Circulation, Vol 67, 1318-1323, Copyright © 1983 by American Heart Association
AB Lewis, W Wells and GG Lindesmith
The initial surgical approach to the infant with pulmonary atresia and
intact ventricular (PA-IVS) is to establish an adequate source of pulmonary
blood flow and, when possible, relieve right ventricular (RV) outflow
obstruction. The selection of patients for pulmonary valvotomy, alone or in
combination with a systemic-pulmonary arterial shunt, depends on the
presence of an RV outflow tract and the adequacy of the RV chamber. To
evaluate the size of the RV cavity in PA-IVS, an RV index (RVI) was
developed using biplane angiographic measurements of the sum of the
tricuspid valve annulus and the RV inflow and the RV outflow tracts. The
RVI was normalized by relating it to the aortic diameter (Ao) at the
diaphragm (RVI/Ao). The RVI/Ao was 13.5 +/- 1.4 in 20 control subjects and
only 7.3 +/- 2.6 in 26 PA-IVS patients (p less than 0.001), and was within
the normal range in only two of the 26. Since 1976, pulmonary valvotomy
plus a Blalock-Taussig shunt has been performed in 10 infants, with one
death. Serial cardiac catheterizations in five of nine survivors
demonstrated substantial RV growth in all, with the RVI/Ao increasing from
an average of 8.0 to 12.5. In contrast, patients who underwent a shunt
alone had no change in RV cavity size. We conclude that pulmonary valvotomy
may be performed successfully in most PA-IVS patients, but usually must be
combined with a systemic-pulmonary shunt. In a small minority of patients,
a normal RV cavity, as evidenced by an RVI/Ao greater than or equal to 11,
appears to be sufficient to sustain adequate pulmonary blood flow after
valvotomy alone. The RVI/Ao ratio is a simple method of quantitatively
evaluating RV cavity size and is helpful in planning the initial surgical
approach for these infants.
ARTICLES
Evaluation and surgical treatment of pulmonary atresia and intact ventricular septum in infancy
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