A management strategy for mild valvar pulmonary stenosis

Pulmonary valve stenosis (PVS) is a common congenital cardiac lesion, 1/1000 live-births, the majority of patients having mild transvalvar gradients. In the present study, we sought to determine the outcome of mild PVS diagnosed by echocardiography and to propose a management algorithm that would identify patients at risk for progression of PVS, yet reduce health care expenditures. In this single-center retrospective study, we included all patients who met the following criteria: first diagnosed with PVS at < 10 years of age, an initial peak systolic Doppler gradient of <= 40 mm Hg, no additional congenital heart lesions, and at least two clinical evaluations. There were 146 subjects who met these criteria. The median age at initial diagnosis was 3.9 months, with a range of 1 day to 9.9 years. The average initial peak systolic gradient (PSG) was 23.3 mm Hg (+/- 7.8) and final PSG 17.1 mm Hg (+/- 10.3). Over a mean length of follow-up of 4.0 years, 107/146 (73%) were later reported to have very mild PVS (PSG <= 25 mm Hg, no clinical change or resolution of murmur). Only 3 of 146 subjects progressed to have a PSG above 40 mm Hg, with 1 undergoing a balloon valvuloplasty. In conclusion, mild PVS diagnosed in early childhood is a benign lesion, with most children essentially demonstrating resolution in the first years of life. Mild PVS identified in infancy requires a brief period of close observation. Based on these data, our management recommendations are that infants be followed closely within the first year of life. Young infants diagnosed at birth that maintain a PSG <= 25 mm Hg at more than 6 months of age as well as those who are older than 1 year of age with a PSG <= 40 mm Hg have a benign course and the utility of ongoing cardiology follow-up is questionable.