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Objectives:The purpose of this study was to determine the incidence, risk factors, and outcomes of acquired stenosis of the neopulmonary valve after the neonatal arterial switch operation.Methods:Reviewed were the preoperative and follow-up echocardiograms from 136 of 288 patients undergoing the arterial switch operation for whom adequate studies were available. Pulmonary stenosis was defined as a thickened and doming pulmonary valve and a pressure gradient of 20 mm Hg or more. Transposition of the great arteries was present with intact ventricular septum in 91 patients, with a ventricular septal detect in 39, with an aortic coarctation in 5, and with double-outlet right ventricle in 1 patient. No patient had preoperative valvular abnormalities (i.e., a bicuspid valve).Results:During a median follow-up of 18 months (range <1 to 90 months), 32 patients (24%) had the development of supravalvular pulmonary stenosis, 15 (11%) with associated pulmonary valve stenosis (group I). Kaplan-Meier estimates of freedom from any intervention were 94% (95% confidence interval, 90% to 99%) at 1 year and 79% (95% confidence interval, 64% to 94%) at 5 years. The valve anulus before the arterial switch operation was significantly larger (p < 0.03) in those in whom neopulmonary valve stenosis did not develop (group II) than it was in those in whom it did (group I). At follow-up, the pulmonary valve anulus had decreased significantly in diameter in group I (p < 0.0005) and had remained larger in group II (p = 0.06) compared with normal diameter. Group I patients had the development of significant pulmonary valve hypoplasia (p < 0.03) whereas group II patients continued to have significantly larger valves compared with normal size (p < 0.0001).Conclusions:Neopulmonary valve stenosis after the arterial switch operation is not uncommon and is associated with growth failure of the valve anulus often associated with supravalvular pulmonary stenosis.

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