Natural history of asymptomatic valvar pulmonary stenosis diagnosed in infancy

Abstract

SummaryBackground 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.Method.v: Between November 1986 and March 1993,51 infants in the Northeast Tennessee and Southwest Virginia region were clinically diagnosed to have isokated 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 valvectomy and the others had percutaneous balloon valvuloplasty). The three other infants showed a more gradual increase of pulmonary stenosis over the fist 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.

Estenose pulmonar valvar discreta: a possível cura espontânea na evolução natural do defeito

“…Few of these 5,7 show an unfavorable evolution of the mild defect, due to the rapid worsening of the condition, similar to what is observed in more severe defects, mainly when exteriorizing occurs early in life. Mody 5 observed that the increase of defect severity was noticed in patients younger than 1 year, even those who presented mild effects, at a faster progression rate than in patients older than 1 year of age.…”

“…The knowledge of the natural history of pulmonary valve stenosis allows us to state that moderate and accentuated defects tend to undergo the intensifying of their effects 2,3 and the mild defects, their decrease [2][3][4][5][6][7][8][9][10][11][12][13] .…”

“…Most of the publications also mention a favorable evolution in pulmonary valve stenosis when they present a mild degree of severity [2][3][4][5][6][7][8][9][10][11][12][13] . Few of these 5,7 show an unfavorable evolution of the mild defect, due to the rapid worsening of the condition, similar to what is observed in more severe defects, mainly when exteriorizing occurs early in life.…”

“…Finally, it can stated that, in mild cases of pulmonary stenosis with a pressure gradient < 30 mmHg, diagnosed and evaluated at any age range, except neonatal and fi rst months of life 5,7 , the evolution is usually favorable up to the spontaneous cure of the defect, and complications that would necessitate some type of intervention are rarely observed in this group of patients in the course of the natural history.…”

“…On the other hand, when the defect has a mild impact, its natural history tends to be even more favorable [2][3][4][5][6][7][8][9][10][11][12][13] , although the "spontaneous cure" is a rare occurrence 8 .…”