Since 1970, 6 patients have undergone repair of aortico-left ventricular tunnel. Four (67%) had repair in childhood. The technique of closure was by direct suture (5 patients) or patch closure (1 patient). Associated anomalies were seen in 5 patients (83%); absent right coronary ostium (1), commissural fusion (stenosis) (2), valvular regurgitation (3), leaflet defects (2), and healed endocarditis (1). All patients survived operation. At early postoperative review, 67% had mild aortic regurgitation regardless of the technique of surgical repair. Late follow-up revealed that 3 patients (50%) underwent aortic valve replacement (AVR) for progressive aortic regurgitation at a mean of 10 years following initial operation. A review of the literature and our results lead us to conclude that progressive aortic regurgitation is common; it is due to associated valve abnormalities and changes in the valve mechanism secondary to the aortico-left ventricular tunnel. Long-term clinical follow-up is necessary, since 50% of patients will require AVR eventually. Early operation is indicated not only to prevent heart failure but also to prevent progression of damage to the aortic valve.