Tetralogy of Fallot, when associated with @entity474 permitting shunting at ventricular level, represents a complex cyanotic @entity600 . Experience with surgical repair is limited, and results vary considerably. Between 1984 and 1996, we repaired 14 consecutive @entity1 with this combination seen in our center. Their ages ranged from 8 months to 21 years (median 7.4 years). Six (42.9%) had @entity1467 . In eight @entity1 the correct diagnosis was made using echocardiography alone. In the remaining six @entity1 , who had previously-constructed arterial shunts and/or suspected @entity849 , catheterization and angiocardiography were also performed. The repair consisted of double patch closure of the @entity1757 , reconstruction of two atrioventricular orifices, and relief of @entity849 at all levels. In five @entity1 with a @entity2150 pulmonary trunk, a monocusp transannular patch (four @entity1 ) or an allograft (one @entity1 ) was used for restoration of continuity from the right ventricle to the pulmonary arteries. Patch enlargement of one or both pulmonary arteries was necessary in five @entity1 . One @entity1 (7.1%) died early, and another late. The twelve surviving (85.8%) @entity1 have been followed for 1.2-12.5 years after surgery (median 4.9 years, mean 5.9+/-3.9 years). During the follow-up, reoperation was necessary for repair of residual @entity1757 and @entity338 in two @entity1 , and closure of an @entity118 and alteration to left @entity338 in one @entity1 . Seven @entity1 are in class I of the New York Heart Association, four in class II, and one in class III. Tetralogy of Fallot associated with @entity474 can be corrected with low mortality and good long-term results. Residual lesions, however, have a tendency to progress, especially when seen in combination. After surgery, all @entity1 need long-term close follow-up.

Repair of tetralogy of Fallot associated with XXXX .

@entity1 :: ('9606', 'Species') :: ['patients', 'patient'] @entity2150 :: ('MESH:D000741', 'Disease') :: ['hypoplastic'] @entity1757 :: ('MESH:D006345', 'Disease') :: ['septal defect', 'ventricular septal defect'] @entity600 :: ('MESH:D000014', 'Disease') :: ['congenital malformation'] @entity474 :: ('MESH:C562831', 'Disease') :: ['atrioventricular septal defect'] @entity338 :: ('MESH:D011665', 'Disease') :: ['pulmonary regurgitation', 'atrioventricular valvar regurgitation'] @entity849 :: ('MESH:D011666', 'Disease') :: ['pulmonary stenosis', 'pulmonary arterial stenosis'] @entity1467 :: ('MESH:D004314', 'Disease') :: ["Down's syndrome"] @entity118 :: ('MESH:D006344', 'Disease') :: ['atrial septal defect']
@entity474 :: (MESH:C562831,Disease) :: ['atrioventricular septal defect']
