The most common long-term sequelae of tetralogy of Fallot (TOF) are related to acquired late postsurgical pulmonary valve pathology. This is often in the form of pulmonic insufficiency (PI) ultimately leading to right ventricular (RV) enlargement and failure, which may necessitate pulmonary valve replacement. We present a case of severe PI in TOF with an incidental large circumflex to RV fistula and single-vessel coronary artery stenosis in a patient with an enlarged RV. The diagnostic dilemma was to determine whether the RV enlargement was caused by severe PI, shunting from the coronary fistula, or a combination of both.
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