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Tetralogy of fallot {S,D,I}: A recently discovered malformation and its surgical management

1995; Elsevier BV; Volume: 60; Issue: 5 Linguagem: Inglês

10.1016/0003-4975(95)00578-9

1552-6259

Richard Van Praagh,

Cardiac Structural Anomalies and Repair

T etralogy of Fallot {S,D,I} is a recently discovered form of tetralogy that was first reported in 1988 by Foran and associates [1] on the centennial of Fallot's classic description of the typical form of this anomaly [2]. Recently, Santini and colleagues [3] reported the first surgical repair of this rare variant of tetralogy without the use of an external conduit. In an accompanying editorial, Anderson [4] raised several questions of general interest that I shall endeavor to answer briefly here. What is tetralogy ofFallot {S,D,I}? This has to be the first question of anyone who has never seen this rare anomaly. As the name indicates, there is situs solitus of the viscera and atria (S), D-loop ventricles (D) with atrioventricular concordance, inverted normally related great arteries (I) with ventriculoarterial concordance, and inverted (mirrorimage) tetralogy of Fallot. There is pulmonary infundibular and valvar stenosis or atresia, aortic-mitral fibrous continuity, and a large subaortic conoventricular type of ventricular septal defect with anterosuperior malalignment of the conal septum. Because the inverted aorta lies to the left of the inverted pulmonary artery, the right coronary artery runs across the obstructed subpulmonary infundibulum from left to right to reach the rightsided atrioventricular groove. This course of the right coronary artery across the obstructed pulmonary outflow tract has been treated by means of an external right ventricle (RV) to pulmonary artery conduit [1]. However, as described in a recent report [3], Jonas and colleagues were able to establish satisfactory RV to pulmonary arterial continuity by myocardial excision beneath the right coronary artery, thereby avoiding an external conduit that would have to undergo repeated changes over time. Does tetralogy of Fallot {S,D,I} 'really have typical morphology of tetralogy of Fallot?' Yes. The morphology of the infundibulum and great arteries was typical of inverted (mirror-image) tetralogy of Fallot, as the angiocardiograms showed well (Fig 2 in [3]). 'Does the chosen title convey the morphology present to the working surgeon? Yes, as long as the surgeon is familiar with this rare form of tetralogy of Fallot [1, 3]. Can one make the diagnosis of double-outlet right ventricle when one of the semilunar valves is in direct fibrous continuity with an atrioventricular valve? Yes. For example, when double-outlet right ventricle coexists with hypoplastic left heart (mitral stenosis or atresia), it is not rare for