The long term results of Tetralogy of Fallot (TOF) repair have been
good, pulmonary regurgitation and consequent right ventricular
dilatation is the most common long term sequel of definitive surgery.
Even in such a setting, overt systemic venous congestion is limited to
the rare setting where right ventricular dysfunction sets in due to
deferred surgery or progressive arrhythmia. Here we report a unique case
where a patient developed right heart failure from an unexpected
etiology 14 years after TOF repair.