Natural History of Asymptomatic Valvar Pulmonary Stenosis Diagnosed in Infancy

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Background and hypothesis: Valvar pulmonary stenosis is a common congenital heart defect. Progression of stenosis over time, even when mild initially, has been shown by serial cardiac catheterization studies in children and adults. We studied the natural history of asymptomatic valvar pulmonary stenosis diagnosed in infancy with two-dimensional echocardiography and Doppler method. Methods: Between November 1986 and March 1993, 51 infants in the Northeast Tennessee and Southwest Virginia region were clinically diagnosed to have isolated valvar pulmonary stenosis. In 40 patients, the diagnosis was confirmed by two-dimensional echocardiogram/Doppler and color- flow mapping study at the time of presentation, and only their course is reported. Of 40 infants, six asymptomatic infants (15%) showed rapid progression of pulmonary stenosis over a relatively short period of time. Within the first 6 months of life, three of the six infants showed worsening of the stenosis needing intervention (one had surgical vulvectomy and the others had percutaneous balloon valvuloplasty). The three other infants showed a more gradual increase of pulmonary stenosis over the first 2 years of life. Results: Pulmonary stenosis even when mild can worsen in infancy, and it is not possible to predict which patients will follow this course. In our group of asymptomatic infants with initial mild pulmonary stenosis, 15% developed significant stenosis that needed intervention. Conclusion: We recommend frequent follow-up of asymptomatic infants with mild pulmonary stenosis during the first 2 years of life to detect rapid progression that may need intervention.