Congenital coronary arteriovenous fistula is easily confused with patent ductus arteriosus. An illustrative case is presented which emphasizes the special tests that are necessary to establish the diagnosis. Finally, the successful surgical correction of this abnormality is described.

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In the operative correction of tetralogy of Fallot with a severely narrowed right ventricular outflow tract, widening of the pulmonic annulus is frequently necessary to prevent a high residual pressure gradient and to reduce right ventricular pressure overload. This can be accomplished by incising the pulmonic annulus and inserting a patch graft across the valve, but this usually results in pulmonary valvular insufficiency. Of 426 patients who underwent total correction of Fallot's tetralogy between 1959 and 1970, 63 required a patch across the pulmonic annulus. The mortality rate for this group was 30.1%, compared with a total mortality among the 426 patients of 18%. The high mortality rate is influenced by the fact that the majority were corrected in the early years of the series. Fifteen patients were restudied by cardiac catheterization and cineangiography an average of 9.1 years after total correction. Twelve patients were asymptomatic and three patients had only mild symptoms on exertion in spite of angiographically significant pulmonic regurgitation. The average right ventricular systolic pressure was 40 mm Hg; right ventricular end-diastolic pressure was 7 mm Hg; and the right ventricular/pulmonary arterial peak systolic pressure gradient was 14.9 mm Hg. This experience suggests that after a more difficult immediate postoperative period, patients who have right ventricular outflow reconstruction with patches across the pulmonic ic annulus tolerate their chronic pulmonic regurgitation very well.