We report the case of a 23 year old patient in whom heart disease was detected in childhood. He began to have suddenly fever and symptoms of right side heart failure. There were signs of aortic regurgitation, a systodiastolic murmur in the left parasternal region and a diastolic murmur in the left 4th intercostal space. The bidimensional and Doppler Echocardiogram showed a saccular dilatation in the right ventricular outflow tract. He was submitted to catheterisation which confirmed aortic regurgitation and detected ventricular septal defect and pulmonary hypertension. During surgery, a subaortic ventricular septal defect was seen as well as a dilatation of the right coronary aortic cusp which prolapsed through the defect to the right ventricular outflow tract. Vegetations were seen in the pulmonary valve. The aortic valve and a pulmonary cusp were excised and a Björk Shiley aortic prosthetic valve was inserted. The septal defect was closed. The patient is still alive, symptomless, with 40 months of follow up. We discuss some problems related to this case, particularly the surgical indication and the rarity of the morphology discovered.

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