indication for reintervention. Chronic severe PR causes progressive RV dilatation and dysfunction. The 2020 ESC ACHD Guidelines recommend PVR when: (1) symptomatic severe PR; (2) asymptomatic severe PR with ≥2 of: progressive RV dilatation (RVEDVi >160 mL/m²), RV dysfunction (RVEF <47%), sustained atrial/ventricular arrhythmias, RVOT obstruction (peak gradient >60 mmHg), progressive TR. Timing is critical — late referral when RV is severely dilated (>200 mL/m²) is associated with incomplete RV recovery. Options: surgical PVR (bioprosthetic, including options from Contegra conduit) or transcatheter PVR (Melody or SAPIEN in suitable anatomy). CMR is the gold standard for RV volume/function assessment.

Reference: ESC (2020): ACHD Guidelines